He had a strong family history of bilateral ptosis, which included his father, grandfather, uncles, cousins, and 4 of his siblings (he had a total of 13 siblings [7 brothers and 6 sisters]).

He had no history of trauma, crossing or drifting of the eyes, dysarthria, dyspnea, or diplopia.

His medical history was unremarkable except for hypertension.External photograph showing bilateral blepharoptosis with dermatochalasis.

The ptosis did not worsen or fluctuate throughout the day.

He had noticed mild lightheadedness and trouble swallowing.

Notice the chin-up head position that the patient has to maintain to look in the primary gaze.His visual acuity was 20/25 OU with an intraocular pressure of 11 mm Hg OU.

He had a mild decrease in elevation and abduction in both eyes on extraocular motility testing.

Pupil reactivity and confrontational visual fields were normal.

The results of anterior and posterior segment examinations were normal.

The margin to reflex distance was −1 mm OD and −3 mm OS.

Levator function was 4 mm OD and 3 mm OS with normal orbicularis function.

Goldmann perimetry showed a 30° improvement in the right eye and a 50° improvement in the left eye, with taping of the upper eyelids.

None of his children or his siblings’ children were affected (the oldest being 31 years of age).


He was otherwise healthy, with no past medical or ocular history.

Despite his headaches, he denied having eye pain.

Prior to referral, his symptoms prompted magnetic resonance imaging of the brain, orbits, and cervical spine which were unremarkable.

Laboratory test results for the presence of Lyme disease, rapid plasma reagin, antinuclear antibodies, angiotensin-converting enzymes, and erythrocyte sedimentation rates were normal.The best corrected visual acuity was 20/50 in the right eye and 20/200 in the left eye.

Motility, intraocular pressures, and pupillary light reflexes were normal.

The anterior segment examination results were normal, and the lenses were clear bilaterally.

A fundus examination showed vitreous cells in both eyes, but the optic nerve and retinal vessels were visualized clearly.

Also, both eyes had optic nerve hyperemia and multiple shallow serous retinal detachments.


His medical history was negative for diabetes and hypertension, and review of systems was positive for recent easy bruising and progressive fatigue.

He reported flashes and progressive blurry vision in both eyes over a period of 6 days.

His initial best-corrected visual acuity was 20/25 OD and 20/50 OS.

Results from examination of his pupils, motility, intraocular pressures, confrontational visual fields, and anterior segments were normal.

A dilated fundus examination was conducted (Figure).

He had bilateral flame-shaped and intraretinal hemorrhages in both maculae along the superior arcades and some scattered peripheral hemorrhages.

There was no vessel tortuosity, and there were hard exudates in the left macula.

Spectral-domain optical coherence tomography showed areas of hyperreflectivity in the middle and outer retina.

Fluorescein angiography of both eyes showed blocking in the areas of hemorrhage, good venous filling, no leakage, and no neovascularization.Wide-field scanning laser image of the left eye showed superficial and intraretinal hemorrhages and hard exudates.


Her history was significant for glaucoma and breast cancer with brain metastasis treated with CyberKnife and whole-brain radiation.

She had a left afferent pupillary defect and left superior visual field deficit.

At presentation, her visual acuity was 20/20 OD and 20/50 OS, and intraocular pressures were 18 and 20 mm Hg, respectively.

Slitlamp examination of the right eye was unremarkable, while the left eye showed 1+ cell in the anterior chamber and 1+ nuclear sclerosis with pigment on the anterior lens capsule.

Posterior examination revealed 2+ whitish vitreous deposits, a cup-disc ratio of 0.8, and inferior retinal detachment with subretinal particulate material (Figure, A).

B-scan showed vitreous, subhyaloid, and subretinal opacities without choroidal involvement (Figure, B).

A magnetic resonance image of the brain and orbits did not reveal any new metastatic lesions.A, Whitish vitreous deposits, inferior retinal detachment, and subretinal material seen on fundus photography of the left eye.

While abroad, she received 2 inferiorly placed periocular steroid injections and was treated with glaucoma drops for elevated pressures of 55 mm Hg.


There was no history of trauma or alcohol intake.

There was guarding and tenderness in the right upper quadrant; the liver was firm, smooth, and palpable 3 cm below the right costal margin.

Clinical examination revealed no icterus or significant lymphadenopathy.

Laboratory investigations were remarkable for white blood cell counts of 13 200/μL (to convert to × 109 per liter, multiply by 0.001) and alkaline phosphatase of 259 U/L (to convert to microkatals per liter, multiply by 0.0167).

Ultrasonography of the abdomen revealed a large hypoechoic area in the right lobe of the liver along with gallstones.

A contrast-enhanced computed tomography (CECT) scan was performed (Figure 1).Sections of the contrast-enhanced computed tomographic scan show a large hypodense area (blue arrowhead) measuring 16 × 11.8 × 12.4 cm, involving segments 4, 5, 6, 7, and 8, along with a gallstone (black arrowhead).


During workup, she was found to have an asymptomatic cystic lesion in the right iliac fossa.

Fifteen months later, a contrast-enhanced computed tomographic (CT) scan of her abdomen showed a progressive 16-mm cystic lesion in close relation to the cecum (Figure 1).

The rest of the colon and small intestine, as well as the adenexa, solid organs, and peritoneum, appeared normal.

There was no other positive radiologic finding except for right partial nephrectomy.

The general physical and abdominal examinations were unremarkable.

The results of other investigations such as a hemogram, liver and kidney function tests, and serum tumor markers, including serum carcinoembryonic antigen levels (0.94 ng/mL [to convert to micrograms per liter, multiply by 1.0]), were within the prescribed normal range.

She had good exercise tolerance, and the results of cardiopulmonary investigations, as a part of preanesthesia workup, were normal.Contrast-enhanced computed tomographic scan of the abdomen showing a cystic lesion (arrowheads) in the right iliac fossa (axial view [A] and planar reconstruction image [B]).


A noncontrast computed tomographic (CT) scan revealed a 15 × 13 × 11-cm heterogeneous cystic mass that appeared to originate from the body of the pancreas and protrude into the gastric wall (Figure 1, A).

Results of CT scan also revealed a 3-cm hypodense lesion on the left lobe of the liver, ascites, and splenomegaly.

Further evaluation with endoscopic retrograde cholangiopancreatography and endoscopic ultrasonography revealed extrinsic compression of the gastric wall from a large mixed solid/cystic lesion with septations and well-defined margins with no clear involvement of the gastric wall.

Analysis of the aspirate showed no tumor cells or mucinous material, and normal carcinoembryonic antigen, CA19-9, and amylase.

A hypodense lesion in segment 3 of the liver is observed (black arrowhead) and also moderate ascites (yellow arrowhead).

A thin, reddish-brown fluid (700 mL) was aspirated from the cystic component.

Fine-needle aspiration was performed on the solid component of the cystic tumor (Figure 1, B-E).A, Coronal view CT scan showing a 15 × 13 × 11-cm complex heterogeneous mass originating from the body of the pancreas (white arrowhead) and protruding into the gastric wall.

B, Hematoxylin-eosin stain (original magnification ×20); C, α-smooth muscle actin immunostain (original magnification ×40); D, CD117 immunostain (original magnification ×40); and D, CD34 immunostain (original magnification ×40).


The patient works as a plumber and attributed his tender rash to a “spider bite” he had incurred while working in an old building.

The patient was afebrile, and physical examination revealed several vesicles with purulent fluid on an erythematous base, at the base of the left thumb (Figure).

There was no lymphadenopathy, lymphangitis, or mucous membrane involvement.

The rash was not found elsewhere on the body.

One day prior to presentation, he went to a local emergency department, where he was prescribed clindamycin and valacyclovir.


He was a former smoker with a 50–pack-year history.

He had been diagnosed with stage I low-grade prostate cancer several years previously.Lung examination showed no wheezing.

Cardiac examination showed a prominent pulmonary component (P2) of the second heart sound.

Noncontrast computed tomography of the chest was unremarkable.An echocardiogram revealed dilated right atrium and ventricle.

Spirometry showed mild airway obstruction.

Duplex ultrasound showed a nonocclusive thrombus in the left femoral and popliteal veins.

Catheterization of the right side of the heart showed a pulmonary arterial pressure of 79/32 mm Hg (mean, 48 mm Hg) with a normal pulmonary capillary wedge pressure.

A ventilation-perfusion (V/Q) scan was obtained (Figure, panel A), followed by pulmonary angiography (Figure, panel B).A, Ventilation/perfusion scan, right anterior oblique view.


His medical history was relevant for acute myeloid leukemia, for which he had received an allogeneic stem cell transplant that was complicated by biopsy-proven grade 3 cutaneous and grade 2 gastrointestinal graft-vs-host disease.

As part of his posttransplant infectious prophylaxis, the patient had been prescribed valacyclovir hydrochloride, voriconazole, and trimethoprim/sulfamethoxazole.

He was also taking tacrolimus hydrate and prednisone for treatment of his graft-vs-host disease.

The patient reported fishing outdoors on a sunny day a few days prior to the onset of these skin lesions.

On physical examination, the patient’s skin was diffusely tan and there were flaccid bullae and erosions on the bilateral dorsal hands, elbows, and knees (Figure, A).

A punch biopsy specimen from a bulla from the upper extremity was obtained (Figure, B).A, Flaccid bulla and erosions on the left dorsal hand at presentation.


He denied associated fevers, chills, night sweats, weight loss, joint pain, cough, difficulty breathing, or chest pain.Findings from the physical examination revealed red-brown monomorphic papules coalescing into plaques on the face, trunk, and extremities (Figure, A and B).

