His comorbidities included congestive heart failure, atrial fibrillation, coronary artery disease, chronic obstructive pulmonary disease, hypertension, and a 50-pack-year history of tobacco abuse.

The mass covered the globe completely, resulting in total vision obstruction (Figure, A).

However, the patient’s vision remained intact when the mass was elevated off the eye with great effort.

Physical examination revealed a firm, nonmobile, nontender, bulging mass situated over the right frontal sinus, which expanded to the upper right eyelid.

His right pupil was round and reactive to light without afferent pupillary defect.

A non–contrast-enhanced computed tomography (CT) scan demonstrated a large, right supraorbital mass with bony destruction of the anterior table of the right frontal sinus (Figure, B).

It extended into the superior aspect of the orbit, displacing the right globe inferiorly.

Air was seen within the mass (Figure, C).

After medical clearance, the patient was taken to the operating room for a wedge excisional biopsy with decompression of the right supraorbital mass under monitored anesthesia care.

Histopathological investigation revealed large nests of neoplastic epithelioid cells surrounded by fibrotic tissue and necroinflammatory debris.

The neoplastic cells contained round nuclei with single prominent nucleoli and abundant clear to vacuolated cytoplasm (Figure, D).A, Enlarged right supraorbital mass.

B, Computed tomography (CT) scan of destruction of the anterior table of right frontal sinus.

A fine-needle aspiration biopsy yielded inconclusive results.


The patient had spent his childhood in eastern Africa, where he swam in Lake Tanganyika.

He also reported asthenia, sleep disorders, and urinary frequency.

The urinary frequency has been treated for 2 years with many drugs (tamsulosin, desmopressin, and solifenacin) without improvement.

He could not walk properly owing to his vertigo.

The ear, nose, and throat examination revealed a right horizontal nystagmus and a poorly delineated hypoesthesia of the left pinna.

The rest of the clinical examination findings were unremarkable.

Additional examinations (ie, pure-tone audiometry, video nystagmography, tympanometry) revealed left profound hearing loss with an air-bone gap of 40 dB and central vertigo.

Otoacoustic emissions were absent on the left side.

Auditory brainstem responses showed asymmetric interpeak latencies of waves I through V and III through V, suggesting slower conduction on the left side.

Computed tomography and cerebral magnetic resonance imaging (MRI) revealed a 24 × 20-mm hemorrhagic lesion in the left middle cerebellar peduncle associated with a thickening of the cochleovestibular nerve (Figure).

The patient was referred to urology, where a 13 × 8-mm mass was discovered in the lateral wall of the prostate gland.

Biopsies of prostate and brain lesions, blood tests, and urinalysis were performed.Computed tomography (CT) scan and magnetic resonance images (MRIs) of the left middle cerebellopontine angle and prostate gland.

A, Axial CT scan showed a hyperdense lesion suggesting hemorrhage in the left middle cerebellar peduncle.

B, The lesion extended to the base of the vestibulocochlear nerve that was thickened (arrowheads) on the Fast Imaging Employing Steady-State Acquisition (FIESTA) MRI (axial plane).

C, Coronal postcontrast MRI demonstrated a T1 hyperintense lesion that was consistent with hemorrhagic lesion with no true enhancement.

His history did not reveal trauma, infection, or any other associated conditions at the onset of the complaints.


His medical history was unremarkable, as were the rest of the mucocutaneous and systemic examination findings.

On examination, there were multiple, erythematous papules coalescing to form a plaque with areas of scarring over the posterior aspect of right ear, along with firm, nontender posterior cervical lymphadenopathy (Figure, A).

There was no history of trauma or intervention prior to the onset of lesions.

A skin biopsy from the plaque was submitted for histopathological examination (Figure, C and D).A, Erythematous papules coalescing to form a plaque with areas of scarring, along with ipsilateral posterior cervical lymphadenopathy.

C, Microphotograph showing unremarkable epidermis with evidence of granulomatous inflammation and central caseous necrosis in the dermis (original magnification ×100).

D, Ziehl-Neelsen stain shows acid-fast bacilli (oil immersion; original magnification ×1000).

B, The lesions have healed with atrophic scarring after 6 weeks of treatment.


He had a history of pseudogout and kidney transplant 13 years ago.

He was taking sirolimus and prednisone, with excellent graft function.

He also reported 3 days of right knee pain and swelling.

He had nuchal rigidity and positive Kernig sign.

His temperature on presentation was 102°F (38.9°C).

There were no focal neurologic deficits and no spinal tenderness.

He had a right knee effusion without erythema and with full range of motion.

Laboratory workup of serum showed a white blood cell (WBC) count of 10 200/μL (neutrophils, 63.8%; lymphocytes, 19.6%; monocytes, 15.1%) and erythrocyte sedimentation rate, 63 mm/h; workup of cerebrospinal fluid showed a WBC count of 0/μL; red blood cell count, 32/μL; total protein level, 109 mg/dL; and glucose level, 59 mg/dL (3.3 mmol/L).

Magnetic resonance imaging (MRI) of the spine showed cervical degenerative changes with reactive marrow (Figure 1).

Blood and cerebrospinal fluid (CSF) cultures were negative.

He was prescribed intravenous vancomycin and cefepime.


Given her erythrocyte sedimentation rate (ESR) of 110 mm/h and symptoms concerning for giant cell arteritis (GCA), a temporal artery biopsy was obtained.

Laboratory results are shown in the Table.

Results showed a normal caliber lumen without infiltrating inflammatory cells.


The patient denied fevers, chills, weight loss, night sweats, cough, lymphadenopathy, abdominal pain, change in bowel habits, or other systemic symptoms.

Skin examination was significant for multiple erythematous and flesh-colored papules and nodules symmetrically affecting the upper and lower lips with extension onto the vermilion border (Figure 1A).

There were no intraoral lesions, and all other findings of his examination were within normal limits.

A biopsy was obtained of the vermilion lip (Figure 1B).A, Symmetric lip enlargement with infiltrative erythematous and skin-colored papules and nodules extending onto the vermilion border.

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


Then, owing to knee pain, a plain radiograph was obtained which demonstrated osteosclerosis of the distal femur.

Computed tomographic (CT) scan of the chest, abdomen, and pelvis demonstrated extensive infiltrative stranding from the thorax through the retroperitoneal space.

Sclerotic changes in multiple osseous structures were noted.

Biopsy of the lower-eyelid yellow plaque returned xanthelasma (Figure, A).

A second retroperitoneal biopsy exhibited fibroadipose tissue, foamy macrophages, and chronic inflammation with fibrosis.

The clinicopathologic presentation was confirmed with a molecular test.A, Clinical image of a soft, nonscaly yellow-tan plaque on the medial canthus.

B, Hematoxylin-eosin stain image demonstrating foamy histiocytes with Touton giant cells (original magnification ×100).

C, CD68 immunohistochemical stain diffusely positive in the histiocytic infiltrate (original magnification ×100).

D, Computed tomographic image with contrast demonstrating retroperitoneal stranding and fibrosis with hydronephrosis (arrowheads indicate hairy kidney appearance of fibrosis).

Prednisone and azathioprine were started.

Azathioprine and prednisone were restarted.

Idiopathic retroperitoneal fibrosis was diagnosed following biopsies.

Ureteral obstruction, chronic kidney disease, and lymphedema complicated her course.

Prednisone was slowly tapered but she relapsed 3 years later.

She required multiple surgeries for spinal stenosis and ureteral obstruction.

Her kidney function continued declining.

Consequently, the previous eyelid and retroperitoneal biopsies were reviewed.


He had emigrated from Japan more than 10 years earlier but had no history of recent travel.

He took no medications, had no significant medical or surgical history, and denied personal and family history of atopy.

While he worked as a biomedical researcher, he denied any caustic exposures.

On physical examination, his face, trunk, upper extremities, palms, and soles were marked by 1- to 2-mm erythematous papules and pustules coalescing into large, partially indurated and polycyclic plaques with centrifugal extension and occasional central clearing (Figure, A and B).

Punch biopsies of lesions on the shoulder (Figure, C) and foot (Figure, D) were performed.A, Annular erythematous plaque studded with papules and pustules on the cheek and temple.

B, Erythematous plaques studded with papules and pustules on the shoulder.

C and D, Lesional specimens under hematoxylin-eosin stain taken from the shoulder (C, original magnification ×400) and plantar foot (D, original magnification ×40).

Treatment with potent topical corticosteroids was ineffective.


She admitted to having developed new intermittent vertigo and more frequent and severe throbbing migraine with transient blurry vision and nonpulsatile tinnitus several months prior.

She had recently developed hearing loss with a robotic quality of auditory perception.

A neurologic examination revealed diminished hearing to a finger rub on the right side.

Fundoscopy showed several segmental retinal arterial plaques, with the right side worse than the left.

A brain magnetic resonance image (MRI) with gadolinium revealed multiple white-matter lesions that were hyperintense on diffusion-weighted imaging (DWI), including some with enhancement, as well as a small DWI-negative lesion in the left thalamus and corpus callosum that was hyperintense on T2/fluid-attenuated inversion recovery magnetic resonance imaging (FLAIR; Figure 1).

An MRI of the spine with gadolinium did not reveal a signal abnormality in the spinal cord.

A lumbar puncture revealed a white blood cell count of 8 cells/μL (normal range, 0-5 cells/μL; to convert to cells × 109/L, multiply by 0.001) with 78% lymphocytes and 22% monocytes (to calculate these as proportions of 1.0, multiply by 0.01), and a protein level of 0.067 g/dL (normal range, 0.015-0.045 g/dL; to convert to grams per liter, multiply by 10).

Cerebrospinal fluid (CSF) glucose and IgG index test results were normal, with culture and oligoclonal bands negative.

An audiogram revealed bilateral sensorineural hearing loss, with the right side worse than the left.

Infectious, rheumatologic, and hypercoaguable test results were negative.

Retinal fluorescein angiography was obtained.A, Pink arrowheads indicate hyperintensities in the right centrum semiovale.


Her medical history included migraine and polycystic ovaries.

The patient was taking oral contraceptives and denied the use of recreational drugs.

She reported a sudden onset of photopsia and visual disturbances described as “fixed swirl circles” in the central vision of both eyes noticed on awakening 5 days prior and persisting since that time.

Ocular movements were full but the patient reported pain and discomfort during movements of the right eye.

Pupils were equal and reactive to light and there was no afferent pupillary defect.

Best-corrected visual acuity was 20/20 OU; color vision was 17/17 OU on Ishishara plates.

The anterior segment was unremarkable and intraocular pressure was 14 mm Hg in both eyes.

Dilated fundus examination revealed a healthy optic disc and no obvious abnormalities at the macula of both eyes.

Near-infrared reflectance imaging and spectral-domain optical coherence tomography (OCT) scans of the right eye are shown in the Figure.A, Near-infrared reflectance imaging of acute macular neuroretinopathy in the right eye at presentation reveals hyporeflective teardrop-shaped lesions (black arrowheads).

B, Simultaneous spectral-domain optical coherence tomography scan passing through the acute macular neuroretinopathy lesions in the right eye reveals increased reflectivity of the outer plexiform layer (yellow arrowheads) and attenuation of the ellipsoid zone (white arrowheads).

