He had been hospitalized for this problem at an outside hospital and treated with antibiotics, without improvement.

The swelling worsened and extended to his upper lip.

He developed difficulty swallowing, headache, and nasal drainage.

On presentation, he was afebrile without leukocytosis.

Physical examination demonstrated diffuse swelling and tenderness of the nose with honey-colored crusting that extended to above his upper lip.Axial and sagittal contrast-enhanced computed tomography (CT) (Figure, A and B) demonstrated marked soft tissue swelling and fullness with abnormal enhancement along the nasal cavity, including bilateral nasal ala, with areas of necrosis and emphysema.

No significant bony erosion or associated calcification on bone windows was noted.

After bedside debridement of the nasal soft tissue region, axial postcontrast T1-weighted fat-suppressed and sagittal T2-weighted fat-suppressed magnetic resonance imaging through the nasal cavity (Figure, C and D) demonstrated soft tissue fullness isointense to muscle on T1-weighted images, hyperintense on T2-weighted images, and with homogenous enhancement of the solid component on postcontrast T1-weighted images.

No intracranial or intraorbital disease extension was noted.Contrast-enhanced computed tomography (CT) demonstrates marked soft tissue swelling and fullness with abnormal enhancement along the nasal cavity, including bilateral nasal ala, with areas of necrosis and emphysema.


On physical examination, the mass was firm, mobile, tender to palpation, and caused restricted range of motion.

Ultrasound imaging of the soft tissue of the neck was obtained, which demonstrated a neck mass with dense acoustic shadowing (Figure 1A).

Further evaluation with contrasted computed tomography (CT) imaging showed a 3.3×2.9×2.6-cm ossified lesion in the left posterior-lateral cervical soft tissue near the atlanto-axial junction.

The lesion was well circumscribed and separate from the underlying bone (Figure 1B).

The gross pathology specimen showed a firm, irregular, encapsulated mass.A, Ultrasonography of the neck soft tissue demonstrating a neck mass with dense acoustic shadowing in the left posterior triangle.

B, Axial computed tomographic imaging of the neck with contrast depicting a 3.3-cm well-circumscribed ossified mass that is separated from the C2 vertebral body.

No aggressive-appearing periosteal reaction is present (arrowhead indicates calcified neck mass).

The patient underwent excision of the mass without complication.


He did not smoke and only drank alcohol occasionally.

He was living in Japan and recently visited a neighboring island but denied any international travel.On examination, his blood pressure was 142/84 mm Hg; temperature, 38.3°C; pulse, 91/min; respiratory rate, 24/min; and oxygen saturation, 97% on ambient air.

Five days before arrival, he developed throat pain, rhinorrhea, nonproductive cough, and fever.

Two days later he noticed a maculopapular rash on his neck and trunk, which later involved his upper and lower extremities.

There were no mucosal lesions or conjunctival injection.

A maculopapular rash was noted on the trunk and extremities (Figure) as well as the palms and soles.

Neurologic examination, including results of motor, sensory, and reflex testing, was unremarkable.

There was no neck stiffness or lymphadenopathy.

Laboratory testing showed a white blood cell count of 2900/μL (80% neutrophils, 13% lymphocytes, 6% monocytes, 0% eosinophils, and 0% basophils); hemoglobin level, 15.2 g/dL; and platelet count, 187 ×103/μL.


She reported no cough, chest pain, orthopnea, leg swelling, or viral-like symptoms.

Her temperature was 36.8°C, heart rate 102/min, respiratory rate 22/min, and blood pressure 124/76 mm Hg.

She had conjunctival pallor and scleral icterus, but no lymphadenopathy, hepatosplenomegaly, or ecchymosis.

Results of initial laboratory testing are presented in the Table.


The pain was described as crushing and substernal, but she denied radiation or associated dyspnea, nausea, vomiting, or diaphoresis.

On physical examination, her blood pressure was 167/105 mm Hg bilaterally, heart rate was 71/min, respiratory rate 18/min, and oxygen saturation 98% on room air.

Initial laboratory evaluation revealed a troponin I level of 0.12 ng/mL (reference range, 0.00-0.04 ng/mL).

An electrocardiogram showed normal sinus rhythm with diffuse ST-segment depressions but without q waves or ST-segment elevation (Figure, left).

Computed tomography angiography did not show pulmonary embolism or aortic dissection.

Echocardiography demonstrated a left ventricular ejection fraction of 53%, with apical akinesis and no valvular disease.Clinical findings from 12-lead electrocardiography (left) and right anterior oblique cranial angiography of the left anterior descending artery during diastole (right).The patient was started on aspirin and ticagrelor, heparin infusion, and metoprolol tartrate for treatment of non–ST-segment elevation myocardial infarction.


He had a history of hypertension that was treated with an angiotensin receptor blocker.

On the second postoperative day, body temperature was 38.6°C.

Blood pressure decreased from 145/72 mm Hg to 96/53 mm Hg with a heart rate of 105/min.

Preoperative estimated glomerular filtration rate (GFR) was 98 mL/min/1.73 m2.

Chest x-ray revealed bilateral lung consolidations consistent with pneumonia.

The patient was admitted to the intensive care unit (ICU) with acute respiratory failure and was placed on mechanical ventilation.

In the ICU, urine output was 50 mL over 3 hours.

On day 3, he received 4.5 L of Ringer lactate over 12 hours.

Laboratory values are shown in the Table.The change in serum creatinine is within the coefficient of variation.The patient has acute kidney injury most likely related to sepsis.There are insufficient data to conclude that a decrease in glomerular filtration rate has occurred.


His medical history was unremarkable, and he noted no environmental triggers or new medications.

He endorsed severe pruritus at these sites that would wake him up at night.

He denied any recent trauma or history of similar lesions.

He denied associated pain, blisters, or systemic symptoms (eg, fever, chills).

Physical examination revealed 3 well-circumscribed, violaceous, annular patches without scale or erosion that are limited to his right axilla (Figure, A).

No other lesions were noted on his skin or mucous membranes.

A punch biopsy specimen from a lesion was obtained (Figure, B and C).A, Clinical photograph shows oval and annular thin violaceous patches in the right axilla.

B, Hematoxylin-eosin stain (original magnification ×40) demonstrates lymphocytes obscuring the dermal-epidermal junction.

C, Hematoxylin-eosin stain (original magnification ×200).

Treatment with over-the-counter hydrocortisone cream, ketoconazole cream, and discontinuation of underarm deodorant yielded no improvement.


She had a 15-year history of oral lichen planus (OLP) managed with betamethasone dipropionate, 0.05%, gel twice daily and clotrimazole troches, 10 mg, 3 times daily as needed for symptomatic OLP flares.

Medical history revealed stage 1A mycosis fungoides/cutaneous T-cell lymphoma affecting the right calf and left thigh managed primarily with halobetasol, 0.05%, cream owing to intolerance of narrowband UV-B therapy and mechlorethamine, 0.016%, gel.

Immunosuppression or history of infectious diseases, including human immunodeficiency virus (HIV), was not reported.

A 1.5 × 1.0-cm nonremovable white, plaquelike lesion was observed on the left lateral tongue (Figure 1A).

Biopsy specimens were obtained with a 3-mm punch instrument at 3 different sites, which demonstrated similar microscopic findings (Figure 1B and C).A, A corrugated, nonremovable white lesion on left lateral tongue.

B, Histologic image showing hyperkeratotic, acanthotic stratified squamous epithelium exhibiting cells with lightly stained cytoplasm (balloon cells) throughout the stratum spinosum and superficial layer (hematoxylin-eosin).

C, Histologic image showing several epithelial nuclei with peripheral chromatin margination also described as chromatin beading (arrowheads) (hematoxylin-eosin).


The lesion first appeared 12 months before presentation and was progressively growing.

He was otherwise well and denied any fevers or weight loss.Clinical examination revealed a dark brown longitudinal pigment band covering the full length of the toenail of the left second digit with corresponding nail thickening over the pigmented band.

The pigment was the same width throughout the band and extended to the proximal nail fold (Figure, A).

No other nail or skin lesions were found at the time of examination.

A biopsy specimen was obtained from the nail plate and nail matrix and stained with hematoxylin-eosin (Figure, B-D).A dark brown longitudinal pigment band on the second toenail with pigment extends to the proximal nail fold (A).

Histopathologic examination of the specimen with hematoxylin-eosin stain is seen at original magnification of ×40 (B), ×100 (C), and ×200 (D).

This was his first time presenting to a physician for this problem, and no prior treatments had been attempted.


He began college 6 months prior but had found the workload difficult and failed his examinations.

He had no family history of mental disorders, denied any current or past use of drugs, and reported no significant medical history.

He reported that he no longer enjoyed his former interests and could not relate to people at college or to friends, but there were no clear signs of depressive disorders.

He was 80% convinced that random people looked and talked about him when he was out in public but was able to question it.

He stated that these people were probably commenting on the way he looked, but he did not believe these individuals meant him harm.

He also reported a vague feeling of perplexity and derealization.

These experiences began when he started college and continued to occur every day for up to an hour at a time, causing significant distress.

He was well kempt, was quiet during his interview, and provided short answers.

No formal thought disorders were elicited.


Her parents were nonconsanguineous, and her family history was unremarkable.

She had unsteadiness and frequent falls at gait initiation since age 16 months.

There was a progressive worsening of her gait difficulties, and she complained of loss of balance.On examination, she had saccadic pursuit, subtle appendicular ataxia, and a spastic gait with a wide base.

She could not stand or walk with her feet in the tandem position.

Achilles tendon reflexes and plantar responses were absent, and vibration sense was decreased in her toes and ankles.Visual acuity, color vision, and visual fields were normal.

Fundoscopy revealed increased visibility of retinal nerve fibers, hiding the contours of retinal vessels bilaterally.

Optical coherence tomography (OCT) showed increased thickness in the retinal nerve fiber layer (RNFL) (Figure 1).Ophthalmological findings include color fundus photograph of the right eye (A) with increased visibility of the retinal nerve fiber layer, which hides retinal vessel contour; an optical coherence tomographic vertical B-scan revealing a markedly thickened retinal nerve fiber layer (asterisks) (B), captured along the green line seen on a red-free image of fundus (C).


History revealed multilevel cervical and lumbar fusion surgical procedures using anterior, left lateral, and posterior approaches over a 10-year period.

The patient subsequently noted a new, slowly enlarging bulge in her left midback that was bothersome with activities of daily living (Figure, A).

She denied pain directly overlying the area and was otherwise tolerating a regular diet and having usual bowel function.

Physical examination revealed a soft bulge in the lateral left midback.

There were no apparent associated skin changes, and the palpable mass appeared fixed and nonreducible.

The mass was most apparent with upright posture and was accentuated with a cough impulse, but it was less distinct when examined in the prone position.

Computed tomography of the abdomen and pelvis was obtained (Figure, B).Patient presentation (A) and computed tomography of the abdomen and pelvis (B).


On examination, he was noted to have some cervical midline spine tenderness.

He had been seen in his primary care physician’s office about 2 weeks prior to emergency department presentation and had been treated for musculoskeletal symptoms.

He admitted to paresthesia and pain radiating down his right arm but denied any weakness.