There were thin erythematous plaques with overlying scale on bilateral extensor upper and lower extremities.

A 4-mm punch biopsy specimen from his right upper extremity was obtained and stained with hematoxylin-eosin for histopathologic analysis.

Periodic acid–Schiff and acid-fast bacilli stains were also performed.A, Distribution of papules over the chest, abdomen, and upper extremities.

B, Diffuse distribution of papules over the back with some papules coalescing into plaques.

C, Hematoxylin-eosin stain (original magnification, ×10).


His medications included efavirenz–emtricitabine–tenofovir disoproxil fumarate for his HIV, with the most recent absolute CD4-positive cell count of 341 cells/mm3 (reference range, 263-2045 cells/mm3), and recent completion of an 8-month course of isoniazid treatment after being exposed to tuberculosis.

The lesion started 1 year prior as a tender red nodule on the medial aspect of his first metatarsophalangeal joint, which would enlarge and become painful approximately once a month (Figure, A).

The patient reported that with manipulation, a clear-to-yellow material drained from the lesion.

The patient denied any purulent drainage, additional skin lesions, fever, chills, or other systemic symptoms.

Physical examination demonstrated an erythematous papulonodule without warmth or drainage on the dorsal left great toe.

A punch biopsy was performed (Figure, B, C, and D) to help establish the diagnosis.A, Clinical photograph of slow-growing red papulonodule on the left toe dorsal foot.

B, Biopsy with hematoxylin-eosin (H&E) staining demonstrating acral skin with acute and chronic inflammation within the deep dermis.

C, Higher magnification highlights eosinophilic material surrounding small cocci.

D, Gram staining of the biopsy supported the diagnosis.


Review of systems was significant for heavy periods since menarche 6 months ago.

The patient was described as a shy eighth grader who earns A’s and B’s in school.

She stated that her pain worsened with walking and was minimally relieved with salicylic acid.

It was negative for easy bruising or bleeding.

On physical examination, the patient was noted to be short for her age (1.3 m [4.4 ft]; height in <third percentile), weight (62.5 kg; 87th percentile), and body mass index (35 [calculated as weight in kilograms divided by height in meters squared]; 99th percentile).

She had a relatively short, thick neck with acanthosis nigricans, dry skin, Tanner stage II breasts, and nonpitting edema of her hands (Figure 1A).

Radiography of the hips was performed (Figure 1B), showing bilateral slipped capital femoral epiphysis (SCFE).

B, Photograph of the patient’s hand, which demonstrates nonpitting edema at time of presentation.

Her parents tried to schedule an outpatient appointment for evaluation of her hip pain but had difficulty owing to a lack of a primary care physician.

The patient described normal stooling and voiding habits.

Her family history was significant for hypothyroidism and coronary artery disease in the father, type 2 diabetes mellitus in the maternal great grandmother, and hypothyroidism in the maternal grandmother.

Prior to undertaking definitive surgical correction of the hips, it is important to consider the potential underlying condition.A, Plain film of the hips revealing bilateral slipped capital femoral epiphysis.


Her daughter had died unexpectedly a few days before presentation, and the burial service was scheduled for that day.

Except for the patient appearing struck with severe grief, the results of an initial physical examination, electrocardiography (ECG), and radiography of the chest and her initial laboratory results were all unremarkable.

Her serial troponin T levels were less than 0.01, 0.04, and 0.1 ng/mL (normal range, <0.01 ng/mL [to convert to micrograms per liter, multiply by 1.0]).

An emergency transthoracic echocardiographic biplane volumetric assessment of left ventricular (LV) function is shown in Figure 1 (Video 1 and Video 2).

Twelve hours after readmission, she was asymptomatic, her troponin level had increased to 2.0 ng/mL, and she again underwent ECG (Figure 1).Transthoracic echo biplane volumetric left ventricular function assessment (A-E).

Note the systolic apical akinesis or ballooning in the 4- and 2-chamber systolic still frames (B and D, respectively).

E, Note the extensive T-wave inversions and mild inferolateral ST elevation in the 12-lead electrocardiogram.

She received aspirin, high-intensity statin, and low-dose β-blocker and was given a therapeutic dose of low-molecular-weight heparin subcutaneously.

Serial ECGs were unchanged, and the patient was completely asymptomatic; thus, she was permitted to briefly attend her daughter’s burial service as long as she returned immediately for direct hospital admission.


The patient reported 2 episodes of streptococcal tonsillitis and infectious mononucleosis over the past 8 months, as well as frequent throat soreness and associated difficulty swallowing throughout the past year.

Results from a review of systems were negative for weight loss, fevers, and malaise.

On physical examination, he appeared well and had 2+ cryptic tonsils.

A lobular mass was noted to be extending from the inferior pole of the right tonsil.

The right tonsil had a smooth, pedunculated, flesh-colored mass extending inferiorly.

Both tonsils were removed without complication.

The right tonsil with the attached mass was submitted to the pathology service fresh to allow evaluation by flow cytometry.

Flow cytometry revealed no lymphoid abnormality.

Grossly, the polypoid oval mass measured 2 × 1 × 1 cm, weighed 8.7 g, and had a narrow 0.4-cm-long stalk connecting it to the tonsil (Figure, A).

Histologic sections revealed a polypoid structure composed of a fibrovascular core covered by stratified squamous epithelium (Figure, B).

A subepithelial band of lymphoid tissue was noted (Figure, C) as well as lymphatic channels accentuated by D2-40 antibody (Figure, D).

The patient was scheduled for tonsillectomy for recurrent tonsillitis and tonsillar asymmetry.

His surgery was delayed for several months to accommodate his college schedule.

In the operating room, the tonsils were noted to be 3+ bilaterally.

The mass had enlarged since the prior examination.


His medical history was positive for hypertension, impaired glucose tolerance, dyslipidemia, and hyperthyroidism, and negative for unusual skin pigmentation or cardiac problems.

He denied any history of trauma or surgery around the ear, and reported only a cosmetic problem without pain or discharge.

A laboratory examination revealed only slightly elevated thyroid-stimulating hormone levels (4.80 µU/mL).

A physical examination showed a solitary, 1-cm, dome-shaped, glistening, flesh-colored mass adherent to the posterior groove of the concha ridge (Figure, A and B).

There were no abnormal pigmented skin lesions on the rest of his body, including the face, trunk, and extremities.

Neither a palpable thyroid nodule nor enlargement of hands and feet was identified.

Gross total extirpation of the mass from the surrounding tissues was enabled by meticulous dissection.

Intraoperatively, the 1-cm mass was noted to be gelatinous and glossy with well-demarcated and smooth border.

It was attached to the posterior surface of the concha cartilage but easily separated using blunt dissection.

Unexpectedly, low-viscosity mucoid fluid had smeared on the surface.

The histopathological examination revealed the tumor to be overall hypocellular and composed of bland spindle cells with no mitotic activity in a slightly basophilic myxoid matrix (Figure, C).

The surgical wound uneventfully healed, and no recurrence was noted during the 6-month follow-up period.A, Photograph of a protruding mass on the posterior surface of the auricle.

B, A preoperative close-up of the solitary lesion.

C, Histopathological image showing overall hypocellular stroma with scattered bland spindle cells in slightly basophilic myxoid matrix without mitotic activity.

Numerous blood vessels with collapsed lumina (arrowheads) are also visible.


He was highly functional and denied abdominal pain, dysuria, anxiety, lethargy, and constipation.

Findings from his physical examination were within normal limits.

He did not have any palpable thyroid nodules or cervical lymphadenopathy.

Given his elevated calcium level, parathyroid hormone (PTH) was drawn, and results returned significantly elevated at 1377 pg/mL (reference range, 10-65 pg/mL).

Because of the extremely elevated PTH level, a technetium Tc 99m sestamibi single-photon emission computed tomographic/computed tomographic (SPECT/CT) scan was obtained, which identified a lesion in the location of the right inferior parathyroid gland, measured at 2.2 cm (Figure, A).

In addition, CT showed associated lytic bone lesions of the left mandibular ramus, left scapula, and C5 vertebra (Figure, B).Preoperative imaging of the neck.

A, A technetium Tc 99m sestamibi single-photon emission computed tomographic (SPECT)/computed tomographic (CT) scan.


No stridor or shortness of breath was reported, but he admittedly “hated to run” and was relatively sedentary.

He was overweight (body mass index, calculated as weight in kilograms divided by height in meters squared, 27.2); breathed comfortably at rest; and had a dysphonic, breathy voice.

His voice quality had always been husky, with recent mild exacerbation.

His parents noted intermittent stertor while he was sleeping but no apnea.

Findings from an otolaryngologic examination were normal other than those from flexible laryngoscopy, which demonstrated 2 laryngeal lesions, 1 in the postcricoid interarytenoid region and 1 at the anterior commissure, the latter of which compromised vocal fold approximation.

Vocal fold mobility otherwise appeared normal.

Operative laryngoscopy confirmed a midline, firm, posterior cricoid mass (Figure, A), and a midline, firm, anterior subglottic mass extending into the anterior commissure (Figure, A).

Tracheobronchoscopy revealed a distal tracheal submucosal mass partially obstructing the left mainstem bronchus (Figure, B).

Histopathologic examination demonstrated mucosal infiltration by a monotonous population of cells with round nuclei and abundant granular cytoplasm (Figure, D), positive for S-100 and lightly positive for CD68, with occasional interspersed more darkly staining cells, consistent with histiocytes and giant cells.