She had a flulike episode 1 week prior to the onset of symptoms.


He had no relevant medical, ocular, or family history, and he denied use of illicit drugs or anticoagulants.Uncorrected visual acuity was 20/30 OD and 20/400 OS.

His ocular symptoms began 2 days prior when he noticed dark scotomas in his central field and decreased visual acuity.

Extraocular motility and intraocular pressures were normal.

Results of an anterior examination was unremarkable.

Dilated funduscopic examination of the left eye revealed a pink optic nerve with sharp margins, diffuse intraretinal hemorrhages and Roth spots extending from the posterior pole to the far periphery, and preretinal hemorrhage overlying the central macula (Figure, A).

Spectral-domain optical coherence tomography through the preretinal macular hemorrhage demonstrated layered blood in the sub–internal limiting membrane space (Figure, B).

The right fundus exhibited a similar appearance aside from the preretinal hemorrhage.A, Bedside indirect ophthalmoscopy (slightly out of focus) of the left eye with a 20-diopter lens reveals diffuse retinal hemorrhages; the white arrowhead indicates hemorrhage corresponding to Figure, B and the blue arrowheads, Roth spots in the posterior pole with a normal-appearing optic nerve.

There was no recent trauma or strenuous physical activity.


His ocular history was notable for occupational exposure to sunlight and occasional smoking.

There was no personal or family history of cancer, and he denied ocular surgery or trauma.

On examination, his best-corrected visual acuity was 20/20 OD/OS, and intraocular pressures were 15 mm Hg OD and 18 mm Hg OS.

A slitlamp examination of the right eye showed no conjunctival pigmentation.

Examination of the left eye revealed a pigmented lesion at the temporal limbus from 3 to 4 o’clock with a 2-mm extension into the cornea (Figure 1A).

Some opalescence underlied the pigment on the cornea.

In addition, there was an adjacent small area of flat, superficial, mobile bulbar conjunctival pigmentation.

The anterior segment high-resolution optical coherence tomography (HR-OCT) of the 4-o’clock corneolimbal lesion of the left eye demonstrated thickened hyperreflective corneal epithelium with an abrupt transition from normal to abnormal (Figure 1B).A, Slitlamp photography of the left eye shows a pigmented limbal lesion at 3 to 4 o’clock; another small area of bulbar conjunctival pigmentation is noted (arrowhead).

B, High-resolution optical coherence tomography of the left eye through the area of the pigmented conjunctival lesion (inset with red line in area of scan), showing epithelial hyperreflectivity and thickening with an abrupt change from normal to mildly thickened epithelium (arrowhead).

The referring physician suspected primary acquired melanosis (PAM).


He had endorsed proper contact lens care and hygiene.

He also noted an upper respiratory tract illness 4 weeks before presentation.

He did not report any redness or discharge but did note mildly increased irritation mainly from soft contact lens wear.

Visual acuity was 20/20 OU with normal intraocular pressure.

Several giant follicles were noted on the right inferior palpebral conjunctiva, and a few smaller follicles were noted in the left inferior fornix.

Papillae were also noted bilaterally on the superior palpebral conjunctiva.

Because of the papillary reaction, prednisolone acetate, 1%, twice a day for 2 weeks was prescribed.After 3 months, the symptoms had improved in the left eye, but conjunctival follicles were still present in the right inferior palpebral conjunctiva (Figure 1).

During this follow-up, Lyme disease and Epstein-Barr virus serologic tests were performed in addition to repeated chlamydia testing.

Initially, he received a giant papillary conjunctivitis diagnosis because of soft contact lens intolerance and a recommendation to take a contact lens break.

The patient subsequently underwent testing for chlamydia and gonorrhea, and the result was negative.

At the 1-year follow-up, the examination findings were unchanged.


He had no history of ocular trauma, drug use, or familial ocular diseases.The anterior segment of both eyes was normal on slitlamp examination, but the fundus examination of the right eye showed multiple refractile small crystals scattered on the posterior pole (Figure 1A).

He denied photopsia or floaters and did not note any visual field defects.

His best-corrected visual acuity was 20/25 OD and 20/20 OS.

Isolated crystals were also present in the vitreous core.

Optical coherence tomography of the macula revealed the crystals to be located preretinally on the surface of the inner limiting membrane with sporadic crystals within the cortical vitreous (Figure 1B).A, Fundus photography at presentation shows multiple refractile crystals at the posterior pole (black arrowheads).

B, The corresponding optic coherence tomography at presentation shows these crystals adhering to the internal limiting membrane and floating in the vitreous (white arrowheads).Apart from this, biomicroscopy, fluorescein angiography, and optical coherence tomography of the posterior pole showed no abnormalities.


He reported owning cats.On presentation, the patient’s blood pressure was 210/150 mm Hg, and he had a history of acute kidney failure and non–ST-elevation myocardial infarction.

Two weeks prior to presentation, he started noticing black spots in his peripheral vision and progressive blurring of vision in both eyes.

The patient denied recent travel, cough, gastrointestinal or genitourinary tract symptoms, ulcers, aching joints, tinnitus, or transient visual obscurations.

On ophthalmic examination, his uncorrected near visual acuity was 20/25 OD and 20/30 OS.

Extraocular motility, Ishihara color plates, intraocular pressure, pupils, and results of slitlamp examination were within normal limits.

Ophthalmoscopy showed Frisen grade 5 optic nerve head swelling in both eyes with extensive flame hemorrhages and nerve fiber layer infarcts surrounding both nerves and retinal exudates extending from the nerves temporally into both fovea’s (Figure 1).

The vessels were of normal course and caliber.

There were scattered peripheral dot-and-blot hemorrhages.

Results of Humphrey visual field testing were reliable and showed bilateral concentric constriction and enlarged blind spots.Grade 5 optic nerve head edema.

A, Fundus photograph of the right eye shows Frisen grade 5 optic disc edema, flame hemorrhages, and peripapillary exudation into the macula.


On examination, she had a florid, erythematous macular eruption over the trunk and limbs (Figure, A) but no hepatosplenomegaly or lymphadenopathy.

In addition to the high hemoglobin level, mild lymphocytosis (absolute lymphocyte count, 6.2 × 109/L, range 1.5-4.0 × 109/L) was noted with the lymphocyte morphology, suggesting reactive changes.

Skin biopsy specimen (Figure, B) showed a normal epidermis with a pericapillary infiltrate of small lymphocytes restricted to the dermis and no leukocytoclastic vasculitis, fungal organisms, or dermal mucin.

The absence of cellular atypia and epidermal involvement suggested a diagnosis of lupus, or gyrate or annular erythema, and the need for clinicopathological correlation.A, Photograph shows erythroderma.

Skin biopsy was repeated 2 months after the original procedure owing to a suboptimal clinical response, but the histological appearances were unchanged.

The hemoglobin level remained high, and the lymphocyte count had increased to 9.0 × 109/L (Figure, C).

C, Blood smear shows a typical lymphocyte (May-Grünwald Giemsa stain, original magnification ×400).Treatment began with a topical emollient, steroid creams, and oral antihistamines.

B, Skin biopsy specimen shows a pericapillary infiltrate of small lymphoid cells restricted to the dermis (hematoxylin-eosin, original magnification ×200).


Laboratory test results were notable for high serum concentrations of IgG4 (5650 mg/dL; to convert to grams per liter, multiply by 0.01) and C-reactive protein (78.1 mg/L; to convert to nanomoles per liter, multiply by 9.524).

Coronary computed tomographic angiography was performed (Figure, A and B).

It revealed a large aneurysm (90 mm × 48 mm) with a thrombosis in the left anterior descending artery (LAD) and a perivascular soft-tissue mass involving the patent proximal right coronary artery (RCA) and left circumflex artery, which showed homogeneous moderate enhancement on contrast-enhanced images.

Coronary computed tomographic angiography with cinematic rendering (Figure, C) showed multiple mass lesions around the RCA and LAD.

Invasive coronary angiography results were unable to visualize the LAD but did identify a hypervascular mass in the proximal RCA.A, Contrast-enhanced axial computed tomographic image revealing perivascular soft-tissue masses involving the left circumflex and right coronary arteries (arrowheads).

B, Maximum intensity projection reformatted image of coronary computed tomographic angiography revealing perivascular soft-tissue masses involving the left circumflex and right coronary arteries (arrowheads) and a large aneurysm with a thrombus involving the left anterior descending artery (asterisk).

C, A 3-dimensional rendering demonstrated mass lesions involving the left anterior descending and right coronary arteries (arrowheads).


He denied recent injury, fever, chills, odynophagia, or difficulty breathing.

Laboratory examination was significant for leukocytosis (white blood cell count, 15 700/μL) and hypercalcemia (total calcium level, 13.1 mg/dL) (to convert white blood cells to ×109 per liter, multiply by 0.001; to convert calcium to millimoles per liter, multiply by 0.25).

Physical examination was notable for a 4- to 5-cm pedunculated, ulcerative mass on the dorsal oral aspect of the tongue near midline, anterior to the foramen cecum, without palpable cervical lymphadenopathy.

Contrast-enhanced computed tomography (CT) demonstrated a rim-enhancing, centrally hypodense lesion in the right dorsal tongue abutting the lingual septum with a somewhat ill-defined border (Figure 1); there was no associated osseous erosion of the mandible or hard palate, no tongue prolapse to suggest hypoglossal nerve palsy, no involvement of the soft palate, and no cervical lymphadenopathy by radiological size criteria.

An excisional biopsy of the anterior aspect of the mass was performed at bedside, and the patient was discharged home with instructions to follow up in the otolaryngology clinic.A, Sagittal contrast-enhanced computed tomography (CT) demonstrates an exophytic mass on the dorsal aspect of the tongue.

B, Axial contrast-enhanced CT demonstrates a peripherally enhancing mass of the oral tongue, to the right of midline.


She had previously been treated with antibiotics and mupirocin nasal irrigation, with temporary improvement.

The patient and her identical twin sister had required multiple surgical procedures in the past for osseous lesions in the upper and lower jaws, including an aggressive resection via bilateral Caldwell-Luc approach 2 years previously; a computed tomographic scan from that initial presentation is shown in the Figure, A.

At the current presentation, examination using flexible nasal endoscopy revealed bulging of the left lateral nasal wall; imaging revealed a 14-mm, dense, expansile, sclerotic mass with moderate calcifications obstructing the left nasolacrimal duct; a similar 2.3 × 1.8–cm lesion of the right mandibular body; and evidence of previous sinus surgery (Figure, B).

Histologic findings are shown in the Figure, C and D.A, Contrast-enhanced computed tomographic image at initial presentation.

B, Non–contrast-enhanced computed tomographic image at 2 years after bilateral Caldwell-Luc resection showing mass in left nasolacrimal duct.

C, Photomicrograph depicting a proliferation of highly cellular, fibrous connective tissue (arrow) and cementum-like hard tissue (arrowhead) (hematoxylin-eosin, original magnification ×100).