No weakness or sensory deficits were identified.

A plain radiograph of the neck demonstrated tracheal deviation, and he then underwent computed tomography of the chest (Figure 1A) and magnetic resonance imaging of the cervical and thoracic spine (Figure 1B).Computed tomography scan and magnetic resonance image.

He had no difficulty with speech or swallowing.

He had no medical comorbidities or prior surgery.

No palpable lymphadenopathy, swelling, or mass was noted.

A, Coronal computed tomography image of the chest; B, sagittal magnetic resonance image of the cervical and thoracic spine.


She denied additional ocular symptoms and reported no relevant medical, ocular, or family history; medication use; or allergies.

One week later, her symptoms had worsened, and she was referred for consultation.Best-corrected visual acuity was 20/15 OD and 20/60 OS.

The findings of a review of systems were unremarkable.

Intraocular pressures, extraocular movements, visual fields by confrontation, and pupil responses were normal.

Anterior segment examination and dilated fundus examination of the right eye had normal findings.

However, the left eye had 3+ anterior chamber cell and 1+ flare, nongranulomatous keratic precipitates, and a clear lens.

The dilated fundus examination revealed 2+ vitritis, a hyperemic disc with blurred margins, macular striae with a blunted foveal reflex, and vascular sheathing with segmental periarteriolar inflammatory plaques in multiple quadrants.

The peripheral retina had well-demarcated peripheral retinal whitening with scalloped margins and intraretinal hemorrhages in all quadrants (Figure 1A and B).

Four days later, repeat fundus photography showed that the peripheral lesions had coalesced into larger circumferential necrotic regions (Figure 1C).A, Color fundus photography montage of the left eye shows hyperemic disc with blurred margins, vascular sheathing, and peripheral retinal whitening.

B, Magnified image of the temporal retina shows well-demarcated infiltrates with scalloped margins and adjacent intraretinal hemorrhages.

A workup for posterior uveitis was initiated, and the patient began therapy with oral antiviral and oral corticosteroid medications.


Previous episodes had been treated successfully with oral prednisone, and the patient had numerous recurrences within the past 10 years.

According to the patient’s report, a biopsy was performed several years ago at an outside institution, and the diagnosis was xanthelasma.

She denied visual changes.A, External photograph demonstrating bilateral periorbital swelling greater in the left than right eyelids, with associated yellow lesions.

B, Axial computed tomographic scan with contrast demonstrating enlargement and homogeneous enhancement of the left lacrimal gland and left lateral rectus muscle and bilateral preseptal soft tissues.Visual acuity was 20/40 OU  and improved to 20/25 OU with pinhole testing.

Intraocular pressures were normal, extraocular motility was full, and the pupils were round and reactive without an afferent pupillary defect.

Results of the examination revealed yellow lesions on both eyelids, palpebral swelling greater in the left than the right eye, erythema, ptosis, an S-shaped deformity of the left upper eyelid, and a palpable mass of the left superolateral orbit (Figure 1A).

Hertel exophthalmometry showed 2 mm of relative proptosis in the left eye.

Results of slitlamp and dilated fundus examinations were unremarkable.

A computed tomographic scan showed preseptal soft tissue swelling in both eyes and an extraconal left lacrimal gland mass extending behind the globe (Figure 1B).

We contacted the outside institution, and no reports of histopathologic examination of the biopsy specimen were found.


The patient reported a negative review of systems and denied recent travel, trauma, use of antibiotics, or pregnancy.

During the session, the physician assistant removed the metal eye shield for better exposure, immediately after which the patient developed eye pain and blurred vision.

Her examination was notable for best-corrected visual acuity of 20/20 OU, anterior chamber (AC) pigment and flare in the left eye, and iris irregularities, including decreased iris reactivity, peripupillary transillumination defects (Figure, A), and posterior synechiae.

She had mild intraocular pressure (IOP) asymmetry of 12 mm Hg OD and 17 mm Hg OS.A, An irregular pupil, iris atrophy, and peripupillary transillumination defects are observed.

B, After discontinuation of the cycloplegic agent, posterior concavity of the iris and iris-lens touch consistent with reverse pupillary block are observed.Gonioscopy revealed increased trabecular meshwork pigmentation in the left eye.

Ultrasonographic biomicroscopy and anterior segment optical coherence tomography demonstrated a normal anterior segment in the right eye and posterior bowing of the iris and lens-iris touch (Figure, B) with a normal AC depth in the left eye.

She was initially treated for acute iritis with difluprednate, 4 times daily, which was tapered to once daily, with minimal improvement in her symptoms.

Six weeks before, she received eyebrow laser epilation with an 800-nm diode laser (LightSheer; Lumenis).


Best-corrected visual acuity was 20/20 OU with a 2-diopter myopic shift in the left eye.

There was no afferent pupillary defect, color vision was normal, and intraocular pressure (IOP) was 10 mm Hg OD and 8 mm Hg OS.

Visual field testing revealed an enlarged blind spot in the left eye, and optical coherence tomography of the left eye revealed thickening of the retinal nerve fiber layer in all quadrants.

The results of examination and testing of the right eye were unremarkable.

The results of workup with a complete blood cell count, basic metabolic panel, angiotensin-converting enzyme measurement, Lyme disease testing, and antinuclear antibody measurement were unremarkable.Ophthalmic examination in the emergency department revealed diminished visual acuity to counting fingers, an IOP of 59 mm Hg, and a relative afferent pupillary defect in the left eye.

She was treated with a topical corticosteroid, with rapid resolution of iritis and commotio.

On her initial ophthalmic examination 3 weeks earlier, she was diagnosed with commotio retinae and traumatic iritis in the left eye after being struck with the brim of a hat.

However, she developed ipsilateral, asymptomatic grade 2 optic disc edema.


He denied any visual or systemic symptoms, and his medical history was significant for hypertension and atrial fibrillation treated with cardiac catheterization and ablation.

Medications included amlodipine besylate, 5 mg daily, and aspirin, 81 mg daily.

His family and social history were unremarkable.

On examination, his visual acuity was 20/20 OU.

The intraocular pressure in both eyes was normal, and there was no relative afferent pupillary defect.

Extraocular motility and confrontational visual fields were also normal.

On physical examination, facial plethora was evident (Figure 1A).

Retinal examination of the left eye revealed 2 blot hemorrhages and dilatation of the retinal veins (Figure 1B).

Retinal examination of the right eye also revealed dilatation of the retinal veins.

Fluorescein angiography revealed a prolonged arm-to-retina transit time of 22.6 seconds (normal, 10-16 seconds).

Optical coherence tomography scans of the macula in both eyes were normal.

In addition, an area of bruising was noted on the right shoulder in the absence of any history of trauma to the shoulder.A, Facial plethora.

These abnormal findings were not present 1 year earlier.


He denied any vision changes, eye pain, or light sensitivity.

A comprehensive review of symptoms was negative, including no fatigue, weight loss, or night sweats.

Best-corrected visual acuity was 20/40 OD and 20/30 OS.

Anterior segment examination revealed ocular surface dryness and a nuclear sclerotic cataract in the right eye.

On dilated fundoscopy, multiple yellow-white subretinal deposits were scattered throughout the left posterior pole, with parafoveal coalescence but sparing the far periphery.

There was no concomitant vitritis (Figure, A).

Optical coherence tomography showed choroidal thickening and irregularity of the overlying retinal pigment epithelium (Figure, B).

The right eye was unremarkable.A, Fundus photograph of the left eye shows scattered hypopigmented subretinal deposits with parafoveal coalescence.

B, Optical coherece tomographic findings show patchy choroidal thickening with irregularity of the overlying retinal pigment epithelium (arrowhead).

The neurosensory retina maintained its regular architecture.


He smoked approximately 10 cigarettes per day for 4 years.

Results of the physical examination were unremarkable.

Radiography of the chest revealed an ill-defined consolidation in the right lower lung field.

Laboratory investigations were significant for elevated levels of lactate dehydrogenase (273 U/L; reference range, 135-225 U/L [to convert to microkatals per liter, multiply by 0.0167]), C-reactive protein (125.3 mg/L; reference range, 0.0-4.9 mg/L [to convert to nanomoles per liter, multiply by 9.524]), and alkaline phosphatase (217 U/L; reference range, 40-129 U/L [to convert to microkatals per liter, multiply by 0.0167]).

Computed tomography of the chest confirmed the presence of a dense consolidation involving most of the right middle lobe with right hilar and subcarinal lymphadenopathy (Figure, A).

He underwent endobronchial ultrasonography-guided transbronchial needle aspiration of a fleshy, whitish endobronchial lesion obstructing both uptakes to the medial and lateral segments.

However, findings were negative for S-100, melan A, placental alkaline phosphatase, CD56, thyroid transcription factor-1, terminal deoxynucleotidyl transferase, CD45, and cytokeratin 7.

Histologic analysis revealed a poorly differentiated malignant neoplasm (Figure, B).

Most of the cells were relatively monotonous, with enlarged vesicular nuclei, prominent nucleoli, and a high ratio of nucleus to cytoplasm.

Foci of abrupt squamous differentiation, including keratinization and intercellular bridges, were identified.

Immunohistochemical staining showed tumor cells to be strongly and diffusely positive for keratin AE1/AE3, cytokeratin 5/6, and p63.

A positron emission tomography–computed tomography scan showed a fludeoxyglucose-enhanced avid right middle lobe mass, a large right-sided pleural effusion, and fludeoxyglucose-enhanced avid mediastinal and right hilar lymph nodes, with extensive skeletal metastases to both proximal humeri and scapulae, the sacrum, spine, iliac bones, and both hips.A, Computed tomography of the chest revealed a dense consolidation in the middle lobe of the right lung with right hilar and subcarinal lymphadenopathy.

B, Histologic analysis of the mass revealed a poorly differentiated malignant neoplasm.

Most cells showed enlarged vesicular nuclei, prominent nucleoli, and a high ratio of nucleus to cytoplasm (hematoxylin-eosin; original magnification ×200).


She had a history of deep vein thrombosis 3 years prior but was not receiving long-term anticoagulation.

She remained comatose and was intubated for airway protection.

A physical examination revealed a rapid irregular pulse, no jugular venous distension, clear lung fields, and no cardiac murmurs.

Laboratory findings were notable only for a troponin T level of 2.41 ng/mL (to convert to micrograms per liter, multiply by 1).

An electrocardiogram (ECG) and a point-of-care transthoracic echocardiogram (TTE) were obtained (Figure 1; Video).

After resuscitation, the return of spontaneous circulation was achieved.


He denied any symptoms related to the lesion, including dysphonia or dysphagia, and reported no fevers or weight loss.

Postoperatively, the patient was doing well and continued to have no symptoms of dysphagia or dysphonia.

Rigid stroboscopy revealed normal vocal fold mobility and a nodular cystic-appearing lesion on the posterior inferior border of the right vocal process (Figure, A).

There was no overlying ulceration, and vocal fold mucosal wave was intact in the adjacent membranous vocal fold.Suspension microlaryngoscopy incisional biopsy of the right infraglottic lesion was performed in the operating room.

Intraoperatively, there was submucosal white caseous debris in the substance of the lesion.