Excisional biopsy of the postcricoid and subglottic masses with the carbon dioxide laser was performed (Figure, C).

He had no dysphagia or aspiration manifestations.

Sections stained negative for epithelial, muscle, endothelial, and glial cell immunohistologic markers.A, Direct laryngoscopy of the larynx.

C, Direct laryngoscopy of the larynx following endoscopic carbon dioxide laser ablation of the lesions.

D, Histopathologic image (hematoxylin-eosin, original magnification ×200).


Gestation was complicated by polyhydramnios and gestational diabetes.

In the NICU, choking and desaturation were noted with oral feeding.

At 2 weeks of age, she decompensated secondary to Klebsiella pneumoniae sepsis and was intubated for persistent left lung collapse.

Videofluoroscopic swallow study demonstrated aspiration with all consistencies.

At that time, computed tomographic (CT) imaging was performed, revealing the etiology of her respiratory decompensation (Figure 1).Coronal computed tomographic image of the chest with contrast.

Microlaryngoscopy and bronchoscopy were conducted, and the patient was diagnosed as having a type II laryngeal cleft.


Her medical history was noncontributory.

She was discharged 10 days later with intravenous (IV) antibacterials, sitagliptin, insulin, and oral opiates.The patient returned to the emergency department 2 weeks later with recurrent orbital pain.

Pain rapidly resolved, repeated MRI showed reduced enhancement, and she was discharged with a prednisone taper.Ten days later, the patient returned once again to the emergency department with pain.

Although imaging was stable, examination revealed visual acuity of no light perception OD and new-onset right-sided third, fourth, and sixth cranial neuropathies.

Examination revealed a right afferent pupillary defect, visual acuity of hand motion OD and 20/30 OS, full extraocular movements, and bilateral retinal dot blot hemorrhages.

Magnetic resonance imaging (MRI) showed enhancement along the right intracanalicular optic nerve and adjacent sinuses (Figure).

Glycosylated hemoglobin level was elevated at 10.3% (to convert to proportion of total hemoglobin, multiply by 0.01).

Workup results for sarcoidosis, IgG4-related disease, seropositive and seronegative arthropathies, human immunodeficiency virus, tuberculosis, syphilis, Epstein-Barr virus, and herpes simplex virus types 1 and 2 were negative.T1 fat-suppressed postgadolinium magnetic resonance imaging.

A, Contrast enhancement centered on the right intracanalicular optic nerve (blue arrowhead), involving right orbital apex and posterior ethmoidal sinus (white arrowhead).

B, Enhancement encircling right optic nerve (arrowhead).Orbital apex and sinus biopsy revealed acute and chronic inflammation without neoplasm or microorganisms.

Although examination was stable, MRI showed increased apical enhancement.

The patient was diagnosed as having type 2 diabetes and MSSA sino-orbital infection.

The diagnosis was revised to nonspecific orbital inflammation and the neurology department initiated a 3-day course of IV methylprednisolone.

Specimen cultures grew methicillin-sensitive Staphylococcus aureus (MSSA).


The patient had a persistent Descemet membrane detachment at the 1-day postoperative visit (Figure).

By the 1-week postoperative visit, the patient began to develop blood in the interface, which had not resolved by the 1-month postoperative visit with a visual acuity of 20/50 (Figure).Hemorrhagic Descemet membrane detachment after canaloplasty.

A, At postoperative day 1, a persistent Descemet membrane detachment was observed with no blood in the detachment interface.

B, At postoperative month 1, blood was observed in the interface, which appeared by the postoperative week 1 visit.

The patient elected to proceed with cataract extraction and a lens implant followed by canaloplasty.

The cataract extraction and lens implant went routinely, but during the canaloplasty, a hard-stop obstruction was encountered at the 7-o’clock position during the passage of the illuminated catheter.

A viscoelastic material was injected into the Schlemm canal to attempt to break the obstruction.

The procedure was aborted, a 50% air bubble was injected into the eye, and the physicians recommended that the patient maintain a face-up position.

This resulted in a large inferior nasal Descemet membrane detachment.

A washout was not performed because of concerns of further exacerbating the detachment or inducing a hemorrhage.


Her medical history included aortic repair and cervical spine abnormalities.

The patient’s family history was unknown because she was adopted.

Her medications included oral aspirin and irbesartan.Visual acuity was counting fingers OD (without correction) and 20/30 OS (with correction).

Her symptoms began 6 hours before presentation after she had been napping face-down on her desk for 30 minutes.

The right pupil was corectopic and fixed at 5 mm while the left pupil was round and reactive; no afferent pupillary defect was seen.

Intraocular pressures (IOPs) were 43 mm Hg OD and 14 mm Hg OS.

Examination of the right eye revealed conjunctival injection, corneal stromal edema with Descemet folds and lenticulocorneal touch, a shallowed anterior chamber, and a lens protruding through the pupil with the superior edge in the anterior chamber (Figure 1A).

The posterior view was limited to red reflex.

The left eye was unremarkable except for a superotemporally subluxated lens (Figure 1B).Slitlamp photography.

Her lenses had been observed for years without intervention.

Her ocular history was pertinent for high myopia and contact lens wear, a posteriorly dislocated crystalline lens suspended in the vitreous cavity in her right eye, and a superotemporally subluxated crystalline lens in her left eye.

A, Right eye demonstrates dislocation of the superior crystalline lens into the anterior chamber.

B, Left eye shows superotemporal subluxation of the crystalline lens.


Preoperative Snellen visual acuity was 20/40 and intraocular pressure (IOP) was 36 mm Hg; on postoperative day 1, IOP was 10 mm Hg and visual acuity fell to count fingers.

Slitlamp examination of the left eye revealed an elevated bleb over the Ahmed plate with no leakage.

The cornea was clear and compact, and there was a well-positioned tube overlying the iris superotemporally.

The anterior chamber was deep with trace inflammation.

Dilated fundus examination revealed disc edema, peripapillary hemorrhages, and diffuse intraretinal hemorrhages throughout the posterior pole and periphery (Figure 1).

Spontaneous venous pulsations were not appreciated.


Physical examination results were normal except for a nontender distended and tympanic abdomen.

For the past year, the patient had been investigated for hypochromic microcytic anemia.

As part of the investigation, he underwent an esophagogastroduodenoscopy and a colonoscopy, which revealed a normal-looking bowel with no obvious source of bleeding visualized.

The patient claimed not to have passed a bowel movement containing the capsule.

Results of routine laboratory testing were essentially normal except for a low level of hemoglobin (8.1 g/dL [to convert to grams per liter, multiply by 10]), with no leukocytosis noted.

Contrast-enhanced abdominopelvic computed tomography demonstrated a small-bowel obstruction, with a transition zone located at the mid-jejunum.

An intraluminal hypodense mass was demonstrated in this area and appeared to be the cause of the obstruction.

Adjacent to this area, the capsule was clearly demonstrated in a dilated proximal loop of the small bowel (Figure 1).Computed tomographic scan showing an intraluminal hypodense mass (pink arrowhead) causing a small-bowel obstruction.

Adjacent to this area, the endoscopic capsule (asterisk) is demonstrated in a proximal dilated loop of the small bowel.

Further investigation involved capsule endoscopy, which was ingested 3 days prior to his emergency department admission.


Although previously healthy, he reported some hospitalizations for nonspecific abdominal pain during his infancy.

Colicky abdominal pain started after eating cornflakes in the morning, then regressed spontaneously during the day.

A relapse of the symptoms occurred the morning after and worsened during the day, with the onset of nausea as well.

His bowel was initially open to gas and normal stools but, afterward, only to gas.

A physical examination revealed a soft and nondistended abdomen, with localized tenderness and guarding in the epigastric region and in the right lower quadrant.

Hyperactive bowel sounds could be heard.

The results of a digital rectal examination were normal.

His vital signs were as follows: an axillary temperature of 37.3°C, a pulse rate of 71 beats per minute, and blood pressure of 120/80 mm Hg.

The results of a urinalysis were negative; blood test results were within the normal limits, except for an elevated white blood cell count of 13 000/μL (to convert to ×109 per liter, multiply by 0.001), an unconjugated bilirubin level of 1.2 mg/dL (to convert to micromoles per liter, multiply by 17.104), an increased aspartate aminotransferase level of 52 U/L (to convert to microkatals per liter, multiply by 0.0167), and an alanine aminotransferase level of 136 U/L (to convert to microkatals per liter, multiply by 0.0167).

His C-reactive protein level was 10 mg/L (to convert to nanomoles per liter, multiply by 9.524).

A plain radiograph of his abdomen was negative for air-fluid levels and for signs of perforation.

Further evaluation of his abdomen using ultrasonography revealed hepatic steatosis and, in the right lower quadrant, thickening of the cecal wall and a hypoechoic area 33 mm in diameter with blurred margins (Figure 1).Ultrasonographic images of the right iliac fossa (A) and the right lower quadrant of the abdomen (B) with thickened cecal wall (a) and hypoechoic area 33 mm in diameter with blurred margins (b).

For this reason, the patient autonomously took 2 tablets of hyoscine butyl bromide without any benefit.


The mass continued to enlarge and she developed progressive generalized weakness, slowed speech, and focal right-handed weakness.

Acute onset of headache and decline in mental status prompted presentation to the ER.

She initially was seen by her primary care practitioner, where neurological examination was nonfocal.

On examination an 8-cm left parietal mass was tender to palpation.

Brain magnetic resonance imaging showed a large enhancing left frontal-parietal mass extending both extracranially and intracranially into the superior sagittal sinus (Figure).