D, Photomicrograph showing whorls of spindle cells (arrow) with relatively acellular calcified masses of cemento-osseous material (arrowhead) (hematoxylin-eosin, original magnification ×200).

Because of these findings, left-sided endoscopic dacryocystorhinostomy and sinus surgery was performed.


Her social history was significant for a 45–pack-year smoking history and social drinking.

She denied associated pain, bleeding, or recent trauma to the lip.

She also denied any other constitutional symptoms.

On physical examination, the patient had right upper lip submucosal masses without any tenderness or mucosal abnormality.

The remainder of the otolaryngic examination was unremarkable.

A computed tomographic scan of the neck with contrast demonstrated multiple hyperenhancing nodules; the largest was 3 × 7 × 6 mm, with no local bony erosion or cervical lymphadenopathy (Figure 1).Computed tomographic (CT) scans of hyperenhancing nodules of the upper lip (red arrows).


The patient, a construction demolition worker, was otherwise healthy and took no medications.

He had 1 lifetime female sexual partner with whom he used barrier contraception.

The number of lesions and degree of pain had alternately increased and decreased over the years.

Occasionally the lesions would rupture and leak clear fluid.

Physical examination revealed an asymmetric cluster of lesions on the right flank measuring 14 × 12 cm at the widest margins (Figure 1).

The cluster contained verrucous papules and plaques in the center and vesicles filled with clear and milky-white fluid in the periphery.

Crust was partially covering some of the lesions.

The surrounding skin was erythematous without warmth or induration.

The remainder of the physical examination was normal except for stretch marks on the lateral abdomen.Left, Vesicular and verrucous skin lesions on patient’s right flank.

One year prior, he was hospitalized for a “skin infection” on his right flank and treated with intravenous antibiotics.


She awakened with severe lower back pain, abdominal pain, nausea, and vomiting.

She came to the emergency department with 10/10 abdominal pain but no fever, chills, vaginal bleeding, or rupture of membranes.

Thirty-six hours after her initial presentation, she still had rebound tenderness in the left side of the abdomen.

Her abdominal examination revealed diffuse tenderness and guarding.

She was afebrile, blood pressure was 104/77 mm Hg, and heart rate was 152/min.

White blood cell count was 10.7 ×109/L; anion gap, 13 mEq/L; and lactate dehydrogenase level, 131.7 U/L (2.2 μkat/L).

A computed tomography (CT) scan of the abdomen and pelvis with oral and intravenous contrast revealed mesenteric vascular compromise (Figure 1); after the scan, the patient had hematemesis.

She was initially diagnosed as having preterm labor and was given antibiotics, steroids, an epidural catheter for pain management, and intravenous fentanyl as needed.

She delivered a healthy baby without complications, but her severe abdominal pain, nausea, and vomiting persisted.


He had a distant history of viral myocarditis and was taking lisinopril, carvedilol, furosemide, aspirin, and a multivitamin.

Twelve years prior, he underwent uncomplicated Roux-en-Y gastric bypass (RYGB) surgery.

Review of systems was notable for fatigue and negative for fever, night sweats, weight loss, dyspnea, or abnormal bleeding.

Examination revealed temperature of 36.7°C; heart rate, 91/min; blood pressure, 120/70 mm Hg; and body mass index, 20.1 (calculated as weight in kilograms divided by height in meters squared).

He was well-appearing with conjunctival pallor but no petechiae, no hepatosplenomegaly, and a normal neurologic examination.Laboratory evaluation showed a white blood cell count of 1.7 × 103/μL with an absolute neutrophil count of 0.17 × 103/μL; hemoglobin level, 7 g/dL with mean corpuscular volume of 103 fL (reticulocyte index, 0.94); and platelet count, 116 × 103/μL.

Results of a complete blood cell count from 8 months prior were normal.

Results of serum protein electrophoresis were normal, as were levels of lactate dehydrogenase, ferritin, folate, vitamin B12, and thyroid-stimulating hormone.

A computed tomography (CT) scan of the abdomen showed no lymphadenopathy, masses, or hepatosplenomegaly.

Peripheral blood smear showed occasional pseudo Pelger-Huet cells and rare teardrop cells.

Histopathologic examination of bone marrow aspirate revealed a normocellular marrow and dyserythropoiesis with vacuolized and dysplastic erythroid precursors (Figure, panel A), left-shifted granulocytic maturation with vacuolized granulocytic precursors, ring sideroblasts (Figure, panel B), and no increase in blasts.

No new medications had been started in the past year.


She reported being vaccinated against varicella zoster virus and was not immunocompromised.A, A clinical photograph shows an annular erythematous plaque with crusts, bullae, and central pallor.

According to the patient, the lesions recurrently appeared during the last 9 years, with a symptom-free period of 2 years (Figure, A).

She could not identify or recall a specific cause for these skin lesions and denied self-inflicting them.

B, Histopathologic specimen shows normal stratum corneum, confluent necrosis of the upper epidermal layers, some multinucleated keratinocytes in the adjacent epidermis, and papillary edema with a subepidermal blister.

A moderately dense perivascular lymphocytic infiltrate with eosinophilic and neutrophilic granulocytes is present in the upper dermis (hematoxylin-eosin, original magnification x200).A skin biopsy of the forearm (Figure, B), direct immunofluorescence, and blood samples were obtained.

She had been treated with antibiotics (cefuroxime) as well as with topical steroids (dexamethasone) but denied any lasting effect.

Her family history was without abnormalities.


The lesions had started about 6 months previously as recurrent crops of pruritic erythematous papules that subsided leaving behind brownish-bluish black hyperpigmentation after each episode.On examination, there were multiple, brown, hyperpigmented patches symmetrically affecting her neck, axillae, antecubital fossa, and intermammary and inframammary areas (Figure, A).

The patches showed a reticulate pattern at the peripheries.

Intermammary patches had central erythema and a few subsiding papules.

Additionally, there were dark brown to bluish-gray patches with reticulation on the lower back and dorsum of the neck (Figure, B).

These patches also had a few resolving erythematous papules in the periphery.

Her IgE levels and thyroid status were normal.

Her fasting blood glucose and basal insulin levels were not raised.

There was no family history of similar lesions.

The rest of the skin, nails, and mucosae were all normal.

Potassium hydroxide mount analysis from skin scrapings did not reveal fungal hyphae or spores.

The patches show a reticulate pattern at the peripheries.

The intermammary patches have central erythema and a few overlying edematous papules.

B, Bluish-gray pigmented patches with prominent reticulation on lower back.

C, Histopathologic lesional specimen shows mild epidermal hyperkeratosis, acanthosis, multiple foci of spongiosis, and an enhanced basal layer pigmentation.

There is scant lichenoid and moderate perivascular lymphomononuclear and polymorphonuclear infiltrate, basal cell vacuolization, and melanin incontinence (hematoxylin-eosin).

There was no history suggestive of allergic rhinitis or bronchial asthma.

A skin biopsy was performed from lesions on the lower back, and the specimen was submitted for histopathologic analysis (Figure, C and D).A, Illustrated are multiple brown hyperpigmented patches symmetrically affecting the neck, axillae, antecubital fossa, and intermammary and inframammary areas.


Each episode of joint pain and cutaneous eruption lasted 24 to 48 hours with episodes occurring anywhere from every other day to once weekly.

The patient’s joint pain and cutaneous symptoms resolved completely between each episode and were not associated with fever.

Review of photos taken during the patient’s flares revealed a variable eruption consisting of oval-shaped, salmon-pink macules on the trunk, urticarial-appearing papules on the dorsal hands, and flagellate arrays of petechial macules on the upper medial arms (Figure 1).

Her joint pain recurred with each episode of cutaneous eruption and primarily affected the wrist, knee, and ankle joints bilaterally.

A complete blood cell count and comprehensive metabolic panel as well as C-reactive protein and erythrocyte sedimentation rate obtained during a flare were normal.

Titers for parvovirus, Epstein–Barr virus, and antistreptolysin O were also negative.A, Approximately 1-cm, oval-shaped, salmon-pink macules on the right lower abdomen and right lateral thigh.

She had been treated with naproxen during these flares, which improved her joint pain but not her cutaneous symptoms.

The patient’s mother was unable to identify any possible triggers because the episodes occurred independent of pulmonary CF exacerbations or antibiotic use.

This eruption was intermittently pruritic but otherwise was asymptomatic.

B, 3- to 4-mm pink urticarial-appearing papules on the dorsal hand.

C, Flagellate arrays of nonblanching 1- to 2-mm petechial macules on the upper medial arm.


A review of his previous records indicated an admission to an outside facility 3 weeks beforehand with severe hyperglycemia and a hemoglobin A1c level of 14.1% (to convert to the proportion of total hemoglobin, multiply by 0.01).

Neurological examination results were significant for choreiform and hemiballistic movements involving the left upper extremity.

Despite an aggressive correction of blood glucose levels and metabolic correction, his choreiform movements had remained unresolved.

He was alert and oriented with healthy vital signs.

No evidence of cognitive impairments was present on bedside testing results.

On admission, a complete electrolyte and toxicology panel, including calcium, magnesium, and phosphorus, yielded normal results.

The patient’s serum glucose level was 159 mg/dL (to convert to millimoles per liter, multiply by 0.0555) and his hemoglobin A1c was 10.9% without ketoacidosis.

Test results for antinuclear antibodies, lupus anticoagulants, and antiphospholipid antibodies were negative.

Serum thyrotropin levels, liver function test results, and parathyroid hormone levels were healthy.

Copper studies, a peripheral smear, and a routine electroencephalogram yielded normal results.

A general screening for cancers yielded negative results.

The patient underwent brain magnetic resonance imaging (MRI) (Figure), which showed a T1 hyperintensity in the right caudate head and a lentiform nucleus with no restricted diffusion.

Oral administration of olanzapine, 2.5 mg daily, in divided doses with careful monitoring of blood glucose levels resulted in the complete resolution of chorea.Magnetic resonance imaging shows T1 hyperintensity in the right caudate head (A) and a lentiform nucleus with no restricted diffusion (B).

The patient denied any recent infections and risk for human immunodefiency virus (HIV) infection.

His home medications were carefully reviewed to rule out any medications that could cause chorea.


The patient had a history of asthma and had recently been hospitalized after radiologic findings of pulmonary infiltrates.

The patient denied rash, headache, and joint pain.

Serologic testing ruled out infectious causes of inflammation.On examination, best-corrected visual acuity was 20/25 OU.

Intraocular pressure for the right eye was 16 mm Hg and for the left eye was 24 mm Hg.

Slitlamp examination showed trace hyperemia of the right eye and 2+ diffuse scleral hyperemia of the left eye (Figure).

Corneas were clear bilaterally, and sensitivity was symmetric and full.

There was no cell or flare in the anterior chamber, and she had clear lenses with normal irises.

At hospital discharge, the patient’s medications were changed to prednisone, 11 mg orally once daily, and azathioprine, 100 mg orally once daily.Although the severity of the ocular symptoms was variable, there was no improvement with the described medication regimen or with currently prescribed topical corticosteroids given 4 times daily in both eyes.

The patient was diagnosed with pneumonia, myocarditis, and hypereosinophilic syndrome and received high-dose intravenous corticosteroids.