Cultures were analyzed for the presence of fungus, bacteria, and mycobacteria.

The endotracheal tube was displaced anteriorly with the laryngoscope.

The surgical specimen was sent for histopathologic analysis (Figure, B).


The swelling was mild at onset and gradually progressive.

There was no history of associated pain, but he reported occasional pruritus over the swelling.

There was no history of fever, weight loss, or loss of appetite.

He denied any history of similar swelling elsewhere in his body.

General physical and systemic examination had normal results.

On examination of the swelling on the right side, there was a diffuse swelling around the pinna measuring approximately 6 cm over the preauricular, postauricular, and infra-auricular region.

It was a nontender, soft swelling without fluctuation and abnormal pulsations.

There were similar findings in the left side.

Mouth opening was adequate, secretions from both parotid ducts were normal, and facial nerve function was intact on both sides.

A contrast-enhanced computed tomographic scan of the head and neck showed bilateral multiple enhancing lymph nodes in the postauricular region, the largest measuring 16 × 11 mm2 on the right side and 15.4 × 10.6 mm2 on the left, along with bilateral intraparotid nodes, the largest measuring 16.4 × 10.2 mm2 on the right and 15.3 × 10.7 mm2 on the left side.

His serum eosinophil count was slightly elevated, and his erythrocyte sedimentation rate was 15 mm/h.

He was prescribed oral antibiotics for 1 week but showed no improvement.

The results of both were consistent with reactive lymphadenitis.

He had undergone fine-needle aspiration cytology and incisional biopsy.

However, the IgE level was not measured.

He was then scheduled for excisional biopsy at our center.

The biopsy specimen included skin and subcutaneous tissue along with 2 underlying lymph nodes.Clinical photographs showing diffuse swelling of the preauricular, infra-auricular, and postauricular region.


The patient had been seen repeatedly for odynophagia that persisted despite multiple courses of antibiotics, reflux medications, and steroids prescribed at clinics and emergency departments.

All showed inconclusive results, and the mass progressively enlarged.

Further confounding the clinical picture, a pacemaker was required for multiple episodes of sinus asystole due to mass effect on the carotid body.

At presentation for the present report, physical examination showed a 2-cm, well-mucosalized, left oropharyngeal mass not extending to the hard palate (Figure, A).

Computed tomography (CT) revealed a 58-mm parapharyngeal mass lateralizing and narrowing the left carotid artery against the styloid process (Figure, B).

A CT-guided fine-needle aspiration biopsy was performed but again was nondiagnostic.

B, Left parapharyngeal mass measuring 4.3 × 2.7 × 5.8 cm and lateralizing the left carotid artery.

C, Fascicular, storiformed architecture with spindly, polygonal-shaped cells with eosinophilic cytoplasm and indistinct borders (original magnification ×10).

D, Positive findings for CD21 (left; original magnification ×10) and CD23 (right; original magnification ×20).

Over the course of 5 months, he underwent a tonsillectomy and 3 separate biopsies at multiple institutions.

To obtain the diagnosis of the parapharyngeal mass, the patient had a transcervical resection with transoral partial glossectomy, palatectomy, and pharyngectomy with reconstruction.

Results of immunohistochemical analysis of the removed tissue were positive for CD4 (weak), CD21, CD23, and vimentin with a low proliferation rate (Figure, C and D).A, A 2-cm well-mucosalized left oropharyngeal soft-palate mass anterior to the tonsillar bed with surrounding erythema.


Her history included bariatric gastric bypass surgery (3 years prior), hysterectomy for uterine leiomyomas (5 years prior), and pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension (7 years prior).

She had recently undergone ovarian stimulation for in-vitro fertilization.The patient’s physical examination showed a tender abdomen without rigidity.

Auscultation revealed no abnormal findings.

Results of routine blood testing were unremarkable.

Imaging studies suggested intussusception at the jejunojejunal anastomosis and a right adnexal mass; the intussusception was successfully reduced laparoscopically.

Multiple ruptured follicles were seen on the surface of the right ovary.


Two years ago, she immigrated to the United States from Bangladesh.

Five months ago, she visited Bangladesh to get married.

She reported hypoesthesia over the lesion.

A biopsy of the lesion obtained there showed multiple perineural granulomas with inflammatory cells.

Gram stain and acid-fast bacillary and fungal stains of the biopsy specimen were negative.

The patient was prescribed 1 dose each of doxycycline, ofloxacin, and rifampin and experienced no improvement.On physical examination there was a mildly erythematous macule located over the medial aspect of the right palm, with loss of touch and temperature sensation over the involved area (Figure).


He had no evidence of advanced liver disease (eg, thrombocytopenia).

Liver ultrasound showed no signs of cirrhosis, such as nodularity.

Vibration-controlled transient elastography (VCTE) performed prior to treatment was negative for signficant fibrosis at 6.7 kPa.

Laboratory studies and VCTE were performed (Table 1).The patient requires a liver biopsy to confirm fibrosis stage.

Hepatitis C was cured, based on viral load, with simeprevir and sofosbuvir for 12 weeks.


She had a non–ST-segment elevation myocardial infarction 1 year ago and was treated with a drug-eluting stent in her right coronary artery.

Additionally, the patient had generalized anxiety and panic disorders (treated with paroxetine, 30 mg), and she smoked for 90 pack-years but quit 2 months ago.

The patient’s home BP measurements were often lower than 120/80 mm Hg but sometimes reached 200/100 mm Hg.

Mean BP levels were 133/86 mm Hg overall (normal, <130/80 mm Hg); daytime, 135/88 mm Hg (normal, <135/85 mm Hg); and sleeping, 124/78 mm Hg (normal, <120/70 mm Hg).1 During the first sudden increase in BP (04:00-06:00; Figure), she awoke from sleep with a panic attack.

The patient’s office BP measurement was 127/74 mm Hg.

Her physical examination was unremarkable.Twenty-four-hour ambulatory BP monitoring was performed to evaluate patterns of elevated BP and potential etiologies.

Her antihypertensive medications included carvedilol (12.5 mg, twice daily), lisinopril (40 mg daily), and chlorthalidone (12.5 mg daily).

The patient kept a log of her diet, sleep pattern, and symptoms.

The second sudden increase (07:00-10:00) occurred after eating a high-sodium breakfast.The patient has normal BP since the office BP was normal.


There was no known history of travel in relation to onset of the lesions.

The eruption had gradually spread from the left palm to the right palm and then focally onto the bilateral volar aspect of the wrists in recent months (Figure, A and B).

Limited clinical examination revealed well-marginated brown atrophic patches on the bilateral palms extending focally onto the volar aspect of the wrist and centrally surmounted by spiny keratotic pits in the palmar creases.

She had not experienced involvement of the plantar surfaces.

No additional concerns were reported, and there was no family history of similar lesions.

Two punch biopsy specimens were obtained for diagnostic clarification (Figure, C and D).A, An atrophic hyperpigmented plaque with spiny papules in the palmar creases.

B, Similar features extending onto the volar wrist.

C and D, Histopathologic images (hematoxylin-eosin, original magnification ×100).


Her medical history included a cadaveric renal transplant 2 years earlier for end-stage renal failure.

Immunosuppressant medications included mycophenolate mofetil, 200 mg, twice daily and tacrolimus, 8 mg, twice daily.

Subsequently it spread to involve nose, chin, upper trunk, and extremities.

Physical examination revealed multiple 1-mm flesh-colored follicular papules and keratin spines against a diffuse erythematous background affecting the face (Figure, A and B) and upper trunk.

Her scalp and eyebrow hairs were unremarkable.

Results of skin biopsy from the right ear demonstrated dilatation and keratotic plugging of the hair infundibula with marked dystrophy and expansion of the inner root sheath.

The inner root sheath cells were enlarged with irregular trichohyaline granules and apoptotic cells with abrupt cornification without formation of a granular layer (Figure, C).

Immunohistochemical analysis for SV40 polyomavirus was positive (Figure, D).Photographs of (A) forehead and (B) nose depicting multiple follicular papules and keratin spines against a diffuse erythematous background.

C, Hematoxylin-eosin staining (original magnification ×40).

D, SV40 polyomavirus stain (original magnification ×40).


She could not recall an arthropod bite prior to the skin eruption and denied having traveled outside Spain in the past few years.

Such lesions had never appeared on the face, hands, or feet.

She did not relate these lesions to any particular hobby or application of any skin care product.

She complained about mild pruritus that had not responded to conventional antihistamine therapy or topical steroids.

She reported a 20-year history of the same annular plaques recurring on the trunk every single year by the beginning of summer and spontaneously resolving by the beginning of autumn.

Physical examination revealed a large, 35-cm semicircular erythematous plaque on her lower back with central clearing and a distinctive, well-demarcated papular border (Figure, A and B).

After obtaining informed consent, 2 biopsy specimens were taken from the papular border of the plaque for both hematoxylin-eosin stain and direct immunofluorescence (Figure, C).A, Large erythematous semicircular plaque with central postinflammatory hyperpigmentation on the lower back.

B, Absence of epidermal changes, mild edema of the papillary dermis, and a superficial sparse perivascular inflammatory infiltrate.

C, Inflammatory infiltrate composed mainly by lymphocytes and few neutrophils.

D, Follow-up visit showing clinical resolution in October.


He also reported a history of painful paresthesias at his feet beginning about 1 year prior.On examination, his visual acuity was initially 20/80 OD and 20/200 OS; it deteriorated over the next 2 months to finger counting bilaterally.

An echocardiogram showed a left ventricle ejection fraction of 20% to 25%, which had been 55% to 60% at the last test 2 years prior.

The Weber test gave a nonlateralizing result, and bone conduction hearing was absent bilaterally.

There was length-dependent loss of vibratory sensation and absent reflexes at both lower extremities.

Magnetic resonance imaging demonstrated abnormal enhancement of bilateral optic nerves (Figure, A).

Optical coherence tomography and fluorescein angiogram were normal.

Nerve conduction studies showed mild sensory axonal polyneuropathy.

Laboratory tests were notable for elevated thyrotropin (63 mlU/ml), low levels of free thyroxine (T4) (0.2 ng/dL), and elevated levels of cerebrospinal fluid protein (94 mg/dL).

Cerebrospinal fluid IgG index, oligoclonal bands, aquaporin 4 antibody, paraneoplastic panel, and protein electrophoresis test results were all normal.

No other lesions were seen in the brain.

B, Axial T-2–weighted magnetic resonance image of the right hip, demonstrating susceptibility artifact of orthopedic hardware, as well as soft tissue heterotopic ossification (corroborated by plain-film radiographs), consistent with pseudotumor of metallosis with decompression into the right hip joint space (blue asterisk), the subiliac bursa (red asterisk), greater trochanter bursa (yellow asterisk) extending into the gluteus maximus muscle (arrowheads), and into a potential space between the tensor fascia lata and the rectus femoris (black asterisk).Four months after initial presentation, the patient reported new symptoms of chest tightness and dyspnea on exertion.

At 5 months after initial presentation, the patient developed acute worsening of dyspnea and was admitted for respiratory distress.