There was left frontal, parietal, and temporal bone involvement, and left dural thickening.

Intracranially, there was adjacent vasogenic edema and associated mass effect.

Computed tomographic (CT) scans of the chest, abdomen, and pelvis revealed a ground-glass sclerotic lesion of the right sacrum.

Treatment with 4 mg of dexamethasone was initiated intravenously.

All biochemical laboratory results were normal.

Owing to involvement of the superior sagittal sinus and progressive growth, an ultrasound-guided core needle biopsy was performed.Large extracranial and intracranial mass extending into the superior sagittal sinus, brain, soft tissue, and bone, with left dural thickening as visualized on T1 postcontrast imaging coronal (A) and sagittal (B) views.

Axial view of brain edema as seen on T1-weighted (C) and T2-weighted imaging without contrast (D).


His medications included amlodipine, hydrochlorothiazide, and ezetimibe.

He worked outdoors for an oil drilling company.

He denied recent travel out of the country.

He described the pain as akin to stepping on a sharp object and characterized it as moderately intense, sharp, continuous, and without radiation.

After first noticing the pain, he had examined the inside of his boot but did not identify anything that would account for the tenderness.

Physical examination revealed 2- to 3-mm grouped vesicles on an erythematous base in a discrete area of the sole of the right foot (Figure) and no additional rashes elsewhere on the body.

Vesicular fluid was sent for analysis.Left, Grouped vesicles on sole of patient’s right foot.

He reported no fevers, chills, or malaise.

He denied history of human immunodeficiency virus (HIV), hepatitis, chickenpox, or recurrent infections.


He took no prescribed medications but had taken an ibuprofen pill a week prior for a mild headache; his symptoms started 3 days after that.

He had no sick contacts or recent travel and reported no use of alcohol or tobacco.On examination, he was febrile (38.9°C) and tachycardic but normotensive.

He noticed multiple acneiform papules and pustules around the neckline that progressed to involve the face, torso, and limbs.

Clusters of pustules, approximately 1 mm in diameter, were present over an erythematous base overlying the face, neck, chest, back, and arms (Figure).

Laboratory analysis revealed values within normal ranges for hemoglobin (13.1 g/dL) and platelet count (186 ×109/L); an elevated white blood cell count (22.3 x109/L; 82% neutrophils, 9% lymphocytes, 5% eosinophils, 4% monocytes); erythrocyte sedimentation rate of 58 mm/h (reference range, 0-20); C-reactive protein level of 232 mg/L (2210 nmol/L) (reference, <5 mg/dL [48 nmol/L]); and normal renal and hepatic function.

Gram stain of the pustule fluid revealed no organisms, and cultures of the blood and pustule fluid were without growth.


He reported smoking marijuana and denied tobacco and intravenous drug use.

He is sexually active, with multiple male partners.On examination, he was alert and oriented, with a temperature of 36.3°C, blood pressure of 131/90 mm Hg, heart rate of 94/min, respiratory rate of 18/min with pulse oximetry of 98% on ambient air, and a body mass index of 28.1 (calculated as weight in kilograms divided by height in meters squared).

His mother reported that he had experienced personality changes.

Neurologic examination was significant for hyperreflexia in all extremities, bilateral shoulder weakness, and right-sided pronator drift.

Laboratory results showed a white blood cell count of 3600/μL (73.5% neutrophils, 16.4% lymphocytes, 7.8% monocytes, 1.4% eosinophils, 0.6% basophils); the remainder of the complete blood cell count and comprehensive metabolic profile was unremarkable.

The result of a human immunodeficiency virus (HIV) type 1 antibody test was positive, and CD4 cell count was 73/mm3.

Results of serologic testing for Toxoplasma gondii IgG were negative.

Computed tomography (CT) imaging of the brain showed a left caudate nucleus and lentiform nucleus lesion with mass effect on the left lateral ventricle (Figure, left panel).

Fluid-attenuated inversion recovery magnetic resonance imaging (MRI) of the brain confirmed the presence of an aggressive mass lesion extending through the corpus callosum (Figure, right panel).

CT of the chest, abdomen, and pelvis and spinal MRI were unremarkable.Left, Computed tomography scan of the brain, axial view, with contrast.


The patient’s medications included methimazole and atenolol, which she had been taking since her diagnosis of Graves disease 1 year prior.

She had not taken other over-the-counter medications or supplements.

She reported chills, bilateral lower extremity swelling, and several small, painful, “pimple-like bumps” appearing on her bilateral lower legs, which ulcerated several days later.

Physical examination of her bilateral lower extremities revealed pitting edema and multiple discrete, round, dry ulcers, most with central eschars, dusky gray borders, and collarettes of scale (Figure, A).

Results from the initial laboratory workup revealed elevated levels of C-reactive protein and increased erythrocyte sedimentation rate.

A punch biopsy specimen of an ulcer edge demonstrated a mid- and deep dermal marked lymphohistiocytic infiltrate with neutrophils and focal abscess, suggesting a nonspecific infection.

No pathergy at the previous punch biopsy site was observed.

The ulcers progressed despite a recent course of trimethoprim-sulfamethoxazole for cultures growing methicillin-sensitive Staphylococcus aureus.

The patient was initially treated with cephalexin, mupirocin, and conservative wound care, but the eruption later worsened, with new lesions appearing on her right leg.

During follow-up, an intact 4-mm papular lesion was identified on her lower right leg (Figure, B).

A biopsy of the new intact papule was performed (Figure, C and D).A, Discrete, round, dry ulcers with central eschar and raised borders on left lateral lower leg.

B, A new, 4-mm intact papule on right medial lower leg.

C and D, Histopathological images from punch biopsy specimen of the papule, hematoxylin-eosin.


On review of systems, he endorsed photosensitivity during childhood only.

He denied any history of blistering, angioedema, dysphagia, hoarseness, dyspnea, or weight loss.

Physical examination revealed multiple skin-colored, waxy, smooth and variably verrucous papules and plaques on the dorsum of the nose and hands (Figure, A and B).A, Skin-colored, waxy, smooth papules on the dorsum of the nose.

B, Waxy papules coalescing into verrucous-appearing plaques on the dorsal surface of the hand.

C, Nodular deposits (hematoxylin-eosin, original magnification ×20).

D, Dermal deposits (periodic acid–Schiff–diastase staining, original magnification ×4).Initial laboratory evaluation, including complete blood cell count, blood chemistry panel, liver function tests, serum/urine protein electrophoresis, and urine porphyrins, had unremarkable results.

A punch biopsy specimen was obtained from the left dorsal hand for further evaluation (Figure, C and D).

He had no improvement following treatment with class 1 topical corticosteroids and keratolytic emollients.


He had initially developed rhinorrhea, cough, and low-grade fever 2 weeks prior.

Over the next several days, he developed worsening fever, chills, and blisters and bullae on the arms, legs, and face.

Physical examination revealed numerous pink to violaceous macules, papules, and patches on the palms, dorsal hands, forearms, elbows, feet, and distal lower extremities (Figure, A and B).

Fewer, scattered macules were on the face, neck, trunk, upper legs, upper arms, and scrotum.

Dusky bullae were seen on the bilateral dorsal aspect of the hands, fingers, toes, and heels.

In the oral cavity, there was 1 violaceous macule on the right buccal mucosa and a few linear, horizontal, white lines on the left buccal mucosa.

Laboratory findings revealed a white blood cell count of 25 600/μL (reference range, 3700-10 500/μL) and a platelet count of 12 400/μL (reference range, 15 000-40 000/μL).

Results from a Tzanck smear from the initial lesion on the right dorsal aspects of the third finger were negative for multinucleated giant cells.

The patient was treated with azithromycin and subsequently developed few small, pruritic blisters on the hands and feet.

He went to an urgent care facility and was diagnosed as having bronchitis and prescribed doxycycline and diphenhydramine.

He was admitted to the hospital, and the dermatology service was consulted.

A punch biopsy was performed on the anterior side of the thigh (Figure, C and D).


He did not take medications, and he drank socially.

He frequently fell and had difficulty climbing the stairs.

He stated he had hearing problems, but with further questioning, it was discovered he actually had difficulty deciphering speech.

One month later, his condition progressed; he required a wheelchair, declined cognitively, and developed personality changes.

He developed seizures and was admitted to a hospital.

His mental status continued to progressively decline, and he became completely mute; he could only open his eyes and follow commands to protrude his tongue but was unable to move his eyes or any limbs.

His neurological examination showed mild cerebellar ataxia without myoclonus or parkinsonism.

His Montreal Cognitive Assessment score was 28 of 30 points (2 points deducted from “delayed recall”).

Pure tone audiometry showed no conductive or sensorineural hearing loss.

Brain magnetic resonance imaging (MRI) revealed no abnormal signal, including from the pons and cerebellum.

A computed tomography scan of the chest, abdomen, and pelvis showed no malignancy.

Results from cerebrospinal fluid (CSF) testing were normal without oligoclonal bands and a normal level of protein 14-3-3.

He had no paraneoplastic antibodies in his serum or CSF.

Results from electroencephalogram (EEG) were normal.

Electroencephalogram showed diffuse slowing.

He did not have dizziness on standing nor bowel or bladder incontinence.

He had no family history of imbalance problems.


She had an ovarian cyst, but her medical history was otherwise unremarkable.

There was no history of recreational drug use, tobacco or alcohol consumption, or sexually transmitted diseases.