Her medical history was significant for a motor vehicle crash that occurred almost a year earlier, with no direct head trauma or eye injury.

On examination, her best-corrected visual acuity was 20/500 OD and 20/30 OS.

Anterior segment examination was remarkable for pseudophakia on the right eye and mild cataract in the left.

Fundus examination was remarkable for a slightly blunted foveal reflex in the right eye.

Spectral-domain optical coherence tomography (SD-OCT) showed segmental atrophy of the inner retina (Figure 1A).

Fluorescein angiography was unrevealing (Figure 1B).A, Spectral-domain optical coherence tomography of the right eye.

B, Fluorescein angiogram of the right eye.


His ocular history was significant for high myopia, a long-standing traumatic retinal detachment in his right eye, and laser-assisted in situ keratomileusis (LASIK) surgery of the left eye 8 months earlier.

Three months earlier, the patient experienced an episode of elevated intraocular pressure (IOP) of the left eye, which was treated with travoprost ophthalmic solution, 0.004%, and oral acetazolamide and resolved.

The patient’s presenting visual acuity was counting fingers OD (baseline) and 20/200 OS.

Two days later, the patient returned to the clinic with decreased vision in his left eye, which showed an IOP measured by GAT of 6 mm Hg, visual acuity of 20/400, and a cornea that was edematous (Figure 1).A, Slitlamp photograph showing a prominent laser-assisted in situ keratomileusis (LASIK) flap in the patient’s left eye.

His initial IOPs were right eye 16 mm Hg and left eye 46 mm Hg by TonoPen.

Examination of the left eye indicated normal gonioscopy results and unremarkable anterior and posterior segment examination findings except for the presence of corneal edema without keratic precipitates.

The patient started treatment with dorzolamide hydrochloride, timolol maleate, brimonidine tartrate, latanoprost, and oral acetazolamide.The next day, the left eye IOP improved to 28 mm Hg as measured by Goldmann applanation tonometry (GAT), with a clear cornea and return of visual acuity to 20/20.

Because of a concern for possible trabeculitis, he also started treatment with topical prednisolone acetate 4 times daily and oral valacyclovir hydrochloride.

Despite continuing maximum medical therapy for 1 week, the patient’s examination findings remained unchanged.


Medical and family history were otherwise unremarkable.

He had mild periorbital edema and low-grade eye pain in the left eye over 2 weeks prior to presentation.

He had completed antibiotic treatment for presumed sinusitis with no improvement.

A recent flulike illness over the past 1.5 weeks responded minimally to ibuprofen.

Magnetic resonance imaging of the orbits revealed an intraocular mass, suspicious for retinoblastoma.

The patient was advised consultation by an ocular oncology service.On examination, visual acuity was fix and follow OU.

The right eye showed normal findings anteriorly and posteriorly.

The left eye revealed mild conjunctival chemosis with periorbital edema.

There was trace eyelid edema and mild leukocoria.

Dilated ophthalmoscopic examination of the left eye displayed a solid gray mass in the macular region, deep to the retina and with surrounding subretinal fluid (Figure 1A).A, Dilated ophthalmoscopic examination found a gray mass in the macular region of the left eye measuring 12 mm in diameter and 5.1 mm in thickness, with additional subretinal fluid inferiorly.

B, B-scan ultrasonography confirmed the fundus mass, dependent subretinal fluid, and echolucency outside the posterior wall of the eye.Examination under anesthesia confirmed the solid mass, measuring 12 mm in diameter and 5.1 mm in thickness and demonstrating echodensity on ultrasonography (Figure 1B).

There were no tumor-feeding vessels, and tumor margins were ill defined.

Fluorescein angiography demonstrated slight hyperfluorescence of the disc and faint hyperfluorescence of the mass with overlying scattered pinpoint hyperfluorescent leakage.

Optical coherence tomography revealed shallow subretinal fluid extending within 3.2 mm of the foveola.


Bulging of the right eye was first noted 6 months earlier elsewhere when she reported progressive proptosis, worsening vision, and tearing in the right eye.

She did not report any pain or diplopia.Visual acuity was 20/150 OD (no improvement with pinhole) and 20/20 OS.

Pupillary reaction and confrontational visual fields were normal.

External examination was notable for proptosis of 7 mm, hypoglobus, and resistance to retropulsion.

A firm, nontender mass was palpable in the right superotemporal orbit.

The palpebral lobe of the right lacrimal gland was erythematous and prominent compared with the normal-appearing left lacrimal gland.

Ocular motility showed −2 abduction deficit, right eye.

Slitlamp examination revealed pseudophakia and posterior capsular opacification (right > left), but was otherwise normal.

Sensation was normal in the V1, V2 distribution, and there was no preauricular, submandibular, or anterior cervical lymphadenopathy.

Computed tomographic scan of the orbits without contrast from an outside facility revealed a spherical mass of unclear cause in the area of the right lacrimal gland.

No lytic bone changes or bone remodeling could be seen (Figure 1).Computed tomographic imaging from outside facility revealed a 1.9 × 2.3 × 1.8-cm mass of an unclear cause within the anterolateral right orbit, exerting mass effect on the globe.


She had no history of ocular disease or trauma, and her medical history was notable for resection of a benign thyroid mass 3 years prior.

Family and social history were unremarkable.

Her new-onset acquired ptosis began 10 months prior.

She had mild anisocoria with the right and left pupils reacting from 6 to 4 mm and from 5 to 3 mm, respectively.

The pupils were equal in light and dark, with no reversal after apraclonidine hydrochloride treatment.

No relative afferent pupillary defect was noted.

Results of the Hertel testing at a base measurement of 100 mm were 20.5 mm OD and 21.0 mm OS.

She had full ductions and versions, mild fullness of the left superior sulcus, and no sensory deficits in her facial cranial nerves.

Her vertical palpebral fissure measured 10 mm OD and 7 mm OS.

Her margin-to-reflex distance was 4 mm OD and 2 mm OS.

With phenylephrine hydrochloride, her margin-to-reflex distance became equal at 4 mm OS.


Two months later, he presented to a hospital with complaint of shortness of breath, cough, and chest pain.

On presentation to our multidisciplinary clinic, he reported constant, bothersome sweating of the left forehead.

A computed tomographic image of the chest (Figure 1) identified a large, 12-cm left upper lobe mass abutting the anterior chest wall pleura and extending into the mediastinum, with mediastinal lymphadenopathy as well as several liver lesions.

On physical examination, a well-demarcated area of erythematous, perspiring skin on the left forehead was noted.A, Coronal computed tomographic image of the thorax with intravenous contrast at initial presentation, demonstrating a left upper lobe mass; asterisk depicts possible area of sympathetic nerve compression or invasion.

B, Radiation planning computed tomographic image taken 2 weeks after initial presentation, demonstrating growth of the left upper lobe tumor.

A biopsy revealed poorly differentiated carcinoma, consistent with a primary tumor in the lung.

The red contour identifies the tumor; the orange contour, the area of sympathetic trunk compression; the yellow contour, the trachea; the teal contour, the left lung; the purple contour, the esophagus; the peach contour, the first rib.


While in the emergency department, she developed monomorphic ventricular tachycardia (VT) with hemodynamic instability and was successfully cardioverted.

She continued to have nonsustained monomorphic VT, so intravenous amiodarone and oral metoprolol were initiated.

Results of tests of electrolyte levels and coronary angiography were normal.

Cardiac magnetic resonance imaging with gadolinium contrast revealed normal-sized cardiac chambers and normal biventricular function without delayed enhancement.

She was admitted for further evaluation.


She was a nonsmoker and nondrinker.On examination, she appeared well.

At the time of presentation, it had begun to cause changes in her voice, globus sensation, and dysphagia.

The patient reported a 20-pound weight loss over the prior 3 months and decreased appetite.

She denied any associated difficulty breathing, pain, fever, chills, rash, or night sweats.

Despite a muffled voice, she was not in respiratory distress.

Fiberoptic laryngoscopy revealed a large, partially obstructing mass in the right oropharynx protruding from the right tonsil.

It descended inferiorly and was partially compressing the epiglottis.

The true and false vocal cords, although partially obstructed, appeared normal and mobile.

The lesion was without ulceration, exudate, or erythema.

The contralateral tonsil was small and normal in appearance.

The remainder of her physical examination findings were unremarkable, including no neck masses.The patient agreed to surgical excision of the mass, including tonsillectomy.

Her airway was secured via an awake transoral fiberoptic intubation without difficulty.

The planned procedure occurred without difficulty or complication.

The specimen excised was a 6 × 3 × 2-cm mass continuous with the right tonsil (Figure 1), without involvement of any surrounding structures.A, Intraoperative photograph of a large, irregularly shaped lesion protruding from the patient’s right tonsil.

B, The excised specimen measured 6 × 3 × 2 cm and was continuous with the right tonsil.


He had been born after an uncomplicated pregnancy, during which routine prenatal ultrasonography had not detected any anatomical abnormalities or lesions.

On physical examination, there were no abnormalities other than symmetrical submucosal swelling of the floor of the mouth and a midline external punctum present in the submental area (Figure, A).

The tongue was displaced posteriorly and superiorly.

Contrast-enhanced magnetic resonance imaging of the neck and face performed shortly after presentation showed a midline sublingual lesion extending inferiorly through the mylohyoid muscle (Figure, B-D).

The intrinsic muscles of the tongue appeared minimally displaced without obvious infiltration; however, the genioglossus muscle was difficult to locate relative to the mass.

A thin-walled, multiloculated lesion was identified, measuring approximately 2.0 × 2.5 × 3.2 cm.

The mass was bright on T2-weighted imaging and dark on T1-weighted imaging and did not enhance with intravenous gadolinium.

There were no obvious fluid-fluid levels or calcifications.

When in-phase and out-of-phase images were compared, there was no significant signal intensity drop, suggesting an absence of fat within the lesion.A, Appearance of the submental neck.

B, Coronal short tau inversion recovery (STIR) T2-weighted magnetic resonance image without contrast enhancement.

C, Coronal T1-weighted, fat-saturated magnetic resonance image after contrast enhancement.

D, Sagittal T1-weighted non–fat-saturated magnetic resonance image after contrast enhancement.


Family history included constipation in a sister and a great aunt with CF.

During her first week of life, she developed diarrhea and vomiting.

With initiation of solid food, she developed laxative-dependent constipation.

She underwent newborn genetic screening before routine cystic fibrosis (CF) screening.

Results of anorectal manometry, spinal magnetic resonance imaging, and thyroid studies were normal.

Physical examination findings were unremarkable, including normal respiratory examination.

Fecal elastase level was within reference range (>500 μg/g); abdominal computed tomographic image revealed a dilated, tortuous sigmoid colon; and full-thickness rectal biopsy was negative for Hirschsprung disease.

She was referred for sweat chloride testing to assess for CF.

Results of 3 separate sweat chloride tests were indeterminate (Table), prompting pulmonology referral.

A 97-mutation CF transmembrane conductance regulator (CFTR) analysis panel was negative.

At the time of presentation to the gastroenterology clinic, she had no respiratory symptoms.