He was treated empirically with high-dose steroids and plasmapheresis, with no improvement in symptoms.A, Axial postgadolinium, fat-suppressed, T-1–weighted magnetic resonance image of the orbits, demonstrating left greater than right enhancement of the optic nerves (arrowheads).

He was found to have nonischemic cardiomyopathy and died on hospital day 13.

The enhancement is centrally located within the optic nerves on coronal sections (not shown).


She denied any hematochezia or history of peptic ulcer disease.

She had no other surgical history and denied alcohol use or smoking.

She was locally tender to palpation in the right lower quadrant with rebound and voluntary guarding.

On examination, she was hypertensive (blood pressure, 185/85 mm Hg) although not tachycardic and was afebrile.

The patient had no abdominal scars, hernias, or palpable masses.

Laboratory results were significant for the following: leukocyte count, 23 600/µL (to convert to ×109/L, multiply by 0.001) with neutrophilia (94%); blood urea nitrogen level, 28 mg/dL (to convert to millimoles per liter, multiply by 0.357); creatinine concentration, 0.01 mg/dL (to convert to micromoles per liter, multiply by 88.4); lipase concentration, 195 U/L (to convert to microkatals per liter, multiply by 0.0167); and lactate concentration, 2.02 mg/dL (to convert to millimoles per liter, multiply by 0.111).

Electrolyte levels and liver function test results were within normal limits.

An electrocardiogram showed normal sinus rhythm.

Computed tomography of the abdomen and pelvis with oral and intravenous contrast enhancement was performed (Figure).Axial cross-sectional computed tomograms of the patient’s abdomen and pelvis with oral and intravenous contrast enhancement show segmental mural thickening of the anterior cecum with wall hypoenhancement and pneumatosis, adjacent fat stranding, and a lucency medial to the cecum (A) and bilateral renal hypodensities (B).


The abdominal pain was associated with diarrhea, nausea, and abdominal bloating.

On examination, the patient was afebrile and her abdomen was soft and nondistended without guarding or rebound but tender in the left lower quadrant.

The patient had a palpable nodule in the posterior vaginal fornix and fullness in the left lower quadrant of the abdomen on bimanual examination.Laboratory investigations had normal findings with the exception of an elevated cancer antigen 125 level of 158 U/mL (to convert to kilounits per liter, multiply by 1.0).

Contrast-enhanced computed tomography (CT) revealed multiple abnormal findings that included a bulky uterus with a thickened myometrium, endometriomas, right pleural effusion, pelvic peritonitis and ascites, enlarged lymph nodes, and solid extracolonic implants in the omentum caudal to the transverse colon (Figure, A) and in the cul-de-sac adjacent to the upper rectum (Figure, B).

Subsequent magnetic resonance imaging (MRI) showed 2 areas of focus corresponding with the extracolonic implants.


Associated symptoms included dysphagia and fever and chills.

A previous otolaryngology evaluation revealed an intranasal mass with enlargement of multiple submandibular lymph nodes.

A needle aspiration biopsy of 1 of the submandibular lymph nodes revealed atypical cells with mature lymphocytes.

Computed tomography of the sinus confirmed a nasal polyp and a soft tissue mass in the right lacrimal gland region.

Incisional biopsy of the lacrimal gland lesion revealed chronic inflammatory infiltrate composed of predominantly T cells with scattered B cells and mature plasma cells, but no evidence of a lymphoproliferative process.

On examination, visual acuity was 20/40 OD and 20/25 OS.

Intraocular pressure was unremarkable and ocular motility was intact in both eyes.

Clinical evaluation revealed a firm mass of 5 × 4 × 3 cm fixed to the superotemporal orbital rim in the right lacrimal gland region (Figure 1) with intact overlying skin.

Slitlamp examination was unremarkable in both eyes without evidence of conjunctival inflammatory signs.

Ophthalmoscopic examination was unremarkable in both eyes without compression of the optic nerve or disc edema.Color photograph depicting swelling over the right lacrimal gland region with intact epidermis and absence of inflammatory signs.

The patient was referred for evaluation in our clinic.Ocular history was noncontributory.


He was born full term with no complications during pregnancy or delivery.

The parents noticed asymmetry between the child’s eyes since birth and progressive right lower eyelid fullness.

On examination, the boy fussed when his left eye was covered, and his left eye was able to fixate and follow.

The right globe could not be visualized within the palpebral fissure because of a large compressible mass that transilluminated (Figure, A).

The remainder of the left eye examination findings were noncontributory.

Orbital magnetic resonance imaging was ordered (Figure, B).A, Clinical photograph of child at presentation displaying a right orbital cyst.

B, Coronal T2-weighted magnetic resonance image showing a large inferior right orbital cyst displacing the microphthalmic globe superiorly.

He was seen by his pediatrician, a general ophthalmologist, and a pediatric ophthalmologist before presenting to our institution.


Additional history revealed that the boy had handled a bloody raccoon carcass without gloves several days before initial presentation.

On transfer to our institution for failure to improve and concern for orbital cellulitis, results of examination by the ophthalmology service revealed a unilateral hemorrhagic conjunctivitis without orbital signs, and computed tomography showed mild preseptal edema.

The patient was discharged with a tentative diagnosis of viral conjunctivitis.Three days later, the patient returned with fever, prominent submandibular lymphadenopathy, and ipsilateral granulomatous conjunctivitis (Figure).

Results of the remainder of the anterior segment and dilated examination were unremarkable.

Results of laboratory tests revealed elevated inflammatory markers (C-reactive protein level, 9.47 mg/dL [to convert to nanomoles per liter, multiply by 9.524], and erythrocyte sedimentation rate, 48 mm/h), neutrophilic leukocytosis, and normocytic anemia.

He received oral trimethoprim-sulfamethoxazole and 2 days of intravenous clindamycin phosphate and ceftriaxone sodium.


His medical history was remarkable for asthma and prostate cancer, and review of systems was negative except for his visual loss.On examination, his best-corrected visual acuity was 20/400 OD and 20/20 OS.

He denied any recent trauma, flashes, floaters, pain, or any prior similar episodes.

Pupils were reactive with no relative afferent pupillary defect, and intraocular pressure measurements were unremarkable, as were results of the external and slitlamp examinations.

Dilated fundus examination of the right eye showed submacular hemorrhage with pigment epithelial detachment (Figure 1A).

Results of a dilated fundus examination of the left eye were unremarkable.

Optical coherence tomography (OCT) of the right eye (Figure 1B) revealed subretinal and sub–retinal pigment epithelium (RPE) hemorrhage; results were unremarkable in the left eye.A, Fundus photograph of the right eye shows macular hemorrhage.

B, Optical coherence tomography (OCT) scan of the right eye.

Green line indicates the position of the OCT scan.


On examination, his visual acuity was counting fingers at 3 ft OD and 20/20 OS.

A slitlamp examination of the right eye revealed conjunctival hyperemia, nongranulomatous keratic precipitates in the inferior half of the corneal endothelium, and 3+ cells in the anterior chamber, while results of a dilated fundus examination showed 1+ vitritis, 360° of optic nerve swelling, intraretinal hemorrhages predominantly along the distribution of the retinal arteries and arterioles, and perivascular sheathing, as well as macula involving retinal whitening, thickening, and subretinal fluid (Figure, A).

Fluorescein angiography revealed late vascular leakage with both arterial and venous involvement (Figure, B) and peripheral ischemia.

Examination of the left eye revealed no abnormalities.A, Wide-field photograph of the right eye demonstrates optic nerve edema (white arrowhead), scattered intraretinal hemorrhages (red arrowheads), perivascular sheathing (yellow arrowheads), and retinal whitening and thickening with subretinal fluid in the macula extending inferiorly (blue arrowheads).

B, Fluorescein angiograph shows arterial and venous vasculitis and peripheral ischemia.


She had persistent postoperative subretinal fluid inferonasally, and she was concerned about a central scotoma.

Her best-corrected visual acuity was 20/20 OD and 20/200 OS.

Extraocular motility was full, but she displayed left exotropia.

Confrontational visual fields were full for the right eye but showed a decreased field inferiorly for the left eye.

Intraocular pressures as determined using Tonopen tonometry were 13 mm Hg OD and 12 mm Hg OS.

A slitlamp examination revealed trace nuclear sclerosis and pigmented cells in the anterior vitreous of the left eye.

An ophthalmoscopic examination indicated laser and cryotherapy scars 360° around a horseshoe tear superotemporally in the left eye, whereas the right eye appeared normal.

In addition, pre-equatorial subretinal fluid in the left eye between the 6-o’clock and 8:30 position was associated with 2 tiny holes at the 7:30 position and with a small area of fibrotic tissue contraction consistent with early proliferative vitreoretinopathy.

The macula had yellow-white, flat, vitelliform-like lesions with a surrounding high watermark (Figure).

No cyst, exudate, or hemorrhage was detected in the macula.Color fundus photograph of the left eye demonstrating yellow-white vitelliform-like deposits in the fovea surrounded by a high watermark.

Three months earlier, she had undergone pneumatic retinopexy for macula-on retinal detachment (RD) with a superotemporal tear, followed by vitrectomy with gas injection for macula-on inferonasal detachment 1 month later.


The patient was afebrile, and vital signs were stable.

On examination, a 4.0 × 2.0-cm, oval-shaped violaceous to black plaque with a central hemorrhagic bulla and surrounding hyperemia was noted on the left lower back (Figure 1).

A complete blood cell count revealed a white blood cell count of 1800/μL (to convert to ×109/L, multiply by 0.001), with 2% neutrophils (absolute neutrophil count, 36/μL [to convert to ×109/L, multiply by 0.001]), hemoglobin level of 8.2 g/dL (to convert to grams per liter, multiply by 10), and platelet count of 10 × 103/μL (to convert to ×109/L, multiply by 1).

Her blood glucose level was 326 mg/dL (to convert to millimoles per liter, multiply by 0.0555).

The patient was administered broad-spectrum antibiotics empirically, and a dermatologist was consulted.

Two punch biopsy specimens from the lower back plaque were obtained and sent for histologic analysis and tissue culture (bacteria, atypical mycobacteria, and fungus).Violaceous to black plaque with central hemorrhagic bulla on the left lower back of the patient.


She reported gradual weight gain over the previous month despite diuretic and diet compliance, and outpatient laboratory testing demonstrated well-controlled international normalized ratios in the range of 3.0 to 3.5.Physical examination revealed jugular venous pressure of 13 cm H2O, crisp mechanical S1 heart sound, and pitting edema in the lower extremities.

Transthoracic echocardiogram showed no pericardial effusion, normal left ventricular systolic function, a well-seated mitral valve prosthesis with normal gradients, a dilated and diffusely hypokinetic right ventricle, and an elevated mean tricuspid valve gradient (Figure 1).Transthoracic echocardiography Doppler recordings shows an elevated mean tricuspid valve gradient.


He had no preceding head trauma or falls.

He had a history of atrial fibrillation with an annual stroke risk of 2.9% (based on points accrued for hypertension and age ≥65 years using the CHA2DS2-VASc score1).

He was being treated with rivaroxaban, 20 mg daily.