A postmortem brain examination reported the presence of multitudinous corpora amylacea, but no specific neuropathological diagnosis was made.Neurologic examination of the patient during the initial consultation revealed a Mini-Mental State Examination score of 24 of 30 (points were missed on attention, calculation, and abstraction), and the patient was abulic, but it was otherwise unremarkable.

Her mother had died at 32 years of age, after having a chronic neuropsychiatric illness, culminating in a rapidly progressive dementia.

Magnetic resonance imaging (MRI) of the brain showed scattered small foci of T2 hyperintensity in the white matter affecting the corpus callosum, bifrontal, and biparietal regions, associated with bifrontal and bitemporal atrophy (Figure1A).

No lesions were identified in the cerebellum or brainstem.

Laboratory investigations including complete blood cell count with differential; electrolytes; liver, renal, and thyroid function tests; vitamin B12 and folate levels; vitamin E; cholesterol and triglycerides; amino acids (serum and urine); syphilis and human immunodeficiency virus serologies; a paraneoplastic panel; and erythrocyte sedimentation rate were unrevealing.

Cerebrospinal fluid analysis showed a slight protein elevation, but no pleocytosis, oligoclonal bands, or elevated immunoglobulin G synthesis rate.

Arylsulfatase activity was normal.A, Axial T2 sequence showing bifrontal cerebral convexity sulcal enlargement greater than expected for patient's age, punctate bifrontal subcortical fluid-attenuated inversion recovery (FLAIR) hyperintense foci (right greater than left) are noted.

B, Brain magnetic resonance imaging axial and coronal T2 FLAIR sequence showing more pronounced global cerebral atrophy, progression of the subcortical FLAIR hyperintense lesions in the frontal predominant distribution, and also affecting the periventricular and deep white matter into the subcortical areas.


He lived with his parents, aunt, uncle, and 3-year-old cousin.

His cousin received a diagnosis of chicken pox 6 months prior, but had persistent skin lesions and pruritus.

The dermatology department was consulted for a diffuse truncal rash concerning for a drug rash given the initiation of several antibiotics during his hospital stay.

On admission, he was noted to have several scattered 2-mm pink papules on his trunk and extremities and a high density of blue-gray macules on his trunk.

The duration of his skin lesions was unknown.

His mother had several pruritic pink papules on her bilateral forearms, which she suspected were from sand fleas.A skin examination revealed a Fitzpatrick skin phototype I and numerous ill-defined 0.5- to 1-cm nonblanching blue-gray macules on his trunk, a few of which contained a central 2-mm dome-shaped pink papule (Figure).

There were also several similar pink 2-mm papules and hemorrhagic punctae scattered on his trunk, extremities, and scalp.Multiple ill-defined 1-cm blue-gray macules, a few of which contain a central 2-mm dome-shaped pink papule (black arrowhead) on the chest and abdomen.

He was up-to-date on all of his immunizations.


Results from basic laboratory testing, including serum electrolytes, troponin, and complete blood cell counts, were unremarkable.

Routine electrocardiogram (ECG) at presentation (Figure 1A) revealed sinus rhythm with first-degree atrioventricular (AV) block, borderline right axis deviation, and right bundle branch block.

Resting echocardiogram revealed normal left ventricular size and function, mild concentric left ventricular hypertrophy, and no significant valvular abnormalities.

Shortly after the patient reached a peak heart rate of 130 beats per minute at 9 minutes 28 seconds of exercise (Figure 1B), his heart rate abruptly decreased to 95 beats per minute, with evidence of AV block (Figure 1C).

Subsequent coronary angiogram revealed no obstructive epicardial coronary artery disease.A, Resting electrocardiogram (ECG) shows first-degree atrioventricular block and right bundle branch block.

The patient was sent for exercise stress echocardiography.

B, ECG rhythm during stage 3 of Bruce protocol.

C, ECG during peak exercise with abrupt decrease in heart rate.


His medical history was significant for an unusual gait with anterior displacement of the pelvis and hips since he began walking at 10 months of age.

Since then he had experienced intermittent episodes of limping accompanied by pain in both femurs.

Radiographs revealed no active fractures but did show extensive bony changes in the long bones and the skull.

On examination, mild asymmetry of the head was noted on palpation with prominence of the right temporal and parietal area.

On limited endoscopic examination, mucosal edema and mucopurulent discharge were observed in the left more than the right naris.

An endoscope could not be advanced posteriorly owing to patient discomfort and right-sided sinonasal obstruction.

Multiple macules with irregular borders were observed on the lower back, upper buttock, upper chest, upper neck, and right postauricular area.

There was also a varus deformity of the lower extremities with mild discrepancy in leg length.

An audiogram showed bilateral conductive hearing loss with a flat tympanogram.

A computed tomographic (CT) scan of the head and neck revealed heterogenous opacification of the right ethmoid and maxillary sinuses with fluid accumulation within the bilateral mastoid bones and middle ears.

His thyroid-stimulating hormone level was 0.19 mIU/mL (reference range, 0.5-2.4 mIU/mL), free thyroxine level was 1.3 ng/dL (0.9-1.6 ng/dL), and free triiodothyronine levelwas 4.6 pg/mL (3.4-5.1 pg/mL) (Figure).Computed tomographic images; bone windows.

Previous treatment with montelukast provided symptomatic relief; however, this was discontinued owing to behavioral changes.


Her perinatal and medical histories were otherwise unremarkable.

On examination, a 1-cm mobile, well-defined, nontender mass over the nasal dorsum was noted.

Findings from the rest of the otolaryngologic examination were normal.

Careful inspection and palpation after excision revealed no bone involvement or intracranial extension.

Histopathologic images of the excised nasal mass are shown in the Figure.Histopathologic images (hematoxylin-eosin) from the nasal mass, showing a disordered admixture of mature skeletal muscle, mature adipose tissue, and skin adnexal structures.

The preoperative plan was to excise the nasal dermal lesion and carefully inspect for any tracts extending intracranially, which would then warrant imaging and a revised intracranial approach.

Intraoperatively, an incision was made above the mass and carried through the subcutaneous tissue to expose a mass with a white wall and pink tissue within.

The 0.8 × 0.5 × 0.3-cm mass was freed from the surrounding tissue and excised down to the nasal bone.

The neurosurgical team removed the superficial scalp cyst, which was found to be an epidermoid cyst.

Given the superficial nature of the nasal cyst on examination and in an effort to minimize radiation and anesthetic exposure for the patient, computed tomographic and magnetic resonance imaging were deferred.


His medical history was notable for congestive heart failure, coronary artery disease, and gout.

He noted a 10-pack-year smoking history and Agent Orange exposure.

Several days into his stay, he complained of having a hoarse voice for the past year.

He denied dyspnea, dysphagia, and reported no weight loss.

On examination, he had no palpable neck masses.

He underwent a flexible laryngoscopy, which showed an exophytic yellow-white mid-membranous subepithelial lesion on the bilateral true vocal folds (Figure, A).

On stroboscopy, there was decreased vibratory amplitude bilaterally and moderate phase asymmetry.

Grossly, pathologic findings showed a chalky white lesion.

Histopathologic review showed submucosal amorphous eosinophilic material (Figure, B).

The amorphous material was negative for Congo red (Figure, C).

Examination of an unstained slide showed needle-shaped birefringent crystals (Figure, D).

With this nonspecific but benign diagnosis, and an inability for the patient to go to the operating room secondary to his comorbidities, he agreed to undergo office potassium titanyl phosphate laser photoangiolysis of the lesions to improve vocal fold contour and vibration.

More of the chalky material was expressed as the overlying mucosa ablated with very little energy delivered.

Several weeks later, he underwent an indirect laryngoscopy and vocal fold biopsy in the office.

On follow-up, the lesions persisted but were less bulky.A, Indirect laryngoscopy showing exophytic yellow-white mid-membranous subepithelial lesion on the bilateral true vocal folds.

D, Unstained slide showing needle-shaped birefringent crystals.


He had a history of hypertension and had been prescribed a blood pressure medication (the name of which he could not remember), but he had not been taking it for weeks.

He had no other pertinent medical history.

The swelling had been progressively increasing over the first 3 days but had remained stable over the past week.

He denied any history of similar symptoms, pain, fever, odynophagia, or difficulty breathing.

He complained of some difficulty swallowing owing to the swelling.

Physical examination revealed significant, soft, nontender swelling of the floor of mouth bilaterally causing elevation of the tongue (Figure, A).

There was no edema of the tongue or lips.

The neck was soft, nontender to palpation, and without palpable lymphadenopathy.

Computed tomographic (CT) imaging of the soft tissues of the neck with intravenous contrast showed a 6.3-cm anteroposterior × 6.9-cm cranial caudal × 4.8-cm transverse cystic nonenhancing mass centered in the root of the tongue, with elevation of the oral aspect of the tongue and some downward displacement of the floor of mouth (Figure, B and C).

The sublingual glands were compressed but otherwise appeared normal, and the sublingual spaces were effaced.

Surgical excision via an intraoral approach yielded a smooth, well-circumscribed mass.


The patient recalled that the lesion was initially discovered 4 years prior to presentation at a different facility.

He denied any previous ocular trauma and his medical and surgical histories were noncontributory.

Results of a complete review of systems were otherwise negative.At presentation, his visual acuity was 20/20 OU.

Pupils and extraocular motility were intact.

The anterior segment slitlamp examination was unremarkable in both eyes.

A dilated retinal examination of the left eye revealed the findings depicted in the Figure, A.

Spectral-domain optical coherence tomography was subsequently performed (Figure, B).

No retinal pigment epithelium (RPE) atrophy, macular edema, retinal exudation, or subretinal fluid was noted.