Her body mass index (BMI) was below the third percentile (eFigureA in the Supplement).


He had no back or joint pain or skin rashes.

Five years ago he had a black ink tattoo placed on his left arm, and 3 years ago a second tattoo was placed on his right arm.

Two years after the right arm tattoo was placed, he noticed tattoo elevation on the left arm and onset of decreased vision.

He had recurrent episodes of ocular redness and blurred vision, coincident with inflammation of the skin surrounding both tattoos.

C, Hematoxylin-eosin stain of skin biopsy sample (original magnification ×100).At presentation, best corrected visual acuity was 20/20 in the right eye and 20/40 in the left.

Intraocular pressure was 34 mm Hg in the right eye (upper limit of normal, 21 mm Hg) and 11 mm Hg in the left.

On anterior segment slitlamp examination, there were no visible inflammatory cells in the anterior chamber of the right eye, but there were more than 26 cells per 1-mm field in the left.

Punch biopsy of the tattooed skin revealed epithelioid histiocytes with macrophages containing coarse black granular pigment (Figure, panel C).

Two months before presentation, bilateral peripheral iridotomies (iris laser procedures) were performed because of inflammation-induced adhesions between the iris and the lens (Figure, panel A).A, Slitlamp photograph of left eye.

The left arm tattoo was elevated, with nodular elevation most prominent at the superior aspect of the tattoo (Figure, panel B).

Results of testing for syphilis, tuberculosis, and sarcoidosis were negative.


The infant was born to a 31-year-old mother by normal vaginal delivery.

The mother had a benign prenatal course during pregnancy.

On further questioning, the parents reported that the infant had had contact with a babysitter who reported pruritus, especially at night.

The sitter had provided domestic care for the infant since birth until leaving that position 3 weeks earlier.On examination, the infant was afebrile with normal vital signs.

Since the rash developed, the infant had been fussy with increasing restlessness at night.

Multiple disseminated, edematous, pin-sized, papulopustulovesicles and 5- to 10-mm coalescing, crusted nodules were found all over the body; some with oval to elongated, serpiginous or J-shaped burrows (Figure 1).

A Tzanck smear showed no multinucleated giant cells in the pustular contents.

A Gram stain and potassium hydroxide (KOH) preparation of the pustular contents showed no bacterial or fungal elements.

Cultures of the pustular contents yielded no bacterial or fungal growth.

The differential of a complete blood cell count showed 12% eosinophils.

The parents denied any personal or family history of rash.


He reported that lesions began as pustules and developed into painful, itchy plaques.

A complete review of systems was notable for the patient’s difficulty with walking because of skin lesions.

On examination, pustules and hemorrhagic-crusted plaques were seen on the scalp, legs, arms, and trunk along with keloidal plaques on the face (Figure, A and B).

A punch biopsy specimen from the arm was obtained for histopathologic study (Figure, C and D).Extensive vegetative plaques with bleeding and ulceration on extremities (A), and pustules and ulcerated plaques on extremities and trunk (B).

Histopathologic examination of a biopsy specimen taken from the arm demonstrates pseudoepitheliomatous hyperplasia (hematoxylin-eosin stain, original magnification ×100) (C) and dermal neutrophilia and microabscesses with eosinophilia (hematoxylin-eosin stain, original magnification ×400) (D).

Before evaluation at a referral hospital in Kenya, the patient was treated with prednisolone tablet 5 mg twice daily, clindamycin hydrochloride 300 mg daily, and fluconazole 200 mg daily, but he continued to develop new lesions.

Enzyme-linked immunosorbent assay testing for HIV was negative.


He had no history of chicken pox or acne or traumatic lesions in those areas.

Before evaluation, his condition had been diagnosed as lupus and lipoatrophy and he tried several treatments with no success.The results of his physical examination showed several round atrophic lesions on his face, symmetric and bilateral, that were located especially on the malar and preauricular areas, with a diameter of 5 to 10 mm.

The patient denied experiencing previous inflammation.

His skin lesions were not indurated and displayed no epidermal changes (Figure 1).

New punch biopsy specimens were obtained for further evaluation.Atrophic lesions on the patient’s left cheek (A) and with a cicatricial appearance on the preauricular right area (B).


He had been diagnosed with multiple myeloma 2 years earlier after discovery of osteolytic bone lesions of the skull and the findings of a bone marrow biopsy.

He denied sexual risk behavior and reported no recent changes in medication.

At the time of the consultation, he was free of disease.The patient was referred for evaluation of an indolent, erythematous papule on his glans penis of 2 weeks’ duration.

Careful physical examination revealed a shiny, tense, erythematous, hemispherical papule measuring 5 mm in diameter located on the lateral aspect of the glans (Figure 1A).

The lymph nodes were not palpable, and there was no organomegaly.

A punch biopsy was performed for histopathological evaluation (Figure 1B and C).A, Shiny, erythematous, hemispherical papule on the lateral aspect of the glans penis.

The patient had been treated with bortezomib and dexamethasone followed by autologous blood progenitor cell transplantation, which resulted in complete response.

B and C, Hematoxylin-eosin stain was performed for evaluation.


On examination, he had a 5 cm × 3 cm palpable and partially reducible right groin mass that extended into the scrotum.

He had a leukocytosis with a leukocyte count of 16 400/μL (to convert to ×109/L, multiply by 0.001), his creatinine level was 1.14 mg/dL (to convert to micromoles per liter, multiply by 88.4), and urinalysis showed leukocyturia with cultures that had positive results for Streptococcus viridians.

Axial and sagittal (Figure) computed tomography scans with contrast are shown.


The pain was located in the periumbilical region and intensified within a few hours.

The physical examination was significant for diffuse abdominal tenderness.

The patient felt normal fetal movements during this time.

Next, the patient was evaluated by an obstetrician-gynecologist, and although the gynecologic examination, ultrasonography, and monitoring were all normal, the patient was admitted for further supervision in the obstetrics department.

The next day, when the abdominal pain and vomiting persisted and an elevated leukocyte blood count of 14 200/mL was noted (to convert to ×109 per liter, multiply by 0.001), a second surgical consultation was done.

Although the patient was pregnant, and owing to nonavailability of magnetic resonance imaging at that time, a contrast-enhanced abdominopelvic computed tomography was performed (Figure 1).Coronal computed tomographic image showing an edematous small intestinal loop on the left abdomen, with uterus and head of fetus in the pelvis.

An initial surgical evaluation was done, and the cause for the abdominal pain was assumed to be not of surgical condition.


The patient had his first liver transplant 12 years before presentation that failed despite immunosuppressive treatment with tacrolimus.

He initiated mycophenolate mofetil treatment 2 months before his second transplant.

In addition, the patient was positive for cytomegalovirus (CMV) IgG antibodies and had an undetectable CMV load 1 month before transplantation.On examination, visual acuities were hand motions OD and J1 OS.

He denied eye pain, floaters, and photopsias.

The patient’s right pupil was sluggishly reactive with a relative afferent pupillary defect.

Intraocular pressure was 16 mm Hg OD and 18 mm Hg OS.

Extraocular movements were intact, and visual fields were full by confrontation.

Anterior segment examination was within normal limits.

Fundus photographs of the right eye are shown in the Figure, A.


Review of medical history revealed lumpectomy and radiotherapy for breast cancer 6 years prior.

Visual acuity was 20/160 OD (her baseline due to amblyopia) and 20/20 OS.

The anterior chamber and vitreous were quiet.

Fundus photographs showed optic nerve edema and a peripapillary choroidal infiltrate in the right eye.

Swept-source optical coherence tomography (OCT) demonstrated retinal thickening with cystoid abnormalities, subretinal hyperreflective material consistent with fibrosis, and choroidal thickening in the right eye (Figure 1).

Optical coherence tomography of the optic nerve head in the right eye demonstrated 360° peripapillary nerve fiber thickening and diffuse nerve head elevation.

Fluorescein angiography demonstrated staining with minimal leakage in the temporal peripapillary retina of the right eye.A, Fundus photography of the right eye revealed optic nerve edema and a peripapillary choroidal infiltrate.

B, Swept-source OCT of the right eye demonstrated retinal thickening with cystoid abnormalities, subretinal fibrosis, and choroidal thickening in the area marked on the fundus photo.Testing results for an underlying neoplastic, inflammatory, or infectious process were unrevealing, including negative results of serology tests for tuberculosis, toxoplasmosis, syphilis, Lyme disease, and sarcoidosis.

Results of complete blood cell count, serum protein electrophoresis, and bone marrow biopsy were not supportive of lymphoproliferative disease.

Results of magnetic resonance imaging of the brain and orbits were normal.

Computed tomographic scan of the chest, abdomen, and pelvis demonstrated no lymphadenopathy.Four months prior to presentation, routine electrocardiogram demonstrated a right bundle branch block.

Eight months later, complete heart block necessitated urgent pacemaker implantation.

The line marks the location of the cut through the retina represented by the optical coherence tomography (OCT).


He had no relevant ocular or other medical history.

The worsening vision was preceded by binocular diplopia, which was intermittent at first and then became constant.

A review of systems was notable for a 2-month history of progressively worsening shortness of breath.

Visual acuity was found to be 20/200 OD and 20/25 OS.

His pupils were round and equally reactive, and there was no relative afferent pupillary defect.

The intraocular pressure was normal in both eyes.

On external examination, bilateral proptosis and increased resistance to retropulsion was observed.

There was very limited extraocular motility in all directions of gaze on the right side and moderately limited extraocular motility on the left side.

In addition, the patient had an esotropia and right hypertropia.

Anterior segment examination revealed bilateral chemosis.

The fundus examination (Figure 1) showed chorioretinal folds in both eyes and intraretinal hemorrhages in the right eye.

The optic discs had normal color and sharp margins bilaterally.A, Fundus photograph of the right eye showing prominent chorioretinal folds throughout the macula and 2 intraretinal hemorrhages near the vascular arcades.

He denied any fevers, night sweats, or unintentional weight loss.


Her medical history was notable for hypertension, treated with clonidine hydrochloride, and opiate abuse, treated with methadone hydrochloride.

Examination revealed periorbital edema, proptosis, and limitation in supraduction, abduction, and adduction in the right eye.

Two weeks later, new proptosis and periocular edema of the left eye developed, with no inciting trauma.

The patient was afebrile with no leukocytosis.

Her visual acuity was 20/20 OU with no afferent pupillary defect and normal intraocular pressures.

There was no periorbital soft-tissue erythema, warmth, or tenderness to palpation.

Computed tomography showed hyperattenuating soft tissue in the superior, medial, and inferior right orbit (Figure 1A, arrowheads).

Magnetic resonance imaging showed no internal flow voids to suggest vascular malformation.

Results of a laboratory workup revealed mildly elevated erythrocyte sedimentation rate (49 mm/h; reference range, 0-20 mm/h) and antinuclear antibody titer (1:80; reference range, <1:40) as well as positive results for anti-Ro and anti-La antibodies.

Results of further autoimmune and infectious workup were negative.

Her examination results were significant for proptosis, periorbital edema, and limitation in supraduction in the left eye.