Other medications included ramipril and rosuvastatin.On physical examination, the patient’s blood pressure was 157/96 mm Hg, heart rate was 72/min, and Glasgow Coma Scale score was 14 (range, 3-15 with 15 indicating best neurological status).

Head computed tomogram (CT) showed a 2.5-cm left-sided acute subdural hematoma with mass effect.

Laboratory test results are shown in the Table.The patient’s hemostatic status is appropriate for surgery because aPTT is normal, which rules out anticoagulant effect from rivaroxaban.The patient’s hemostatic status is not appropriate for surgery because the abnormal PT suggests anticoagulant effect from rivaroxaban.The patient’s hemostatic status cannot be assessed because the thrombin clotting time is unknown.The patient’s hemostatic status cannot be assessed because the INR is unknown.

He required urgent surgical evacuation and the neurosurgical team requested advice regarding his perioperative bleeding risk prior to surgery.

He took his last dose 14 hours prior to presentation.


His medications were glipizide, metformin, metoprolol, and atorvastatin.

He had no history of fractures or osteoporosis.

He had no headache, palpitations, fatigue, muscle weakness, or weight change.

His blood pressure was 139/88 mm Hg, heart rate 92 beats/min, and body mass index (BMI; calculated as weight in kilograms divided by height in meters squared) 37.0.

Purple striae, proximal muscle weakness or supraclavicular fat pads were not present.

The mass was round and homogenous with a CT attenuation of 24 Hounsfield units (HU).

Laboratory evaluation was performed (Table).Perform an oral sodium load test for hyperaldosteronism.


She was not sexually active and had no medical history of sexually transmitted infections.

Medical history included lower urinary tract symptoms of 1-week duration for which she was prescribed cotrimoxazole with no improvement.

She also reported progressively worsening vulvar and buttock pain of 5 days’ duration.

Anogenital examination revealed vulvar (Figure 1A) and buttock ulcers (Figure 1B) tender to palpation.

Initial blood workup showed leukopenia with a white blood cell count of 2400 cells/μL (to convert to cells/L, multiply by 106), a differential of 72% neutrophils, and a hemoglobin level of 8.5 g/dL with a hematocrit differential of 27% (to convert hemoglobin to g/L, multiply by 10.0).

C, Hematoxylin-eosin stain (original magnification ×40).

Cotrimoxazole was discontinued on admission.

The patient was admitted to the intensive care unit 3 weeks prior to presentation for pneumonia and remained hospitalized for 10 days.

A biopsy specimen was obtained from the left vulvar ulcer (Figure 1C).A and B, Clinical photographs showing vulvar and buttock ulcers at the time of presentation.


Her medications included only an oral birth control pill.

On examination, on the cheeks and nasal bridge there were multiple erythematous, annular plaques with focal areas of atrophy.

In addition, the patient began to develop a burning sensation in these areas.

Serum chemical analyses and complete blood cell count showed no abnormalities, and anti-Ro, anti-La, and qualitative ANA antibodies were negative.

She had been previously treated with doxycycline, 100 mg/d, and topical sulfacetamide with no effect.

A clinical diagnosis was made, and over the next 9 months, treatments with minocycline, desonide, topical metronidazole, 0.75%, cream and azelaic acid, 15%, were unsuccessful, and the plaques developed more scarring and atrophy (Figure, A).

A punch biopsy specimen was obtained and submitted for histopathologic review (Figure, B and C).


There was no family history of autoimmune disease, and the child had not experienced any fevers or exposures to ticks or other arthropods.

The eruption would wax and wane, with each episode lasting for approximately 1 week, without residual pigmentation.

Clinical examination was significant for faint, nonscaling annular serpiginous, erythematous plaques with central clearing, and barely elevated borders.

Test results for erythrocyte sedimentation rate, complete blood cell count, antinuclear antibodies, anti-Ro/SSA and anti-La/SSB antibodies, and Borrelia burgdorferi antibodies were within normal limits.

A punch biopsy specimen from the left thigh was obtained, and histopathological analysis was subsequently performed (Figure, B and C).A, Annular serpiginous plaques with areas of central clearing on the patient’s right thigh and buttocks.

B, Perivascular and inflammatory infiltrate.

C, Perivascular and inflammatory infiltrate that includes eosinophils.

B and C, Histopathologic images (hematoxylin-eosin).


She reported, “I wash everything anyone else touches…I hardly let anyone into our home, and if I do, I wash down furniture and often have to wash the floors.” She avoided handling groceries, mail, and money and would not visit with family members owing to concerns that they were contaminated.She had experienced OCD symptoms since age 10 years but did not formally receive a diagnosis until 10 years later.

She reported mild hoarding symptoms but denied symptoms of body dysmorphic disorder, trichotillomania, excoriation disorder, or an eating disorder.

She reported a history of motor and phonic tics including eye blinking and blurting out words.

Her OCD symptoms consisted of concerns about dirt or germs and fears that she or others would get sick because of spreading these contaminants.

She had extensive rituals involving washing her hands repeatedly and cleaning household items.

Augmentation trials of antipsychotic medications, including risperidone, olanzapine, and aripiprazole, were either ineffective or intolerable.In addition to OCD, she experienced periods of depression but denied any history of mania, psychosis, trauma, or substance abuse.

Treatment with selective serotonin reuptake inhibitors (SSRIs) resulted in little benefit even at maximum doses (eg, 400 mg of fluvoxamine daily, 200 mg of sertraline daily).

A trial of clomipramine up to 250 mg daily also failed to reduce her OCD symptoms.


She denied weakness, difficulty swallowing, or breathing.

Over the subsequent weeks, she experienced worsening gait, falls, inattention, and short-term memory loss.Her brain magnetic resonance imaging (MRI) with contrast results were unremarkable.

On initial examination, she had a normal mental status, no pupillary abnormalities, normal visual acuity, and full visual fields.

She had moderate exotropia, severe bilateral ptosis, impaired bilateral adduction and vertical ductions, and impaired convergence.

Her abduction and vertical oculocephalic reflexes were intact.

She had full strength and normal sensation and reflexes but had a slightly wide-based and unsteady gait.

The test results for acetylcholine receptor antibodies and the muscle-specific kinase antibody were negative.

Her electrodiagnostic test results with repetitive stimulation and single fiber electromyography were unremarkable and there was no evidence of neuropathy.

The test results for thyrotropin, thiamine level, Lyme antibodies, rapid plasma reagin, and the serum ganglioside antibody panel were unremarkable.

A cerebrospinal fluid (CSF) analysis revealed a nucleated cell count of 6/μL (to convert to ×109 liters, multiply by 0.001) with 88% lymphocytes (to convert to the proportion of 1.0, multiply by 0.01), a red blood cell count of 10/μL, a protein level of 0.087 g/dL (to convert to grams per liter, multiply by 10), and glucose level of 63 mg/dL (to convert to millimoles per liter, multiply by 0.0555).

Serum paraneoplastic antibody testing results were negative for anti-Hu, Ri, Yo, and voltage-gated potassium channel complex (VGKCC) antibodies, and the CSF had negative results for anti-VGKCC, voltage-gated calcium channel, Hu, Ri, Yo, and amphiphysin antibodies.

A repeated brain MRI 3 weeks later revealed an interval development of T2-weighted fluid-attenuated inversion recovery hyperintensities in the right thalamus, inferior temporal gyrus, and hippocampus (Figure).Repeated brain magnetic resonance imaging 3 weeks after the patient’s initial presentation: T2-weighted fluid-attenuated inversion recovery hyperintensities (arrowheads) in the right inferior temporal gyrus (A) and right thalamus (B).


Follow-up ultrasonography at 32 weeks’ gestation identified fetal hydrops.

The fetal ultrasonogram showed a large left-sided hydrothorax, mediastinal shift, and ascites.

Results of follow-up ultrasonography on postoperative day 3 showed improvement in the fetal hydrothorax and resolution of hydrops and identified a feeding vessel originating from the descending aorta supplying a lung mass (Figure 1A).A, Feeding vessel (arrowhead) from descending aorta identified on ultrasonography at 32 weeks’ gestation.

B, Feeding vessel (arrowhead) to lung mass (outlined) over left hemidiaphragm on computed tomography at age 9 months.Owing to premature rupture of membranes, the mother delivered at 33 weeks.

On the day of birth, radiographic findings showed a left-sided pleural effusion.

During the following weeks, the thoracostomy tube output decreased, the tube was removed, and the patient was discharged home.

At 9 months of age, follow-up computed tomography confirmed a lung mass with a feeding vessel (Figure 1B), and the patient returned for elective thoracoscopic resection.

After ruling out infectious causes, an ultrasonography-guided thoracoamniotic shunt was placed successfully at 32 weeks’ gestation.

The neonate showed no respiratory distress after birth, and the shunt was removed.

Ongoing pleural effusion caused respiratory distress, and on day 3 of life, a temporary thoracostomy tube was placed.


He had a medical history of basal cell carcinoma of his left cheek and brow, both removed several years previously.

His medical history also included type 2 diabetes with diabetic neuropathy.Slitlamp examination revealed bilateral meibomian gland dysfunction with upper and lower eyelid laxity, scurf, and collarettes.

The patient complained of 6 months of worsening tearing and crusting of both eyes that was worse on the left.

The patient tried this treatment, but his symptoms did not improve.

Three weeks before the evaluation, he was seen by his primary ophthalmologist, who epilated 2 misdirected eyelashes of the patient’s left lower eyelid and recommended eyelid scrubs and artificial tears.

His ocular history was significant for cataracts, proliferative diabetic retinopathy, and macular degeneration.


The patient reported blurry vision in both eyes for 1 week.Visual acuity was 20/100 OD and 20/50 OS.

Physical examination was notable for a genital vesicular rash; a culture of the skin lesion yielded herpes simplex virus (HSV).

Blood cultures were positive for Candida glabrata, triggering an ophthalmology consultation to rule out ocular candidiasis.

Intraocular pressures, pupillary responses, and results of a portable slitlamp examination were normal.

Dilated fundus examination revealed bilateral optic nerve edema with disc hemorrhages in the right eye (Figure, A).

On extended ophthalmoscopy, subtle areas of granular retinal whitening were noted in the peripheral retina.

Fluorescein angiography exhibited disc hyperfluorescence (Figure, A [inset]) and late peripheral staining in both eyes.

Optical coherence tomography demonstrated thickening of the papillary nerve fiber layer (Figure, B).

Automated perimetry demonstrated bilateral enlargement of the physiological blind spot and bilateral arcuate scotomas, greater in the right eye.A, Right fundus image shows optic nerve edema with disc hemorrhages and peripheral retinal whitening (arrowhead).


His medical history was significant for obesity and a recent 45-kg intentional weight loss.

Visual acuity on presentation was 20/20 OD and 20/30 OS with full-color vision plate testing in both eyes and no relative afferent pupillary defect.

He reported ptosis and marked proptosis of his left eye for 3 weeks before presentation.

He denied any signs of diplopia, change in vision, or systemic symptoms, including difficulty breathing, swallowing, or articulating speech.

By Hertel exophthalmometry, he had 2 mm of left-sided proptosis and hypoglobus, with right-sided eyelid retraction (Figure, A).