Posterior segment examination and imaging of the right eye demonstrated no abnormalities.A, Fundus photograph of the left eye demonstrating a nodular, darkly pigmented lesion with well-defined margins located inferonasal to the foveola.


Aside from mild hyperopic astigmatism, there was no significant ocular, medical, or family history.

The patient was not taking any prescription or over-the-counter medicines; however, she had recently used a short-term course of oral antibiotics for a tooth infection.On examination, Snellen visual acuity with correction was 20/20 OD and 20/25 OS.

The patient denied any change in vision, scotomas, transient visual obscurations, dimming of light, photophobia, or pain.

She denied headache, pulsatile tinnitus, weight gain, joint pain, lethargy, decreased appetite, fever, or chills.

Confrontation visual fields and ocular motility were normal.

External examination was unremarkable, and slitlamp examination revealed normal eyelids, conjunctiva, and sclera.

There were 1+ anterior chamber white blood cells and trace anterior vitreous cells bilaterally.

Posterior segment examination revealed 360° nerve hyperemia with elevation of nasal disc borders bilaterally (Figure 1), without vitreous haze, snowballs, or snowbanks.

There was no vascular sheathing, choroidal granulomas, or chorioretinal scarring in the periphery.Fundus photography from initial consultation showing 360° nerve hyperemia with mild elevation of the nasal disc border bilaterally.


He complained of right ocular irritation, redness, and discharge that had begun shortly after removal of the eyelid lesion 2 years prior.

Eight months prior to referral, he developed a progressive right blepharoptosis followed by recurrent corneal erosions, which was treated with bandage contact lenses.On examination, he was found to have complete ptosis of the right eyelid, asymmetric erythema of the eyelid margin, and meibomian gland dysfunction, as well as papillary conjunctivitis with thickening of his medial right lower eyelid (Figure 1).

His left eyelid had minimal inflammation.

He was also noted to have right-sided subconjunctival hyperemia with a 7.2 mm × 2.5 mm corneal erosion and 2 mm of pannus on the right eye.A photograph of the patient demonstrating asymmetric clinical findings and scarring of the medial canthal area from previous biopsy.

A, Fullness of the right upper eyelid with ptosis.

B, Severe right-sided meibomian gland dysfunction, thickening of the medial lower eyelid, and subconjunctival hyperemia.

He was prescribed a treatment regimen for blepharitis, which subsequently included topical tobramycin-dexamethasone ointment, with little improvement in symptoms.


Medical history included well-controlled type 2 diabetes treated with metformin and sitagliptin.Best-corrected visual acuity was 20/20 OU.

Review of systems was positive for a skin rash on the upper arms and trunk, which he noticed 2 months earlier.

There was a barely perceptible right relative afferent pupillary defect.

Anterior segment examination was normal.

On ophthalmoscopy, there were few vitreous cells with no detectable haze and optic nerve head edema grade IV on the Frisén scale, with peripapillary flame hemorrhages in the right eye (Figure); the optic nerve was normal on the left.

Automated perimetry using the 24-2 pattern and Swedish interactive threshold algorithm strategy (Humphrey Field Analyzer; Carl Zeiss Meditec) was normal in each eye and optical coherence tomography showed marked elevation of the peripapillary retinal nerve fiber layer on the right.


The patient reported undergoing an appendectomy when he was 10 years old.

The patient did not have a fever and he was hemodynamically normal.

Laboratory workup revealed leukocytosis with a white blood cell count of 11 600/μL (to convert to ×109/L, multiply by 0.001).

Physical examination revealed tenderness in the right lower quadrant without overt signs of peritonitis.

Abdominal computed tomography was obtained (Figure 1).Coronal computed tomography scan showing calcification in the terminal ileum with surrounding enteritis (arrowhead).


Intriguingly, the heartburn was most severe while she was sitting up but lying supine relieved the symptoms.

Paradoxically, dysphagia was worse while she was reclining and partially resolved in the upright position.

The physical examination was normal while laboratory workup revealed mild anemia.

Subsequently, further workup with an esophagram revealed a 2.7-cm filling defect at the gastroesophageal junction, suggestive of extrinsic compression.

Computed tomography demonstrated a 2.5-cm low-attenuation ovoid lesion at the gastroesophageal junction (Figure 1A).

Considering a diagnosis of gastric tumor, she then underwent upper endoscopy that showed a mobile smooth-surfaced polypoid subepithelial lesion at the posterolateral wall of the gastroesophageal junction.

Mucosal biopsies were performed, which were consistent with normal squamous mucosal epithelium.

Following this, endoscopic ultrasonography was performed, which demonstrated an anechoic, loculated, subepithelial lesion located within the submucosa of the distal esophagus (Figure 1B).

The endosonographic borders were well defined without invasion into the nearby structures.

Manometry and 24-hour pH testing revealed preserved motility but abnormal acid exposure of the distal esophagus.

Ultrasonography of the liver, gallbladder, and pancreas revealed no abnormalities.Diagnostic imaging for the patient.

A, Contrast computed tomogram (CT) revealing a mass at the gastroesophageal junction protruding into the cardia of the stomach.

B, Endoscopic ultrasonography demonstrated a cystic lesion originating and localized to the wall of the esophagus but not invading local structures.

She was administered a trial of acid-suppression therapy for 6 weeks, which transiently alleviated heartburn but the dysphagia persisted.

The mass flopped back and forth into the gastroesophageal junction.


She reported pain with chewing, which led to a 5-kg weight loss, but denied trismus.

On examination, there was craniofacial deformity with left facial preauricular swelling.

The left parotid was firm and a fixed mass was felt throughout the left preauricular space, expanding into the temporomandibular joint area and zygoma.

Soft-mobile lymphadenopathy was palpable in the left neck, and a firm, adherent, immobile right supraclavicular mass extended under the clavicle.

Cranial nerve examination findings were normal.Magnetic resonance imaging and computed tomography of the neck showed a 3.2 × 4.4 × 2.8-cm lobulated enhancing paramandibular mass extending into the left masticator space and infratemporal fossa, with frank erosion of the left mandibular condyle (Figure).

Several enlarged lymph nodes were noted.

Computed tomography of the chest showed a 4.6 × 3.2 × 3.5-cm supraclavicular mass with infiltration of the surrounding fat and scalene muscle and extension into the chest wall and axilla.

Positron emission tomography showed a large metabolically active left masticator space mass (standardized uptake value for body weight at maximum counts per pixel [SUVmax], 18.2) and previously mentioned pathologic nodes.

The right supraclavicular mass was also metabolically active (SUVmax, 19.4), and metabolically active right axillary, left mediastinal, and intra-abdominal lymphadenopathy was also noted.A, Axial computed tomography of the head and neck showing a lobulated paramandibular mass (arrowhead).

B, Magnetic resonance imaging of the brain showing a lobulated paramandibular mass (arrowhead).

C, Coronal computed tomography of the chest demonstrating a paramandibular mass (arrowhead).

D, Coronal computed tomography of the chest demonstrating a supraclavicular mass (arrowhead).


He had no other symptoms and reported taking no medications.

Findings on skin examination included near-complete loss of eyebrows and eyelashes, a scaly plaque on the right shin, and mild bilateral erythema of the earlobes, distal fingers, and toes (Figure).


She was a nonsmoker and took no medications.

A review of her social history revealed that she was working at a horse stable and rode for many hours a day.

She reported that the eruption was worsening during the winter months.

She had not experienced any fevers, chills, joint pains, recent weight loss, or any other constitutional symptoms.

On physical examination, distributed over the bilateral posterior lateral thighs, there were several arcuate to circinate erythematous to violaceous plaques.

There were also several focal areas of ulceration with overlying scale-crust (Figure, A and B).

A biopsy was performed during this initial visit (Figure, C and D).


He reported a medical history of rheumatoid arthritis (RA), chronic obstructive pulmonary disease, chronic kidney disease, and heavy tobacco use.

The patient denied any recent changes to his medications or recent travel.

The lesions started spontaneously as erythematous tender papules with white purulent discharge.

No other household members were affected.Physical examination of the left lower extremity revealed a 2.5-cm keratotic plaque with central ulceration that expressed a straw-colored fluid.

The limb was dusky with scattered hyperpigmented patches and surrounding erythema (Figure, A and B).

Both lower limbs exhibited pitting edema of 1+ severity, and negative Homans sign bilaterally for deep vein thrombosis.

Fungal and bacterial culture samples were negative.

Rheumatoid factor was elevated at 198 IU/mL, and other laboratory data were noncontributory.

A punch biopsy specimen was obtained from the edge of the lesion (Figure, C and D).A, Left pretibia on initial visit.

He recently failed a course of amoxicillin and trimethoprim-sulfamethaxazole initiated by his primary care physician.

C, Periodic acid–Schiff stain (original magnification ×4).

D, Hematoxylin-eosin stain (original magnification ×20).


The patient had occasional numbness in both legs in stocking distribution, attributed to diabetic neuropathy.

The patient reported no fevers, chills, weight loss, or night sweats.

He had long-standing, intermittent dyspnea on exertion, but no baseline shortness of breath, orthopnea, or bilateral lower extremity edema.

Physical examination revealed a 4.5 cm waxy, nontender, smooth-surfaced, cobblestoned plaque located in the sulcus between the second and fourth toes (Figure, A).

A biopsy specimen obtained by the patient’s podiatrist was sent to the dermatopathology laboratory for review.A, Clinical presentation revealed smooth-surfaced cobblestoned plaque that was asymptomatic.