Computed tomography showed new hyperattenuating soft tissue in the left superior and lateral orbit (Figure 1B, arrowhead).A, Coronal contrast-enhanced computed tomographic scan shows hyperattenuating soft tissue in the superior, medial, and inferior subperiosteal space of the right orbit (arrowheads).

B, Computed tomographic scan, not contrast enhanced, shows hyperattenuating orbital soft tissue in the left orbit (arrowhead).


She reported no history of diabetic retinopathy.

Visual acuity with correction was 20/80 OD and 20/30 OS.

Dilated fundus examination results were notable for mild cataracts in both eyes and fine superficial retinal hemorrhages surrounded by a cluster of exudates in the right retina (Figure 1A).

An examination of the left retina produced unremarkable results.Color fundus and optical coherence tomographic imaging of the right eye.

A, A color fundus photograph of the right macula showing superficial retinal hemorrhages surrounded by a cluster of exudates; B, Optical coherence tomography of the right eye showing nasal intraretinal fluid and exudates as well as a distinct cystoid cavitary abnormality just temporal to the center of the macula.Optical coherence tomography of the right eye showed nasal intraretinal hyporeflective spaces that were likely consistent with intraretinal fluid and hyperreflective areas most likely consistent with transudates of lipid, as well as a distinct cystoid cavitary abnormality just temporal to the center of the macula (Figure 1B).

A similar cystoid change was seen in the left eye (not shown).

Fluorescein angiography showed an area of bright hyperfluorescence nasal to the fovea that progressively intensified with the leakage of dye, obscuring the borders of this area in the late phases, as well as mild leakage temporal to the fovea in both eyes (not shown).Recommend intravitreal anti–vascular endothelial growth factor (anti-VEGF) injection.Recommend continued blood glucose control and consider intravitreal anti–VEGF injection or focal laser treatment if the macular edema persists.Explain to the patient that these changes are medication-associated and recommend close observation.Recommend referral to the patient’s primary care physician for stricter blood pressure control.


She also reported a 1-week history of left eye discomfort and left-sided tinnitus and a 1-day history of binocular horizontal diplopia.

She denied any history of recent trauma.

Ophthalmologic examination at the time revealed visual acuities of 20/30 OD and 20/40 OS.

Intraocular pressures were 15 mm Hg OD and 21 mm Hg OS, with a widened pulse pressure of the left eye.

Extraocular motility revealed a left eye abduction deficit.

Pupils and confrontational visual fields were unremarkable.

Hertel measured 16 mm and 17 mm, respectively.

Slitlamp examination of the right eye was unremarkable, but examination of the left eye revealed a large subconjunctival hemorrhage and engorged episcleral veins (Figure 1).

Fundus examination of both eyes was unremarkable.Clinical photographs taken 3 weeks apart.

A, Dilated nasal episcleral vessels and conjunctival chemosis near the onset of the patient’s symptoms.

B, Photograph taken 3 weeks later revealing increased dilated episcleral vessels and subconjunctival hemorrhage.


He denied flashing lights, visual field defects, trauma, pain, redness, discharge, or other systemic symptoms.On examination, the patient’s uncorrected visual acuity was 20/20 OD and 20/50 OS (no improvement on refraction), his pupils were equally round and reactive with no relative afferent pupillary defect, his intraocular pressures were normal, and extraocular motility and confrontational visual fields were full.

Results from slitlamp and dilated fundus examinations of the right eye were normal.

Anterior segment examination of the left eye showed mild fine keratic precipitates and pigmented cell; dilated fundus examination showed significant vitritis but no obvious vascular sheathing or chorioretinal lesions (Figure 1A).

Optical coherence tomography of the macula confirmed there was no intraretinal or subretinal fluid (Figure 1B), and fluorescein angiography did not show any vascular leakage or areas of nonperfusion.

Results of further workup showed normal complete blood cell count and comprehensive metabolic panel laboratory testing, undetectable HIV viral load, and unremarkable chest radiography; laboratory results for tuberculosis (T-SPOT.TB testing), syphilis (rapid plasma reagin with reflex treponemal testing), cytoplasmic and perinuclear antineutrophil cytoplasmic antibodies, and blood cultures were all negative as well.A, On color fundus photography, significant vitreous haze obscuring most of the retinal vessels was observed in the left eye, but no apparent chorioretinal lesions were seen.


Nine months before the current presentation, he was treated with intramuscular penicillin for syphilis.

His medical history was also significant for HIV, for which he was receiving highly active antiretroviral therapy, and metastatic programmed cell death ligand 1–positive squamous non–small cell lung cancer.

Four months before the current presentation, the patient had started pembrolizumab monotherapy as a first-line treatment for his cancer.

One month before this presentation, multiple ulcers developed on his buccal mucosa, as well as a nonpainful shallow penile ulcer and pruritic, mildly painful, hyperpigmented papules and plaques on the bilateral soles of his feet in a pattern identical to previous syphilitic eruption.

The patient denied fever, chills, and weight loss.

Physical examination revealed multiple hyperpigmented papules and plaques with violaceous borders on his bilateral soles and lateral feet (Figure, A) and scattered, violaceous papules and nodules with overlying scale on his bilateral lower extremities.

He was referred to the dermatology service, and a punch biopsy specimen was obtained from the right medial sole (Figure, B).A, Right foot with multiple hyperpigmented, lichenified papules, and plaques with violaceous borders.

Lymphocytes, eosinophils, and plasma cells can be seen within the infiltrate (hematoxylin-eosin, original magnification ×40).

B, A punch biopsy specimen from the right medial sole.


A computed tomographic examination of the brain showed a right frontal lobe abscess with vasogenic edema (Figure 1).

She underwent burrhole aspiration of the abscess, which grew mixed oral flora.

Treatment involved a prolonged course of intravenous antibiotics, steroids, and medications for seizure prophylaxis.


Nonenhanced computed tomography of the paranasal sinuses revealed a soft-tissue mass extending from the nasopharynx into the left sphenoid sinus with erosion of the sphenoid sinus floor and the clivus (Figure 1A).

The sella was apparently normal with no disruption of its floor.

The intracranial and orbital soft tissues were grossly normal, as were the remaining soft tissues under the skull base.A, Sagittal nonenhanced computed tomography images of the paranasal sinus depicting a soft-tissue mass involving the sphenoid sinus, the nasopharynx, and the clivus.

B, Postcontrast sagittal T1-weighted magnetic resonance shows avid enhancement of the mass; involvement of the clivus is also depicted.A magnetic resonance imaging study was also performed (Figure 1B).

There was a lobulated mass centered to the nasopharynx, measuring about 2.8 × 2.6 × 4.5 cm (anteroposterior × transverse × craniocaudal planes, respectively) in maximal diameters.

The mass demonstrated intermediate T2 hyperintense signal and avid postcontrast enhancement.

It extended inferiorly down to the oropharynx and superiorly along the floor of the left sphenoid sinus with extension into the sinus itself; posteriorly, there were erosions involving the sphenoid bone and the clivus.

The floor of sella was intact, and a normal enhancing pituitary gland and infundibulum were seen within the sella.

No sellar or suprasellar mass was evident.

Both Meckel caves were normal, and the cavernous sinuses were also normal and without evidence of asymmetric mass or abnormal enhancement.

The patient underwent a total mass resection via endoscopic anterior cranial base approach, with extradural tumor resection, reconstruction of the skull base, and a nasoseptal flap.


She reported diminished left-sided hearing that would temporarily improve with autoinsufflation.

She denied facial numbness or weakness, and reported no dizziness or vertigo.

The patient was initially observed but then experienced progressive hearing loss that no longer improved with the Valsalva maneuver, persistent ear fullness, and rare facial twitching.

Physical examination revealed a retrotympanic, nonpulsatile, tan-gray mass anterior to the malleus handle.

A tuning fork examination revealed lateralization to the left ear and bone conduction louder than air conduction on the left side with a 512-Hz tuning fork.

The remainder of the physical examination, including facial nerve function, was normal.

An audiogram confirmed mild conductive hearing loss in the left ear.

Computed tomography (CT) revealed a middle ear lesion lateral to the geniculate ganglion with possible extension into the peritubal space.

Magnetic resonance imaging (MRI) demonstrated an enhancing lesion lateral to the geniculate ganglion (Figure, A-C).

The middle ear mass appeared enlarged on repeated MRI and began to obstruct the eustachian tube.

Transcanal endoscopy demonstrated a gray middle ear mass occupying the anterior mesotympanum and epitympanum and extending into the eustachian tube (Figure, D).

The specimen revealed predominantly spindled stroma with a focus of tumor that was positive for antiepithelial membrane antigen and somatostatin receptor 2A.A, Noncontrast axial computed tomogram (CT) of the left temporal bone demonstrates an anterior mesotympanic mass lateral to the cochlea (yellow arrowhead) with blockage and extension into the supratubal recess (white arrowhead).

A partial mastoid effusion (asterisk) is also visible.

B, The coronal CT demonstrates an intact tegmen tympani with hyperostosis (arrowhead).

C, Axial, fat-suppressed, postgadolinium, T1-weighted magnetic resonance image (MRI) reveals an enhancing mass of the left perigeniculate region (yellow arrowhead).

The geniculate ganglion is marked (white arrowhead).


She was born at 36 weeks’ gestation and spent 10 days in the neonatal intensive care unit.

Her parents noted that the lesion was present at birth and she always seemed congested on the left side.

Physical examination showed an approximately 1-cm, firm, pedunculated polypoid mass in the anterior nasal cavity at the vestibule.

The base was located just anterior to the septum and encompassed nearly the entire left nasal cavity.

There was no fluid in the mass, and there was a negative Furstenberg sign.

Nasal endoscopy was performed in the clinic, and there were no additional masses or mucosal abnormalities.

The patient was also noted to have left eye ptosis and left preauricular branchial remnants but no other significant abnormalities on examination.

Magnetic resonance imaging was performed (Figure 1B) and showed a 0.8 × 0.5–cm hyperintense mass on T1-weighted images, suggesting a fatty component and no intracranial tract.Photograph (A) and magnetic resonance imaging (MRI) (B) of the left anterior nasal cavity mass.


His parents were first cousins, but no similar cutaneous presentation was reported by other relatives.The physical examination revealed scaly skin with diffuse warty lesions on sun-exposed areas.

These lesions developed from local freckles beginning at 6 months of age and gradually spread with protruding growth.

Over the past 2 years, tumors started to form and develop rapidly, becoming ulcerative and bleeding.

The patient’s face was severely disfigured, with numerous ulcerative, malodorous tumors (Figure 1A).

The general examination findings, including neurological and ophthalmologic tests, were unremarkable.

The lesional discharge culture revealed infection with Proteus vulgaris.

Punch biopsy of a paranasal mass was performed (Figure 1B).

Craniofacial computed tomography showed heterogenous soft tissue masses located superficially without bony invasion.

No signs of cerebral atrophy were noticed.

B, Infiltrating basal cell carcinoma (original magnification ×40).

The patient was intellectually standard and denied blurred vision, impaired hearing, or any other systemic symptoms.

Genetic testing was advised but was declined.A, Preoperative clinical photography revealed a face severely disfigured by diffuse warty lesions and numerous tumors.