Margin-to-reflex distance 1 in the left eye was 1.0 mm, worsening to −2 mm on prolonged eyelid opening, with subjective fatigue.

On extraocular muscle (EOM) testing, the patient exhibited −2 restriction in upgaze of the left eye, with double vision in supraduction.

Slitlamp examination showed no significant anterior segment findings, and examination of the patient’s left optic nerve demonstrated no optociliary shunt vessels, edema, pallor, or hemorrhage.

A computed tomography (CT) scan of the orbits was obtained (Figure, B), which showed enlargement of the left orbital fat compartments but no evidence of left orbital mass or EOM enlargement.A, The patient shows hypoglobus and ptosis in the left eye, with left lower eyelid and right upper eyelid retraction.

B, Noncontrast axial computed tomography (CT) scan of the orbits shows mild proptosis in the left eye, absence of retro-orbital mass lesion or extraocular muscle enlargement, and mild fat compartment expansion.


The pregnancy was full-term and uncomplicated, and he was delivered spontaneously without the use of forceps.

His corneal abnormalities were noted by the neonatologists on the first day of life.

A systemic survey revealed no other dysmorphic findings.

Examination under anesthesia revealed bilateral corneal opacities with peripheral clearing and absent red reflexes.

The white-to-white corneal diameters were 10 mm horizontally and vertically in both eyes, and B-scan ultrasonography revealed unremarkable posterior segments.

Ultrasound biomicroscopy confirmed the diagnosis of Peters anomaly type I with iridocorneal adhesions and no apparent lens involvement.

At the same session, a large iridectomy with lysis of iridocorneal adhesions was performed in both eyes to establish a clear, noncentral optical axis.Shallowing of the anterior chamber was observed immediately after the iridectomy.

However, the patient’s corneal opacity precluded an accurate assessment of the chamber depth and stability (Figure 1).A temporal iridectomy was present, but the anterior chamber depth could not be assessed accurately owing to corneal opacity.Inflate the anterior chamber with ophthalmic viscosurgical device.Tilt the microscope for better visualization of the anterior chamber.

He was referred to the clinic for further evaluation and management.


The patient reported taking 400 mg of hydroxychloroquine sulfate daily (6.3 mg/kg/d) during the past 2 years, with normal renal function, but denied any recent change in medications.On initial examination, visual acuity (VA) was counting fingers at 2 ft OD and at 3 ft OS.

The patient had initially seen a general ophthalmologist 1 week after noticing a change in vision and was diagnosed as having bilateral cataracts.

Given a continued decline in vision over the next 2 weeks, the patient sought a second opinion.

Intraocular pressures were 14 mm Hg OD and 16 mm Hg OS.

Pupils were equal and mildly sluggish to react to light but demonstrated no relative afferent pupillary defect.

Slitlamp examination revealed 1+ nuclear sclerosis cataracts.

Dilated fundus examination demonstrated normal-appearing retinal and optic nerve architecture with the exception of mild arteriolar narrowing bilaterally (Figure 1A).

Results of optical coherence tomography (OCT) of the retinal nerve fiber layer and macula, as well as fundus autofluorescence, were normal (Figure 1B).

In addition, magnetic resonance imaging (MRI) of the brain and orbits with and without gadolinium were unremarkable.A, Baseline color fundus photograph of the right eye demonstrates normal optic nerve color and contour, with mild arteriolar narrowing.


She was asymptomatic except for 2 years of progressive, painless, left-sided proptosis.

Her physical examination findings were unremarkable except for resistance to retropulsion of the left globe and 5 mm of left-sided proptosis by Hertel exophthalmometry.

Magnetic resonance imaging (MRI) revealed a superonasal orbital mass that was T1 isointense to brain parenchyma and heterogeneously T2 hyperintense (Figure 1A), with a small cystic component and avid peripheral contrast enhancement (Figure 1B).

Bony remodeling without destruction was noted, as well as displacement of the superior oblique and superior rectus muscles.


The patient reported vague pain around the left ankle but no functional limitations and no history of trauma at that site.

A magnetic resonance imaging (MRI) scan showed an inhomogeneous nodule in the subcutaneous layer with a largest diameter of approximately 5 cm.

No enlarged lymph nodes were detectable in the groin.

Results of a complete blood cell count and levels of alkaline phosphatase, lactic dehydrogenase, and C-reactive protein were unremarkable.

The patient then underwent disease staging with computed tomography (CT) of the chest and whole-body fluorodeoxyglucose positron emission tomography (FDG-PET).

The CT findings were negative for secondary lesions; the FDG-PET scan demonstrated, as expected, high uptake in the right thigh (maximum standard uptake variable [SUVmax], 8.4) at the site of the subcutaneous tumor and at a second lesion in the left ankle joint (SUVmax, 12.0).

A radiograph of the ankle did not show calcifications within the soft-tissue lesion.

An MRI scan of the ankle showed a 1.5-cm soft tissue mass in the posterior recess of the ankle joint with a low to intermediate signal in T1- and T2-weighted sequences and moderate contrast enhancement throughout the lesion (Figure 1).Magnetic resonance imaging fast spin echo of the left ankle showing the soft-tissue mass in the posterior recess.

The sagittal T1-weighted image shows an isointense, bean-shaped mass (arrowhead) (1.5 × 0.7 cm) (A), whereas the sagittal T2-weighted image shows the isointense signal of the mass (arrowhead) (B).

An ultrasonography-guided core needle biopsy was performed, and histopathologic evaluation showed the aspects of a small round-cell tumor, which was confirmed to be a Ewing sarcoma by immunohistochemical and molecular analysis (with positivity for the EWS-FLI1 fusion gene [OMIM 133450]).


His medical history included transcatheter aortic valve replacement (TAVR) 12 months earlier for symptomatic aortic stenosis with an uncomplicated placement of a balloon-expandable bioprosthetic valve.

He also had a history of nonobstructive coronary artery disease, for which he was treated with atorvastatin and metoprolol.On presentation, he denied having chest pain, orthopnea, and lower extremity edema.

He was asymptomatic following TAVR until the recurrence of dyspnea 3 weeks before admission.

His blood pressure was 121/47 mm Hg, his pulse was regular at 83 beats per minute, and he was afebrile.

His physical examination revealed a jugular venous pressure of 9 cm H2O, no stigmata of endocarditis, and an absence of peripheral edema.

There were faint bibasilar rales and a harsh grade III/VI, late-peaking systolic murmur along the left sternal border that radiated to the carotids.

Laboratory data included a negative troponin, NT-proB natriuretic peptide of 55 pg/mL.

Serum chemistry levels and complete blood cell counts were otherwise normal.A TTE result showed an interval development of bioprosthetic aortic stenosis with mean gradient of 29 mm Hg.

He was discharged and prescribed aspirin at 81 mg and clopidogrel at 75mg daily.


She was a former smoker and had unilateral conductive hearing loss associated with the mass.

On examination, a large, nonmobile, and firm tumor extended from the postauricular region to the parotid gland, scalp, and a portion of the neck.

On otoscopy, the growth occluded view of the tympanic membrane.

Computed tomographic imaging (Figure, A and B) demonstrated a mass emanating from the parapharyngeal space, measuring approximately 10 × 7.3 × 1.5 cm.

There was no radiographic evidence of osseous extension or destruction.

The patient underwent a staged procedure, including a radical resection of the scalp and neck mass, followed by a transcervical and transparotid approach to the parapharyngeal space.

The scattered tumor cells were spindled to stellate shaped and had the morphology of myofibroblasts.

The tumor was noted to stretch the pes anserinus to 4 times its normal length and had to be resected off the internal carotid artery, as well as the fascia overlying the medial pterygoid muscle and the tympanomastoid suture.

The patient recovered well with only temporary marginal mandibular weakness.

Microscopy (Figure, C) demonstrated a well-circumscribed, paucicellular spindle cell neoplasm with abundant myxocollagenous matrix.


Despite taking omeprazole (20 mg once daily) 30 minutes before breakfast, avoiding foods that precipitated symptoms, and sleeping with his head elevated, heartburn and regurgitation while supine continued.

He reported intermittent solid-food dysphagia for 2 years but no weight loss (254-lb [115 kg] body weight); physical examination results were unremarkable.Esophagogastroduodenoscopy (EGD) revealed normal esophageal mucosa and normal biopsies.

Given his persisting symptoms, an esophageal high-resolution manometry (HRM) study was performed (Table 1; eFigure in the Supplement).


The child was born via vaginal delivery at full term.

Pregnancy was complicated by gestational diabetes.

The child had no major medical issues and met all developmental milestones.

There was no relevant family medical history.

The lesion had developed acutely over 48 hours, starting as symmetric pink patches leading to pustules, erosions, and ulceration.

Associated symptoms included generalized fatigue, but no fevers, chills, or other systemic symptoms.

Dermatology was consulted prior to scheduled debridement and skin grafting.Physical examination revealed a well-appearing infant with normal vital signs.

Extending from the gluteal cleft to just medial to the scrotum, there was a symmetric, well-defined ulcer with a cribriform base, central necrosis, and rolled, pink-to-violaceous, undermined border (Figure, A).

Initial laboratory workup was significant for leukocytosis (22 000/mm3) and thrombocytosis (764 000/mm3).

Serum zinc and alkaline phosphatase were within normal limits.

A punch biopsy of the edge of the ulcer was obtained and stained with hematoxylin-eosin (Figure, B and C).

Tissue cultures for anaerobic and/or aerobic, mycobacterial, and fungal organisms were all negative, though wound cultures grew Escherichia coli, Klebsiella oxytoca, and Enterococcus faecalis.

Viral cultures were negative.A, Clinical photograph shows a symmetric, well-defined ulcer with a cribriform base, central necrosis, and rolled, pink-to-violaceous, undermined border extending from the gluteal cleft to just inferior and medial to the scrotum.

B, Histopathology demonstrating an ulcer with surrounding mild epidermal hyperplasia and a neutrophil predominant perivascular and interstitial infiltrate.

C, Histopathology demonstrating an ulcer with surrounding mild epidermal hyperplasia and a neutrophil predominant perivascular and interstitial infiltrate.

Broad-spectrum intravenous antibiotics were initiated on admission.

The child’s parents denied any new topical exposures or medications.


The dermatosis was characterized by polymorphic erythematous macules and plaques with elevated and indurated borders without evanescence to pressure (Figure, A and B).

Dysesthesia was recorded on her lesions.

Results from complete blood cell count, erythrocyte sedimentation rate, and blood chemistry analyses were normal, as was a thyroid profile.

A punch biopsy specimen from a trunk plaque was obtained, and histopathological analysis was performed (Figure, C and D).A, Polymorphic erythematous macules and plaques with elevated and indurated borders without in vitro pressure evanescence were noticed during consultation on patient’s back.

B, Polymorphic erythematous macules and plaques with elevated and indurated borders without in vitro pressure evanescence on the trunk and abdomen were present.

C, Histopathological analysis revealed a chronic dermic granulomatous process, as well as (D) a chronic dermic nonencapsulated granulomatous infiltrate with a perineural distribution formed by mature epitheloid cells and encapsulated by lymphocytes.

Previous treatment included antihistamines and oral corticosteroids for several months without improvement.