B, Hemotoxylin-eosin stain (original magnification ×100) results show a diffuse infiltrate of pale eosinophilic material with prominent clefts.

C, Congo red stain (original magnification ×200).

The patient was then referred to a dermatology specialist for further treatment.


His medical history was significant for seizures and behavioral disturbances managed with stable doses of lamotrigine and chlorpromazine.

Associated symptoms included fatigue, decreased appetite, abdominal discomfort, and intermittent scleral icterus noted by his mother.

His lower extremities were tender to palpation and he resisted passive ranging of his joints.A, Markedly edematous and friable gingiva.

On physical examination, he was nonverbal but in no visible distress.

There was hemorrhagic gingivitis (Figure, A) without cervical lymphadenopathy, and the bilateral lower extremities had marked asymmetric pitting edema, large ecchymoses on the thighs, ankles, and feet (Figure, B), and scattered petechiae (Figure, C).

C, On the lower leg, perifollicular hemorrhage and corkscrew hairs (arrowhead) are present.Initial laboratory workup revealed microcytic anemia with hematocrit level of 22.8% (to convert to proportion of 1.0, multiply by 0.01), mean corpuscular volume of 75.9 µm3 (to convert to femtoliters, multiply by 1), white blood cell count of 5.9/µL (to convert to ×109 per liter, multiply by 0.001), platelet count of 248 ×103/µL (to convert to ×109 per liter, multiply by 1), elevated erythrocyte sedimentation rate of 52 mm/h (to convert to millimeters per hour, multiply by 1), and C-reactive protein level of 5.5 mg/L (to convert to nanomoles per liter, multiply by 9.524), with hypoalbuminemia of 3.4 g/dL (to convert albumin to grams per liter, multiply by 10), but normal creatine kinase level of 83 U/L (to convert to microkatals per liter, multiply by 0.0167), and unremarkable activated partial thromboplastin time and international normalized ratio.

Ultrasonography of the lower extremities showed soft tissue swelling without thrombosis.

Lower extremity magnetic resonance imaging/magnetic resonance angiography showed diffuse round mildly enhancing bone marrow lesions without vascular changes.

One week prior, a dentist diagnosed acute necrotizing gingivostomatitis based on gum inflammation and bleeding, prompting treatment with metronidazole and penicillin.

There was no history of trauma, fevers, hematuria, or hematochezia or family history of coagulation disorders.


He denied substance abuse or addiction.The abdomen was soft with hypogastric tenderness.

The pain was constant in the hypogastric region, with no radiation.

He did not have bowel movements for 5 days but passed gas normally.

He did not complain of nausea or vomiting.

There was no abdominal distension, guarding, or rebound tenderness.

Neither hernia nor surgical scars were seen on examination, and vital signs and results from laboratory tests were normal.

An axial contrast computed tomography (CT) scan of the abdomen was performed (Figure 1).A, A computed tomography scan of the abdomen did not show abnormal intestinal air-fluid levels nor free peritoneal air.

B, A computed tomography scan revealed that the rectum was not empty.


It grew rapidly throughout the past year, becoming increasingly painful and bleeding easily with light contact.

Clinical examination revealed an 8 × 6 × 4-cm multilobulated, purplish lesion that was fungating and friable (Figure 1).

He also had a palpable, hard mass in the left axilla.

A punch biopsy of the back mass revealed immunohistochemistry strongly positive for S-100, MART-1, and HMB-45.

Computed tomographic and positron emission tomographic scans demonstrated a fludeoxyglucose-avid lesion on the back extending into the subcutaneous fat and fludeoxyglucose-avid uptake in the left axilla.An 8 × 6 × 4-cm multilobulated, purplish lesion on the back that was fungating and friable.

He had no other lesions elsewhere on his body.


The patient then developed deep ST-segment depressions associated with chest and throat pain.

Transthoracic echocardiography showed an atrial septal defect, and transesophageal echocardiography confirmed a 1.2-cm secundum atrial septal defect with bidirectional shunting without a clear superior rim.

A computed tomographic (CT) pulmonary angiogram showed a dilated pulmonary artery consistent with pulmonary hypertension (Figure 1A).A, Computed tomographic (CT) pulmonary angiogram showing a dilated pulmonary artery size in compare to the aorta size.

B, Coronary angiogram revealing diffuse narrowing of the left main coronary artery at its origin.Right and left heart cardiac catheterization was performed.

During coronary angiography, the patient was noted to have diffuse 90% to 95% narrowing of the left main coronary artery at its origin (Figure 1B; Video).

The left anterior descending and left circumflex arteries were normal in caliber and appearance.The question arose whether the left main coronary artery stenosis represented an anatomic anomaly, a spasm, or a combination.

Intracoronary nitroglycerine was administered with no change in angiographic appearance of the left main coronary artery.

The catheter was re-engaged, and additional intracoronary nitroglycerine was administered with the resolution of ST-segment depression and pain.The patient then developed ventricular tachycardia requiring cardioversion and intravenous amiodarone and lidocaine.

The patient became unstable with recurrent episodes of ventricular tachycardia/fibrillation requiring cardiopulmonary resuscitation.


He had no history of trauma, surgery, or intubation.

He experienced a popping sensation during the sneeze followed by neck stiffness 15 minutes later.

He did not have associated shortness of breath, hemoptysis, or other pertinent medical history.

Anterior neck examination revealed tenderness over the thyroid notch and left side of the laryngotracheal complex.

On physical examination, his vital signs were stable with normal oxygen saturation on room air.

His voice was rough and without stridor.

Flexible laryngoscopy was performed, which showed hematoma of the left true vocal fold and laryngeal surface of the epiglottis with normal vocal fold movement and complete glottic closure (Figure, A).Hematoma of the left true vocal fold and laryngeal surface of the epiglottis.


He reported no perceived association between these episodes and breathing and head position, and denied precipitation of symptoms with exposure to loud sound or pressure change.

He had not experienced hearing loss, aural fullness, autophony, or headache.

On physical examination, objective, intermittent, clicking tinnitus at a frequency of 1 click per second was noted on placement of a stethoscope in close proximity to the right ear.

The tinnitus coincided temporally with the patient’s complaints but was nonsynchronous with pulse.

Results from prior imaging including a computed tomographic (CT) scan of the head with multiplanar reconstruction (Figure, A, C, and D), were normal.

Steady state tympanometry showed mildly decreased compliance of the tympanic membrane when the patient reported hearing the clicking sensation.

The patient was tentatively diagnosed as having tensor tympani and stapedius myoclonus and after discussion of treatment options, he decided to undergo middle ear exploration for muscle sectioning.

The incidental findings observed during middle ear exploration are shown in the Figure, B.A, Computed tomographic (CT) image of the head using bone window.

B, Intraoperative photograph of incidental surgical findings.

C, Multiplanar oblique coronal reconstruction of computed tomographic image.

The inset shows the plane of reconstruction in an oblique coronal view longitudinal with the petrous pyramid.

D, Computed tomographic image of the head using bone window.


She had not experienced dyspnea, hoarseness, dysphagia, hemoptysis, or chest pain.

Flexible fiber-optic nasolaryngoscopy demonstrated a smooth hypervascular mass arising from the posterior membranous trachea (Figure, A).

The patient underwent tracheostomy and complete excision via a transtracheal approach.

The patient was decannulated on postoperative day 5.


His appetite was intact; he had not lost weight; and no dysphagia, stomach pain, or stool irregularity was reported.

Blood tests revealed serious iron deficiency anemia (hemoglobin level, 8.6 g/d [86 g/L]) and a positive result for a fecal occult blood test.

Initially, we performed an upper endoscopy that revealed normal esophageal mucosa with folding of the posterior esophageal wall through the entire length of the esophagus.

Distal ending of the folding was not clearly seen in forward viewing, but in retroflexion, a necrotic polypoid lesion was observed protruding from the esophagogastric junction.

A computed tomographic scan of the chest with oral contrast revealed an esophageal intraluminal tubular mass of soft-tissue density, about 15 mm in diameter originating just below the pyriform recessus and extending through the esophagus in the stomach lumen for about 20 mm (Figure 1).

The patient was referred to an ear, nose, and throat specialist who performed esophagoscopy of the hypopharynx with the patient under endotracheal anesthesia.Intraluminal tubular mass extending through the entire esophagus.


He had experienced blurred vision of the right eye for 2 months; medical history included hypercholesterolemia.

Best-corrected visual acuity was 20/80 OD and 20/20 OS.

The anterior segment was normal and intraocular pressure was 16 mm Hg in both eyes.

Dilated fundus examination of the right eye revealed a normal optic disc, mild venous engorgement, and widespread peripheral dot-blot retinal hemorrhages mainly located in the temporal midperiphery (Figure 1A).

No spontaneous retinal arterial pulsation was noted.

Examination findings of the left eye were unremarkable.

Fluorescein angiography in the right eye showed delayed venous filling, mild perifoveal leakage, and scattered microaneurysms in the temporal sector (Figure 1B).A, Montage color fundus photograph of the right eye at presentation shows mild venous dilation and midperipheral dot and blot hemorrhages mostly located in the temporal sectors.

B, Fluorescein angiography frame (2 minutes, 3 seconds) shows multiple microaneurysms in correspondence of the dot-blot retinal hemorrhages and a barely visible perifoveal vascular leakage.


Her medical history was significant for hypertension, asthma, hepatitis C, and essential tremor.

Her medications include lisinopril, albuterol sulfate, and amantadine hydrochloride.

She denied any previous ocular surgical procedures or trauma.