She did not report a history of xerostomia, keratoconjunctivitis sicca, pleural effusions, pneumothoraces, seizure, cognitive impairment, recurrent sinopulmonary infections, or previous autoimmune disease diagnosis and had no relevant family history of lung disease.

She had slowly progressing dyspnea over the past year and general fatigue.

Her physical examination findings were unremarkable and there was no evidence of cutaneous lesions.

A high-resolution computed tomographic (CT) scan of her chest revealed diffuse thin-walled pulmonary cysts without nodules, parenchymal changes, or lymphadenopathy (Figure).

Imaging of her abdomen did not show kidney lesions or abdominal masses.

The patient’s serum vascular endothelial growth factor D (VEGF-D) level was elevated at 1300 pg/mL (reference range, <600 pg/mL).High-resolution computed tomography scan of the chest showing numerous, diffuse, thin-walled lung cysts surrounded by normal-appearing lung parenchyma.

There is no mediastinal or hilar adenopathy.The VEGF-D level is nonspecific and the patient should undergo further evaluation with a lung biopsy.The VEGF-D level is supportive of a diagnosis of pulmonary Langerhans cell histiocytosis.The VEGF-D level is supportive of a diagnosis of lymphangioleiomyomatosis.The VEGF-D level indicates that the patient should undergo folliculin gene test for Birt-Hogg-Dubé syndrome.


She noted pain and swelling in her right knee, which migrated to the right ankle and then affected the metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joints of her fingers.

A malar rash was present (Figure), but there was no rash on the trunk or extremities.

She had swelling and tenderness of the MCP and PIP joints and had difficulty making a fist and fully extending her fingers.

Laboratory studies revealed a mild normochromic normocytic anemia with a hemoglobin level of 11.5 g/dL, normal white blood cell and platelet counts, and normal results for a comprehensive metabolic panel.

She had a positive antinuclear antibody (ANA) result (1:160 [homogeneous pattern; negative <1:80 serum dilution]).

About 2 weeks before her symptoms began, her 5-year-old grandson had a febrile illness characterized by headaches, body aches, malaise, and rash on the cheeks, trunk, and extremities but no arthritis.

Ten days later, her 10-month-old granddaughter developed a similar febrile illness.

Eight days ago, her 25-year-old daughter developed headaches, malaise, and significant arthralgias affecting her wrists and knees but no rash or fever.

Manifestations in all of the family members resolved spontaneously within 1 week.On examination, the patient was afebrile with normal vital signs.


A computed tomography scan showed diffuse enlargement of the pancreas with peripheral hypoenhancement but no discrete mass.

Soft tissue surrounded the infrarenal abdominal aorta, extended along the common iliac arteries, and obstructed the left ureter, causing hydronephrosis.

Laboratory studies including immunoglobulin G subgroup 4 (IgG4) levels are shown in the Table.

Fine-needle aspiration of the pancreas showed no malignancy.

Colonoscopy results 8 months ago were normal.Test results indicate pancreatic cancer but the patient is a carbohydrate antigen (CA) 19-9 nonsecretor.

He was referred for evaluation of possible malignancy.


His medical history was significant for Philadelphia chromosome-negative B-cell acute lymphocytic leukemia refractory to multiple treatments, including chimeric antigen receptor T-cell therapy, with transformation to acute myeloid leukemia (AML) status posttreatment.

He was not receiving active cancer treatment.

At the time of evaluation, he had been hospitalized for 5 months with severe protein-calorie malnutrition, pancytopenia, and recurrent neutropenic fevers.

Intraoral examination revealed poor oral hygiene.

The left hard palate exhibited a large swelling with a 5×3-cm indurated area of ulceration, central necrosis, and desquamation (Figure, A).

The patient was febrile (38.3°C), with a white blood cell count of 0.5/μL (absolute neutrophil count 0.00; to convert to ×109/L, multiply by 0.001), hemoglobin levels of 7.6g/dL (to convert to g/L, multiply by 10.0), hematocrit 21%, and platelet count 9/μL (to convert to ×109/L, multiply by 1.0).

A maxillofacial computed tomography (CT) scan completed the etiological investigation.A, The left hard palate shows enlargement with a large, focal, necrotic lesion.

B, Hematoxylin-eosin stain of palatal tissue (original magnification ×1000).

C, Grocott methenamine silver stain of palatal tissue (original magnification ×1000).

D, Computed tomographic image of the maxillofacial complex shows tissue enlargement in the left hard palate and maxillary sinus.

The patient was treated palliatively with dexamethasone solution and nystatin rinse.

A biopsy was not recommended owing to medical instability.

However, the patient retained a sloughed fragment of the affected tissue, which was submitted for histologic evaluation.


There was no history of any systemic disorder and no family history of similar lesions.

Physical examination revealed multiple, well-demarcated, hypopigmented 1- to 5-mm macules on the lower abdomen and pubic area (Figure, A and B).

Direct microscopic examination of scales soaked in potassium hydroxide did not reveal hyphae.

Wood light examination revealed no accentuation.

A punch biopsy specimen was obtained for further evaluation (Figure, C and D).A, Multiple discrete hypopigmented macules on the lower abdomen and pubic area.

C, Histopathological examination revealed mild hyperkeratosis, acanthosis, and scattered clear cells in the epidermis (hematoxylin-eosin).

D, The clear cells are larger than the neighboring keratinocytes and contain abundant pale cytoplasm (hematoxylin-eosin).

B, Well-demarcated hypopigmented macules on the lower abdomen.


The plaque first appeared as a small inflammatory papule on the left nostril.

One month before presentation at our facility, the patient began developing erythematous papules on his arms and legs.

At admission, the patient was tachycardic and febrile to a temperature of 38.5°C.

Routine laboratory testing revealed normocytic anemia (hemoglobin, 7.7 g/dL [to convert to grams per liter, multiply by 10]).

Shave biopsies of necrotic nodules on the left arm and left cheek (Figure, B-D) and, later, an excisional biopsy of a cervical lymph node were performed.

Head computed tomography demonstrated a soft-tissue mass that encased the nasal bone and extended into the right maxillary sinus and left nasal cavity.

Chest computed tomography showed bilateral pulmonary nodules.

Physical examination revealed a necrotic plaque with surrounding erythema on the distal nose (Figure, A).

A 1 × 1-cm crateriform papule with central necrosis was present on the left malar cheek.

Multiple nodules with central, dry necrosis and areas of poxlike scarring were scattered across the extremities.

Slight contractures of his fingers were also noted.A, Clinical photograph.

Shortly thereafter, the patient was hospitalized at an outside facility for multifocal pneumonia.

He was also treated for a methicillin-resistant Staphylococcus aureus infection of his nose.


Four months earlier, he had presented to another hospital and received a diagnosis of adhesive small bowel obstruction, for which he underwent laparotomy and lysis of small adhesive bands that provided only temporary symptomatic improvement.

At his current presentation, he was unable to tolerate any oral intake and entirely dependent on total parenteral nutrition.

On examination, he was cachectic with a moderately distended, nontender abdomen.

The result of a blood test (QuantiFERON-TB Gold; Qiagen) was positive for tuberculosis exposure.

An abdominal computed tomography scan showed markedly dilated stomach and duodenum with decompressed, thickened distal small bowel without a clear transition point.Nonoperative management with persistently high nasogastric tube output failed, and the patient was subsequently taken to the operating room for exploratory laparotomy.

Intraoperative findings are shown in Figure 1.Exploratory laparotomy showing the appearance of the small bowel.


His medical history was significant for hypertension, hyperlipidemia, and kidney stones.

Physical examination revealed poor dental hygiene and findings in the lower extremity suggestive of postthrombotic syndrome.

The patient was afebrile with a heart rate of 108 beats per minute and a leukocyte level of 11 600/cm (normal range, 4000-11 000/cm), with 84% polymorphic neutrophils (normal, <75%); erythrocyte sedimentation rate was 57 mm/h (normal range, 0-10 mm/h), and C-reactive protein level was 15.9 mg/dL (normal, <0.6 mg/dL [to convert to nmol/L, multiply by 9.524]).

His surgical history was significant for a bilateral staged carotid endarterectomy in 1999 and 2000 and an open repair of a 7.4-cm transverse diameter juxtarenal abdominal aortic aneurysm with a knitted Dacron tube graft (18 mm) in 2014.

Ligation of the left renal vein adjacent to the inferior vena cava was performed to gain proximal control of the aorta.

The patient underwent extraction of a tooth secondary to an abscess 4 months earlier.

Computed tomography (CT) of the abdomen and pelvis was performed (Figure 1A and B).CT indicates computed tomography; WBC, white blood cell.


Her medical and ocular histories were significant for pseudophakia of both eyes, obstructive sleep apnea, and hypertension treated with hydrochlorothiazide.On examination, visual acuity was 20/40 OD and 20/64 OS.

She described her vision as being blurred in the left eye, with areas of her vision being “blocked.” Her review of systems was unremarkable.

Intraocular pressure, pupillary reaction, and ocular motility were normal.

Confrontation visual fields revealed a deficit in the inferotemporal quadrant of the left eye.

External and slitlamp examinations were unremarkable.

Dilated fundus examination revealed an epiretinal membrane, as well as extramacular, elevated, hypopigmented, yellow lesions with retinal pigment epithelium changes in both eyes, along with overlying subretinal fluid, subretinal hemorrhage, and exudates in the left eye (Figure, A).

Optical coherence tomography showed an epiretinal membrane with cystoid macular edema and drusen in both eyes, along with subretinal fluid extending into the left macula, explaining her decreased vision.

B-scan ultrasonography demonstrated hyperechoic, plaquelike, solid lesions with posterior acoustic shadowing corresponding anatomically to the lesions seen on clinical examination (Figure, B).

Fluorescein and indocyanine angiography demonstrated leakage in the area of subretinal hemorrhage in the left eye (Figure, C).A, A yellowish lesion is located in the superonasal quadrant with nasal subretinal hemorrhage (asterisk).


His medical history included hypothyroidism and meningioma of the cervical spine.

There was no history of trauma or surgery in the previous year.

The symptoms started in the right eye and progressed to the left eye 4 months later.

The patient had been evaluated elsewhere for suspected autoimmune retinopathy, with normal results for serum antibodies against recoverin and enolase, a complete blood cell count, antinuclear antibody, rheumatoid factor, anticitrullinated protein antibody, extractable nuclear antigens, and serologies for syphilis and Lyme disease.

A previous neuro-ophthalmologic evaluation, including magnetic resonance imaging of the brain and orbits, had been unrevealing.His best-corrected visual acuity measured 20/400 OD and 20/100 OS.

The results of a kinetic perimetry with a Goldmann perimeter demonstrated dense bilateral cecocentral scotomata.

There was no afferent pupillary defect in either eye.

A fundus examination showed trace temporal optic disc pallor in the right eye and a normal-appearing optic disc in the left eye (Figure 1A).

Full-field electroretinography results were normal, but multifocal electroretinography (mfERG) showed reduced wave amplitudes nasal to the fovea in each eye (Figure 1B).