She was referred from another clinic with the presumptive diagnosis of urticarial vasculitis.


The patient reported that these asymptomatic lesions were present since birth and did not appear to change on sun exposure or with seasons.

She had no family history of similar cutaneous feature.Physical examination revealed that these lesions were distributed in a Blaschkoid pattern.

On close inspection, numerous reddish punctate keratotic papules were observed on the patient’s right forearm that coalesced into plaques on the dorsum of her right hand (Figure 1).

No extracutaneous abnormalities were found.

A skin biopsy specimen from the patient’s right arm was obtained.Reddish linear keratotic papules and plaques on the right forearm and dorsal hand distributed in a Blaschkoid pattern.


Medical history was unremarkable with no recent weight loss, travel, or trauma.

Visual acuity without correction was 20/20 OU.

The right pupil was 3.5 mm and briskly reactive, while the left pupil was 8.5 mm and unreactive to light or near effort; there was no relative afferent pupillary defect.

Results of testing with the use of an Amsler grid and Ishihara color plates were within normal limits.

Visual fields were full to confrontation.

The optic nerves and retina were normal in both eyes.

External examination was notable for left periorbital swelling, proptosis, and moderate ptosis of the upper eyelid (Figure 1A).

Extraocular motility examination results were normal in the right eye but demonstrated severe ophthalmoparesis of the left eye: 0% supraduction (Figure 1B), 20% of normal abduction/adduction, and 10% of normal infraduction (not pictured).

Corneal reflex was normal in the right eye but diminished in the left eye, and facial strength was normal bilaterally.

The remainder of the neurologic examination results were normal.An 8-year-old boy presented with 2 weeks of painless proptosis of the left eye (A) accompanied by upper eyelid ptosis and ophthalmoplegia of the left eye (B).


With fever, change in mental status, and a recent cardiac procedure, there was concern for infective endocarditis and septic emboli and he was transferred to our facility.On examination, the patient was drowsy with transient eye opening and significant expressive aphasia with mild receptive aphasia; he intermittently followed commands.

Laboratory studies were significant for elevated white blood cell count of 9500/μL (to convert to 109 per liter, multiply by 0.001).

Cerebrospinal fluid revealed white blood cell count 0 cells/μL, red blood cell count 28 cells/μL, glucose level 76 mg/dL, and protein level 48 mg/dL.

Magnetic resonance imaging showed left-hemisphere watershed areas of diffusion restriction.

Examination revealed right-sided facial droop, right hemiparesis, and an up-going right toe.

A second magnetic resonance image revealed worsening in both hemispheres with perivenular and leptomeningeal enhancement, areas of cortical diffusion restriction not typical of ischemic injury, and significant T2 changes (Figure 1A).A, Magnetic resonance imaging findings.

B, Pneumocephalus on head computed tomography (CT), highlighted by white arrowheads.

C, Sagittal computed tomography imaging of the chest with contrast.Transesophageal echocardiogram (TEE) showed no vegetation.

Left-heart catheterization demonstrated nonocclusive coronary artery disease.

Blood cultures grew Streptococcus mitis.

While in the supine position for nursing care, he became abruptly hypotensive, bradycardic, hypoxic/cyanotic, and nonresponsive; an electrocardiogram demonstrated inferior ST-segment elevations.


The patient had no significant medical story.

The patient noted that tumefaction was more prominent in the morning on waking, and he denied any associated fever, weight loss, or other symptoms.

Physical examination showed a painless but tender right scrotal tumefaction in an apparently healthy obese man.

No inguinal lymphadenopathy was observed.

Swelling was not reducible, the right testicle was not palpable, and the transillumination test was nondiagnostic.

Laboratory evaluation demonstrated a hemoglobin level of 13.5 g/dL (to convert to grams per liter, multiply by 10.0), leukocyte or white blood cell count of 11 350/μL (to convert to ×109 per liter, multiply by 0.001), and lactate dehydrogenase level of 240 U/L (to convert to microkatal per liter, multiply by 0.0167).

Unenhanced computed tomography of the abdomen and pelvis was performed (Figure 1).Computed tomographic images.

Axial (A) and coronal (B) reconstruction.


She had a history of migraine headaches, for which she took nonsteroidal anti-inflammatory drugs for symptomatic relief.

At her examination, she reported feeling tired but denied other medical problems.On examination, her visual acuity was 20/20 OU.

Intraocular pressure was 15 mm Hg OU, extraocular motility was normal, and findings of the anterior segment examination were normal.

There was no afferent pupillary defect, and dilated fundus examination revealed healthy-appearing optic nerves and normal retinas.

Automated perimetry demonstrated a right-sided homonymous hemianopia.


She had no other symptoms and was otherwise healthy with no medical history of any disease or surgeries.

Her family history was also unremarkable.The patient’s best-corrected visual acuity was 20/20 OU, results of pupillary examination were normal without relative afferent pupillary defect, and results of the rest of the anterior segment examination were unremarkable.

Posterior segment examination of the right eye revealed a slightly elevated, white, multicystic lesion approximately 3 disc diameters that was located inferior to the optic nerve (Figure 1).

Results of examination of the left eye were unremarkable.


She had recently traveled to Ireland, where she rode a horse.

She denied previous upper respiratory and gastrointestinal tract infections.

She denied any eye pain with ocular movement or positive visual phenomena during presentation.

She had no other abnormal neurologic signs or symptoms.

Best-corrected visual acuity was 20/400 OD and 20/20 OS.

Results of slitlamp examination were normal.

A relative afferent pupillary defect 1+ was noted in the right eye.

Dilated fundus examination showed no evidence of vitritis, optic nerve swelling, or retinal pigment epithelial abnormalities.

Automated visual field testing showed a central scotoma in the right eye, and the visual evoked potential showed a delayed P100 response in the right eye.

Results of an initial ophthalmologic workup, including fundus photography and fluorescence angiography, were considered to be unremarkable.

Contrast-enhanced magnetic resonance imaging of the brain and orbit revealed no enhancement of the optic nerves.

At a subsequent visit, 2 small glistening lesions in the retina were identified, and optical coherence tomography (OCT) showed an attenuated ellipsoid zone at a position nasal to the fovea (Figure 1).Fundus photography shows 2 small glistening lesions (white arrowheads) (A).


She was not taking any ophthalmic or neurotoxic medications such as ethambutol hydrochloride.

Family history revealed vision loss in a maternal cousin that was attributed to optic neuritis from multiple sclerosis.

Symptom onset was approximately 3 months prior to presentation when the patient began reporting difficulty reading.

Magnetic resonance imaging findings of the orbits were normal.At presentation 3 months after onset of decreased vision, the patient reported that her vision continued to worsen.

She was initially evaluated by her local eye care professional and noted to have visual acuity of 20/100 OD and 20/25 OS with cecocentral visual field defect in the right eye.

Results of ophthalmoscopic examination of the optic nerves at this time revealed mild elevation of the optic nerves.

Best corrected visual acuity was measured at 20/400 OD and 20/40 OS.

Both optic nerves were found to be mildly hyperemic with a few telangiectatic vessels on the left and trace temporal pallor on the right.

Color vision was 3/13 in the right eye and normal in the left.

There was no relative afferent pupillary defect.

The left eye had a very similar pattern.A, Fundus photograph of the right eye shows mild hyperemia of the optic nerve with trace temporal pallor.

B, 24-2 Humphrey visual field shows a cecocentral scotoma.The patient had no significant medical history.

The patient’s 24-2 Humphrey visual field in the right eye is shown in the Figure.


He had traveled from Gabon to the United States 2 weeks earlier.

He had no significant medical history except use of correctional lens due to myopia; his body mass index was normal.

He had worked in Gabon as an engineer for the past 10 months and reported numerous insect bites, but denied other exposures.

He was up to date on required vaccinations and had received doxycycline for the past 10 months for malaria prophylaxis.

The patient had no symptoms on presentation and denied fevers, headaches, or visual disturbances.

Examination of the fundus showed bilateral elevation of the optic nerve head with blurring of the disc margins (Figure); an ocular ultrasonography showed no optic nerve drusen.

A magnetic resonance image of the brain with and without gadolinium was normal, and a magnetic resonance venogram showed no evidence of venous sinus thrombosis.

A lumbar puncture demonstrated elevated intracranial pressure (ICP) of 35 mm Hg; white cells, 1/μL (to convert to ×109/L, multiply by 0.001); protein, 0.02 g/dL (to convert to g/dL, multiply byt 10.0); glucose, 55 mg/dL (to convert to mmol/L, multiply by 0.0555); and a negative gram stain.Optic disc edema with a circumferential halo (arrowhead).


Physical examination showed short stature (height in the third percentile) with mild muscle wasting in both lower extremities.

He had an esotropia of 30 prism diopters with mild bilateral inferior oblique muscle overaction.

External examination showed hypertelorism, bilateral upper eyelid ptosis, and a broad nasal bridge.

Slitlamp biomicroscopy disclosed bilateral posterior embryotoxon, iris stromal hypoplasia, and translucent strands extending from the inferior segment of the iris toward the cornea in both eyes, with right corectopia (Figure, A).

Intraocular pressures (IOPs) were 11 mm Hg OD and 10 mm Hg OS.

Situs inversus of the optic discs and a cup-disc ratio of 0.2 in both eyes was noted.

Results of the retinal examination were otherwise normal.

SITA (Swedish Interactive Thresholding Algorithm) 24-2 visual field revealed superior arcuate defects in both eyes.

Optical coherence tomography (OCT) showed mild peripapillary retinal nerve fiber layer thinning inferiorly in both eyes.

Magnetic resonance imaging of the brain disclosed dysgenesis of the left cerebellar hemisphere and vermis (Figure, B).

Family history and examination of other family members disclosed no similar abnormalities.A, Slitlamp biomicroscopy showing posterior embryotoxon, peripheral iris strands (arrowhead), and corectopia of the right eye.

B, Axial T2-weighted magnetic resonance image showing dysgenesis of the left cerebellar hemisphere (arrowhead) and vermis (the unpaired median portion of the cerebellum that interconnects the 2 hemispheres [asterisk]).


The patient was initially diagnosed with MDS 3 years ago and treated with azacitidine followed by CLAG (cladribine, cytarabine, granulocyte-colony stimulating factor).

His physical examination on this presentation showed multiple dark brown cutaneous papules measuring at 3.0 to 5.0 cm (Figure, A), but the rest of the examination results were negative for hepatosplenomegaly or lymphadenopathy.

Posttreatment bone marrow biopsy findings at that time showed no evidence of disease.

Complete blood cell count showed a white blood cell count of 3.3 × 109/L with absolute neutrophil count of 1.0 × 109/L, Hb 12.7g/dL, platelet counts 118 × 109/L and differential counts showed no evidence of circulating blasts.

In addition, results of laboratory workup including peripheral blood culture, methicillin-resistant Staphylococcus aureus (MRSA) swab, human immunodeficiency virus test, and flow cytometry performed on his peripheral blood were all negative.

Pathologic sampling from the skin lesion showed the presence of monomorphous small to medium cells extending through the dermis and into the subcutaneous fat (Figure, B).

Immunohistochemical staining demonstrated CD4-, CD33-, CD43-, CD56-, CD123-, and TCL1-positive neoplastic cells with Ki-67 of 85%, but staining results for TdT, MUM1, CD3, CD20, Bcl-6, CyclinD1, and CD8 were negative (Figure, C).

Results of bone marrow biopsy showed normocellular marrow maturing trilineage hematopoiesis with no increase of blasts or other abnormal populations.

Cytogenetics results were abnormal, showing del(20)(q11.2q13.3)[8]/46,XY[12].A, Skin image showing a single, brownish nodule and adjacent scar from prior biopsy.

B, A section of cutaneous blastic plasmacytoid dendritic cell neoplasm with subepidermic confluent atypical cellular infiltrate composed of medium sized, round to oval, or elongated immature precursors with finely granular to open chromatin, inconspicuous to prominent nucleoli and a small amount of cytoplasm (hematoxylin-eosin stain, original magnification ×600).

C, CD56 (immunoperoxidase stain, original magnification ×600).


She had 3 previous pregnancies (2 live births and 1 spontaneous abortion); her last child was born more than 5 years ago.

Her chest pain began spontaneously 1 hour prior to presentation and was described as retrosternal with radiation to her upper and middle back.On physical examination, her blood pressure was 128/88 mm Hg, her pulse was regular at 96 beats per minute, her respiratory rate was 16 breaths per minute, and her temperature was 36.9° C. Pulse oximetry was 100% in room air.

Pertinent physical findings included normal central venous pressures, clear lung fields, normal first and second heart sounds, an audible fourth heart sound, and no murmurs.

The electrocardiogram (Figure 1A) demonstrated anterior ST-segment elevations consistent with an anterior acute current of injury.

Results from the chest radiography were normal.

Initial troponin I concentration was 0.02 ng/mL but rose to 177.76 ng/mL over the ensuing 4 hours (the patient’s chest pain had resolved by this time).

Thrombolysis in myocardial infarction flow grade 2 was observed in the left anterior descending artery and second diagonal branch.A, Initial 12-lead electrocardiogram on presentation showing ST-segment elevation on anterior leads.

Urgent coronary angiography was performed (Figure 1B).


The patient’s parents denied any associated fevers, chills, night sweats, sore throat, abdominal pain, or weight changes.

Medical history was noncontributory.Physical examination showed bilateral, mobile, nontender cervical lymphadenopathy without erythema or swelling.

Tonsils were symmetric and not enlarged.

There was no noticeable thyromegaly or nodules.

Laboratory testing was significant for an elevated erythrocyte sedimentation rate (37 mmol/h).

Ultrasonography demonstrated cervical lymphadenopathy bilaterally with the largest node measuring 3.8 cm in maximum dimension.

Lymph node needle core biopsy demonstrated increased histiocytes and numerous plasma cells on tissue section (Figure, A).

Scattered neutrophils and rare giant cells were also present.

Findings of Grocott methenamine silver, acid-fast, and Warthin-Starry stains were negative.

The CD-68 immunostain showed numerous histiocytes.

The specimen stained positive for CD-30 and S-100 immunostains showing emperipolesis (Figure, B and C).

Lymphoid markers by flow cytometry showed a polyclonal mixed B-cell and T-cell population.Lymph node needle core biopsy specimens from the patient.


He had no history of underlying medical diseases, such as hypertension, diabetes, hepatitis, and tuberculosis.

On physical examination, a whitish, firm, painless, nodular mass on the superficial layer of the left upper lip was observed (Figure, A).

On histopathologic examination, an encapsulated salivary gland tumor with epithelial and myoepithelial cells, ductlike structures, and mixed stroma was observed (Figure, B).

Given the possibility of a tumorous lesion, complete excision was planned under local anesthesia.

In surgery, the 1.5 × 1.2 cm, whitish, firm, nodular, and well-encapsulated mass was excised completely without rupture of the capsule from the surrounding mucosa.

One month after surgery, the surgical wound on the left upper lip was clean and well healed.

One year later, there had been no recurrence, and the patient had no symptoms at the left upper lip.A, Clinical image of the left upper lip.

B, Histopathologic image (hematoxylin-eosin, original magnification ×10) showing areas of ductal epithelial components with myxoid stroma.


Physical examination revealed a single erythematous scaling plaque involving the left lower eyelid, including its border.

Eyelashes were totally absent (Figure 1A).

No other lesions were found after complete physical examination.

Dermoscopy showed an erythematous background, telangiectatic vessels, whitish structureless areas, and whitish scales (Figure 1B).

A 4-mm punch biopsy specimen was obtained (Figure 1C).A, Erythematous scaling plaque involving the left lower eyelid.

B, Dermoscopy shows erythematous background, telangiectatic vessels, structureless whitish areas, and white scales.

C, Histopathological analysis (hematoxylin-eosin).


No history of local trauma, prolonged standing, and occupational exposure to mineral dust or recent drug intake was recollected.

The lesions started to appear on the left pretibial region as small papules, which gradually increased in size and coalesced to form bigger plaques.

There were no lesions elsewhere on the body.

On examination, large, ill-defined, erythematous plaques were present on the pretibial areas extending onto the lateral aspect of both legs.

The plaques were surmounted by multiple, discrete atrophic scars.

The periphery of the plaques showed increased vascularity in the form of telangiectasias and venous prominences, along with atrophy of the overlying skin (Figure, A).

The periphery of the plaques shows telangiectasia.

B, Skin biopsy showing unremarkable epidermis and multiple epithelioid cell granulomas in the entire dermis.

C, Compact, noncaseating, epithelioid cell granulomas with multiple Langhans giant cells.

D, Photograph after 6 weeks of treatment showing considerable decrease in erythema and induration.

A skin biopsy sample was obtained from the plaque and sent for histopathological examination (Figure, B and C).A, Clinical photograph on presentation showing ill-defined, erythematous plaques surmounted by multiple atrophic scars.


Approximately 15 years earlier, he had sustained injuries from an accidental land mine explosion that had lacerated his face, neck, and thorax in several places.

The wounds apparently healed without difficulty, leaving only scars of expected severity.

Approximately 6 months before presentation, the patient began to notice an increase in the size of these lesions.

Physical examination showed multiple firm, erythematous, nontender papules, nodules, and small plaques on his face, his chin, the anterior portion of his neck, and the anterior thorax (Figure 1).Multiple firm, erythematous, nontender nodules and small plaques on the patient’s face.


On examination, the patient reported moderate pain in the abdomen and right side and demonstrated a distended abdomen in the absence of any peritoneal signs.

Computed tomography (CT) showed a right-sided grade IV renal injury involving his known renal cyst with active arterial extravasation (Figure, A).

The patient underwent selective angioembolization of the renal laceration.A, Abdominal computed tomography (CT) with contrast demonstrating grade IV laceration of the right kidney and active extravasation.

B, Abdominal CT obtained on day 2 of hospitalization showing large right-sided retroperitoneal hematoma and grade IV renal laceration displacing the patient’s abdominal viscera to the left, with contrast-filled structure medial to the liver.In the 24 hours after embolization, the patient required 2 U of red blood cells via transfusion for anemia.

Contrast-enhanced CT of the abdomen and pelvis was performed to assess for a source of continued bleeding (Figure, B).


He had been diagnosed as having type 2 diabetes for 10 years.

Fundus examination showed venous dilatation, retinal hemorrhages, hard exudates, and serous macular detachment (arrowheads) (Figure 1).

Optical coherence tomography scans showed intraretinal fluid accumulation with serous macular detachment in both eyes.

Blood analysis revealed a low hemoglobin level of 8 g/dL, red blood cell count of 3.3 × 106/μL, white blood cell count of 3460/μL (15% neutrophils, 84% lymphocytes, and 1% monocytes), fasting blood glucose level of 120 mg/dL, normal lipid profile, serum urea nitrogen level of 45 mg/dL, and an increased serum creatinine level of 1.4 mg/dL (to convert hemoglobin to grams per liter, multiply by 10.0; red blood cell count to ×1012 per liter, multiply by 1.0; white blood cell count to ×109 per liter, multiply by 0.001; blood glucose to millimoles per liter, multiply by 0.0555; serum urea nitrogen to millimoles per liter, multiply by 0.357; and serum creatinine to micromoles per liter, multiply by 88.4).A and B, Fundus photographs showing retinal venous dilatation, hemorrhages, hard exudates, and serous macular detachment (arrowheads).

He received 3 intravitreal bevacizumab injections at 4 weekly intervals and 2 intravitreal triamcinalone acetonide injections in both eyes, with minimal resolution of edema.

He was diagnosed as having nonproliferative diabetic retinopathy with macular edema in both eyes.


Her medical history was notable for cystic fibrosis, receipt of bilateral lung transplant, cytomegalovirus (CMV) viremia, and chronic kidney disease.

The findings of a workup for meningitis were unremarkable.

Ophthalmic consultation occurred 1 week after admission.The patient’s visual acuity was 20/30 OD and 20/25 OS.

Pupils were minimally reactive and without an afferent pupillary defect.

Intraocular pressures were 15 mm Hg OD and 12 mm Hg OS.

Portable slitlamp examination findings included bilateral posterior synechiae and a shallow anterior chamber in the left eye.

No anterior segment cell was appreciable by portable examination.

The patient had early nuclear sclerosis and posterior subcapsular cataracts in both eyes.

Results of a fundus examination demonstrated bilateral temporal choroidal elevations without vitritis, vascular sheathing, retinitis, or optic nerve abnormalities.

Earlier treatment for CMV viremia included valganciclovir hydrochloride therapy that was discontinued because of the development of drug resistance and foscarnet sodium therapy with resultant renal dysfunction.

The patient was subsequently treated with intravenous renally dosed cidofovir therapy; however, she again developed a nephrotoxic reaction and required dosage reduction in the weeks before presentation.

At admission, cidofovir therapy was discontinued because of acute kidney injury (creatinine level, 5.75 mg/dL) (to convert to micromoles per liter, multiply by 88.4).


Her medical history was remarkable for type 1 diabetes diagnosed at 16 years of age and proliferative diabetic retinopathy in both eyes that had been treated with panretinal photocoagulation 7 years earlier.

She had undergone pars plana vitrectomy with endolaser to treat a tractional retinal detachment in her right eye 2 years before this presentation.

She also had a history of hypertension and chronic kidney disease, and she was 15 weeks into pregnancy.

The patient was in no apparent distress and denied any headache, chest pain, or focal weakness.Ophthalmoscopic examination (Figure) revealed mild optic nerve head edema that was greater in the left eye than the right eye with associated nerve fiber layer hemorrhage in the left eye.

Visual acuity was 20/50 OD and 20/100 OS.

Intraocular pressure was normal bilaterally, and no relative afferent pupillary defect was detected.

Findings of an anterior segment examination were normal.

Nerve fiber layer infarctions, dot and blot hemorrhages, and lesions caused by panretinal photocoagulation also were seen bilaterally.

Optical coherence tomography showed macular edema that involved the center of the macula in both eyes (Figure, inset).Fundus photographs obtained at presentation that show bilateral peripapillary hemorrhages and mild optic nerve head edema.