Fundus autofluorescence and spectral-domain optical coherence tomography (SD-OCT) scans of the macula and optic nerve showed no abnormalities.A, Fundus photography shows a normal-appearing optic disc of the left eye.

B, Multifocal electroretinography demonstrates reduced amplitudes of waveforms nasal to the fovea of the left eye.


He received a diagnosis of type 1 diabetes at 10 years of age and has been taking insulin since the diagnosis.

He had consulted several physicians for hearing loss during the past 2 years.

He had received multiple courses of antibiotics for frequent urinary tract infections.

The results of a systemic examination revealed hypochondroplasia, alopecia (Figure 1), and generalized hypotonia.

His best-corrected visual acuity was 6/60 OU.

Bjerrum tangent screen visual field analysis showed preservation of central 10 degrees of fields.

His color vision was defective, and his pupils were slow reactive to light.

The results of an optic nerve examination revealed bilateral pale optic discs with no evidence of edema and normal retinal vasculature.

The results of the examination of the macula were normal in both eyes, with no diabetic retinopathy.


Her medical history was remarkable for long-standing hypertension, diabetes, and mixed connective tissue disease.

On examination, the best-corrected visual acuity was 20/40 OD and 20/25 OS.

Slitlamp examination showed prominent nuclear sclerosis and a posterior subcapsular cataract in the right eye and a well-positioned intraocular lens in the left eye.

Ophthalmoscopic examination revealed bilateral pigment mottling with extensive peripheral depigmentation of the retinal pigment epithelium, sparing the central fovea bilaterally.

Spectral-domain optical coherence tomography showed complete loss of photoreceptors outside the fovea, with retinal pigment epithelial atrophy, irregular proliferation, and some remodeling (Figure, A).

Fundus autofluorescence showed a ring of parafoveal hypofluorescence and hyperfluorescence, with speckled hypofluorescence peripherally (Figure, B).

Goldmann perimetry of both eyes (Figure, C) revealed significant constriction of the V-4-e isopter with an extensive ring scotoma between approximately 10° and 30° eccentricity.

Central vision with the I-4-e isopter was constricted to 5° to 7°.

A multifocal electroretinogram (ERG) showed profound signal attenuation throughout the examined area.

The full-field electroretinogram was nonrecordable under both scotopic and photopic conditions.A, Spectral-domain optical coherence tomography (SD-OCT) reveals extensive and diffuse retinal thinning (arrowhead) beyond the fovea, with loss of photoreceptors and pigment migration into the retina.


He was initially seen locally for new-onset double vision in upgaze without other visual symptoms.

During the subsequent 6 months, the patient experienced graying of vision on rightward gaze.

The patient presented to our clinic 2 years after initial symptom onset with continued graying of vision on eccentric gaze.

The patient had a 3–prism diopter left hypertropia in upgaze with full motility and was incidentally found to have optic disc edema in the right eye.

His best-corrected visual acuity was 20/15 OU.

Automated visual fields were normal, and there was no relative afferent pupillary defect.

Magnetic resonance imaging (MRI) revealed no apparent abnormalities (Figure 1A).

Results of an evaluation again demonstrated normal visual acuity and visual fields.

His best-corrected visual acuity on examination was 20/25 OD and 20/20 OS.

Relative afferent pupillary defect was noted in the right eye.

There was prominent chronic-appearing optic disc edema in the right eye without abnormalities in the left eye (Figure 1B).

Results of visual field testing showed superior greater than inferior peripheral field loss in the right eye and normal visual field in the left eye.A, T1-weighted magnetic resonance imaging scan of the orbit with contrast reveals trace enhancement at the orbital apex 2 years prior to presentation (arrowhead).

B, Fundus photograph of the right eye demonstrates prominent chronic-appearing optic disc edema.

Results of a local workup were negative, including acetylcholine receptor antibody.


According to the patient’s report, he had experienced this vision abnormality in the left eye for 1 week.

Two weeks later, he experienced acute worsening of visual acuity to 20/200 OU.

At presentation, visual acuity was 20/20 OD and 20/400 OS.

Results of a dilated fundus examination were normal in the right eye and notable for a macular serous retinal detachment in the left eye (Figure 1A).

Findings on optical coherence tomography (OCT) showed a dome-shaped collection of subretinal fluid in the macula (not shown).

Late-phase fluorescein angiography revealed multifocal pinpoint areas of hyperfluorescence with mild leakage within the area of detachment (Figure 1B).Left eye.

Examination and OCT showed resolution of subretinal fluid.

Examination and OCT findings showed subretinal fluid in the macula bilaterally.

Late-phase fluorescein angiography again revealed multifocal pinpoint areas of hyperfluorescence with mild leakage.

B, Late-phase fluorescein angiography shows multifocal pinpoint areas of hyperfluorescence with mild leakage.The patient was given a diagnosis of central serous chorioretinopathy (CSR) in the left eye.

A, A color fundus photograph shows serous macular detachment.

After 3 weeks of observation, visual acuity in the patient’s left eye improved to 20/40.


Previous work-up had revealed minimal abdominal adenopathy, but the patient declined further workup.

The lesion was itchy but without generalized pruritus.

The patient reported a weight loss of 10 pounds over the preceding 3 months.

In addition, he reported a longer history of vague, nonspecific, intermittent abdominal pain without nausea, vomiting, or fever starting about 3 years prior to presentation.

On examination, he had no other cutaneous lesions, but he did have cervical lymphadenopathy.

All other physical findings were within normal limits.

Laboratory findings were unremarkable, save for a mild normochromic normocytic anemia and a mildly elevated levels of lactate dehydrogenase.

A biopsy specimen from the skin lesion is shown in Figure 1B.A, Clinical image of the patient at presentation.

B, Biopsy specimen from the skin lesion is shown.


His medical history was significant for remote percutaneous coronary intervention 19 years ago, with no available details at the time of the patient’s presentation.

On arrival, the patient was hemodynamically unstable, with a blood pressure of 85/55 mm Hg.

He was tachycardiac, with a heart rate of 105 beats per minute, and his oxygen saturation level was 91% on room air.

Physical examination showed no jugular vein distension or peripheral edema, the presence of bibasilar rales, and no cardiac murmurs.

A 12-lead electrocardiogram showed inferior ST-segment elevation in leads II, III, and aVF.

The patient was transferred urgently to the cardiac catheter laboratory.


He reported no fevers, night sweats, abdominal pain, emesis, and diarrhea.

Physical examination revealed a thin habitus; no palmar erythema, spider angiomas, or gynecomastia were observed.

Laboratory tests showed normal complete blood cell count and chemistry panel findings.

Hepatic panel showed an elevated alkaline phosphatase level (209 U/L; upper limit of normal [ULN], 150 U/L [3.49 μkat/L; ULN, 2.5 μkat/L]), normal total bilirubin level (0.4 mg/dL [6.84 μmol/L]), elevated alanine aminotransferase level (41 U/L; ULN, 35 U/L [0.68 μkat/L; ULN, 0.58 μkat/L]), and normal aspartate aminotransferase level (27 U/L [0.45 μkat/L]).

A single-phase computed tomography (CT) scan of the abdomen revealed multiple hypoattenuating masses with rim enhancement throughout the liver, intussusception of the small bowel, and normal liver contour with no signs of portal hypertension (Figure 1, left).

α-Fetoprotein (AFP) level was elevated (1252 ng/mL; ULN, 9 ng/mL); levels of carbohydrate antigen 19-9 (<2 U/mL) and carcinoembryonic antigen (2.8 ng/mL) were normal.

A triple-phase CT scan was obtained to further evaluate the small bowel, which revealed no small bowel abnormalities but instead demonstrated a 3.3 × 2.8-cm heterogeneously enhancing pancreatic tail mass not seen on the previous single-phase CT (Figure 1, right).Left, Axial view from single-phase computed tomography (CT) scan of the abdomen revealing bilobar liver masses.


She presented with obvious biphasic stridor and a report of dyspnea on mild exertion.

Vocal cord mobility was normal on fiberoptic laryngoscopy, and the patient verbally denied having dysphagia.

Noncontrast computed tomography of her chest showed a 1.1-cm nodular lesion in the upper trachea that appeared to be originating from the right lobe of the thyroid (Figure 1A).

A CT of the chest showed a soft-tissue mass protruding into the trachea in the posterolateral aspect of the right side of the tracheal region that appeared to be contiguous with the thyroid gland.

Ultrasound-guided fine-needle aspiration of the thyroid was performed; however, there was no nodule in the right thyroid lobe that correlated with the CT findings.

The left thyroid lobe contained an 8-mm nodule.

Examination of a biopsy specimen from this nodule showed medullary thyroid cancer (MTC).

The patient’s serum calcitonin level was 23 pg/mL.

Magnetic resonance imaging (MRI) of the neck revealed a hyperintense tracheal mass on T2-weighted imaging (Figure 1B) that showed enhancement on administration of contrast medium (not shown).

In addition, a 0.3-cm nonenhancing nodule at the lower pole of the right thyroid lobe, separate from the tracheal mass, was identified.

Both ultrasonography and CT/magnetic resonance imaging failed to identify any suspicious adenopathy.A, Axial computed tomogram (CT) of the chest without contrast medium shows a 1.1-cm hypodense, exophytic right posterolateral intratracheal mass with apparent continuity with the right thyroid lobe.

B, Axial, T2-weighted magnetic resonance image (MRI) with contrast medium revealed a hyperintense soft-tissue mass within the tracheal lumen.

A T1-weighted image before the addition of contrast medium (not shown) showed a slightly hyperintense mass to the thyroid gland.

She was referred to our center for evaluation and management of what appeared to be an aggressive thyroid malignant neoplasm.


She denied a medical history of sinonasal issues, immunodeficiency, autoimmune disease, malignant disease, or diabetes.On examination, she was afebrile with stable vital signs.

She described a 2-week medical history of diplopia and blurry vision as well as 4 months of nasal congestion and progressive headaches despite multiple antibiotic courses.

Ophthalmologic evaluation revealed a right afferent pupillary defect and cranial sixth-nerve palsy.

Computed tomographic and magnetic resonance imaging of the sinuses revealed abnormal soft tissue throughout her sinuses with destruction of the sinus walls and skull base as well as extension of abnormal appearing tissue into the right orbital apex and posterior nasal septum.

Laboratory evaluation demonstrated a normal white blood cell count and an elevated erythrocyte sedimentation rate and C-reactive protein level.

Nasal endoscopy showed bloody mucus and crusting around the right middle turbinate and sphenoethmoidal recess.The acute nature of her vision loss amid concern for a destructive sinonasal process warranted operative intervention.

A representative final pathology slide is shown in the Figure.Sinonasal mucosal biopsy results demonstrate numerous multinucleated giant cells (black arrowheads), necrosis (white arrowhead), and inflammatory cells invading vessel walls (blue arrowheads).

Abnormal soft tissue with associated destruction throughout her sinonasal cavities and right orbital apex was identified.

Frozen sections revealed negative results for fungal elements and cellular dysplasia but demonstrated widespread inflammation with giant cells.

Hematoxylin-eosin stain (original magnification ×10).