She endorsed mild photophobia but denied pain, redness, tearing, or discharge.

Her central corneal thickness at that time was recorded at 798 μm OD and 827 μm OS.Her best-corrected visual acuity at presentation was 20/70 OD and 20/50 OS.

Her extraocular motility, pupils, and confrontation visual fields were normal.

Intraocular pressures were 7 and 9 mm Hg OD and OS, respectively.

An anterior segment slitlamp examination was significant for central bilateral stromal edema, Descemet folds, microcystic edema, and endothelial guttae in both eyes (Figure).

There were no keratic precipitates, and the anterior chamber did not show cells or flare.

No abnormalities were noted during an ophthalmoscopic examination.

Her central corneal thickness was found to be 833 μm OD and 925 μm OS.Slitlamp photograph at presentation showing corneal edema in the left eye.

She was seen 2 weeks prior and was treated with 400 mg of oral acyclovir sodium 5 times a day and sodium chloride, 5%, ointment 4 times a day.

There was no history of oral/genital ulcers, rashes, or atopy.


Her medical history included sarcoidosis, type 2 diabetes, hypertension, and coronary artery disease.

Her ocular history included pseudophakia and proliferative diabetic retinopathy in both eyes; she had also undergone pars plana vitrectomy and panretinal photocoagulation in both eyes.

However, her symptoms persisted and eventually worsened.

She reported increasing swelling and pain in the affected region.

She denied changes in vision, eye pain, or pain with eye movements.

The patient’s best-corrected visual acuity was 20/60 OD and 20/50 OS, her pupils were reactive, extraocular motility was full and painless, and intraocular pressures were 18 mm Hg in both eyes.

External examination revealed an erythematous, tender, firm mass in the medial periorbital area (Figure, A).

Results of anterior slitlamp examination and dilated fundus examination were unremarkable.

Contrast-enhanced computed tomographic imaging of the maxillofacial region was performed and showed a peripherally enhancing mass in the affected area (Figure, B).A, External photograph demonstrating a firm, tender, erythematous mass located in the medial periorbital area.

Seven days prior to presentation, an ophthalmologist prescribed the patient oral amoxicillin-clavulanate, 500 mg 3 times daily, and topical antibiotics.


The patient had a concussion 1 year prior after becoming dehydrated during marching band practice, fainting, and hitting her head on pavement.

Her postconcussion symptoms included headaches, dizziness, and dysarthria.

The headaches and dizziness resolved but the dysarthria remained.

Brain magnetic resonance imaging (MRI) within the last year was normal.

Family history was unremarkable for vision loss, glaucoma, inherited retinal degeneration, and autoimmune disease.Best-corrected visual acuity was 20/80 OU.

She complained of progressively declining, blurred vision for 2 years.

She denied floaters, flashes of light, headaches, and eye pain.

Goldmann visual field testing showed mild bilateral nasal constriction.Two months after presentation, the patient returned with new balance difficulties and worsening dysarthria.

Horizontal and vertical saccades were markedly slowed bilaterally.

Ishihara testing revealed red-green color deficiency bilaterally.

Intraocular pressures, extraocular motility, confrontational visual fields, and pupils were normal.

Anterior segment examination was unremarkable.

Ophthalmoscopic examination demonstrated mild mottled retinal pigment epithelial abnormalities bilaterally (Figure, A).Right eye multicolor fundus image and retinal spectral-domain optical coherence tomography (SD-OCT).

A, Multicolor fundus image demonstrates mild macular mottling.

The optic disc and retinal vessels are unremarkable.

B, Retinal SD-OCT shows normal foveal contour with disruption and loss of the interdigitation and ellipsoid zones subfoveally.Fundus autofluorescence showed bilateral mild macular hyperautofluorescence likely within normal range without evidence of flecks.

Retinal spectral-domain optical coherence tomography demonstrated bilateral disruption of the interdigitation and ellipsoid zones subfoveally (Figure, B).

Electroretinogram (ERG) revealed delayed and decreased amplitude responses to 30-Hz flicker with normal dark-adapted responses.


Three months into treatment, she developed multiple thick, mildly pruritic, yellow hyperkeratotic plaques on her lower extremities (Figure, A).

B, Shave biopsy of skin (hematoxylin-eosin; original magnification ×10).

C, Deeper biopsy demonstrating bandlike lymphocytic infiltration (hematoxylin-eosin; original magnification ×20).

Biopsy results demonstrated a squamous cell carcinoma (SCC) of the lung, and chemoradiotherapy with carboplatin and paclitaxel for stage 3A disease was initiated.

An allergic reaction prompted a change to cisplatin-etoposide, which continued as adjuvant chemotherapy after the radiation was complete.

At first restaging, a recurrence of disease in the lung was detected, which was treated with cisplatin and gemcitabine for 4 months before she enrolled in a clinical trial (NCT02009449) in which she received combination therapy with pembrolizumab, 2 mg/kg intravenously for 21 days, and an investigational agent, AM0010 (pegylated recombinant human interleukin-10), 10 μg/kg subcutaneously on days 1 through 14 of the 21-day cycle.

A referral to a dermatology expert was initiated.A, Clinical image showing representative plaque on left leg of patient on initial presentation.


Six weeks later, he presented with abdominal discomfort for 3 weeks relieved by bowel movements associated with increased stool frequency (4-5 Bristol type 4 stools per day).

He was diagnosed with Clostridium difficile infection (CDI) with a positive stool polymerase chain reaction (PCR) for the toxin B (tcdB) gene.

Laboratory studies showed unremarkable results (Table 1).

Vital signs and physical examination were unremarkable and a repeat stool test for C difficile toxin PCR was positive.Diagnose recurrent Clostridium difficile infection (CDI) and prescribe metronidazole.Diagnose postinfectious altered bowel habits with C difficile colonization and recommend a high-fiber diet.Diagnose refractory CDI and consider fecal microbiota transplantation.

He was successfully treated with a 10-day course of oral metronidazole, and his bowel movement pattern returned to its baseline of 2 to 3 Bristol type 4 stools per day.


Her medical and family histories were unremarkable.

The lesions had been present since childhood, but the size of the lesions and the intensity of pain associated with them had recently increased with even light touch causing paroxysms of pain.

The pain associated with these papules worsened as the day progressed, and she could not tolerate elastic compression due to excessive pain on compression.

Physical examination revealed multiple discrete and agminated blue papules on the face, arms, hands, and thighs with a cobblestoned appearance (Figure 1).

On palpation, the papules were exquisitely tender and partially compressible.

The patient underwent duplex ultrasonography of representative papules on the thigh, and a skin biopsy specimen of a papule on the thigh was obtained for histopathologic evaluation.Clinically, multiple discrete and agminated blue papules are apparent, nodular, blue cutaneous lesions cobblestone-like in appearance, on the thigh (A) and hand and thigh (B).


The lesions were present since childhood, progressively increasing in number.

They involved primarily her face, and, less so, her extremities and trunk.

The papules were present in childhood and were increasing in number.

Previous studies and imaging of the 2 siblings had failed to reveal any systemic findings.Physical examination revealed multiple smooth skin-colored and erythematous papules of various size, some with telangiectasia, with a medial but asymmetrical distribution on the face (Figure, A).

Identical, less numerous lesions were also present on her hands and back.

Three biopsy samples were taken and showed similar findings (Figure, B-D).

B, Hematoxylin-eosin stain (original magnification ×20).

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

D, Alcian blue stain (original magnification ×10).

There was no medical history of spontaneous regression.

She had a young brother, who had the same papular lesions that had followed the same natural history, displaying a similar bodily distribution although fewer in number.

A, Multiple skin-colored to erythematous asymptomatic facial papules in a woman in her 20s.


Two ulcers developed at the surgical site, and 2 similar ulcers developed on his back shortly after cyst excision.

A few days after establishing care, he presented to the emergency department with extreme weakness and intolerable pain related to his ulcers.

Physical examination revealed an ill-appearing man.

In the right upper lobe, there were decreased breath sounds with clear auscultation in the remaining chest.

On the right anterior neck were two, 3- to 4-cm tender ulcers extending to the deep subcutaneous fat with erythematous, friable borders (Figure).

On his back were 2 similar 2- to 2.5-cm ulcers.

Inflammatory markers were notably elevated.

A complete autoimmune panel, indirect immunofluorescence assays, enzyme immunoassays for antineutrophil cytoplasmic antibodies (c-ANCA)/proteinase 3, antineutrophil cytoplasmic antibody/myeloperoxidase, a quantiferon gold test, cultures of blood, broncheoalveolar washings, and tissue analysis were performed.

Only c-ANCA testing revealed positive results.

A CT scan of the sinus showed multiple bony defects and acute and chronic sinusitis.

A transbronchial biopsy of the lung and excisional biopsy of the skin bridge between back ulcers were performed.A, Two deep ulcers of the right mid-lower back with erythematous borders.

He had been recently diagnosed with lung cancer based on computed tomographic (CT) and positron emission tomographic imaging showing an enhancing, hypermetabolic, 7-cm cavitary right upper lobe mass with mediastinal adenopathy.

A chest x-ray 6 months prior revealed negative results.

B, Hematoxylin and eosin stain (original magnification × 5).

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

D, Hematoxylin and eosin stain (original magnification × 200).


Until 18 months ago, he had worked as a clerk, but he retired because of a history of hypertension and diabetes.

His family history revealed no neurological disorders, except for a brother who had a brain lesion excised.

Four days before presentation, he experienced sudden, severe neck pain that swiftly progressed to neck stiffness and bilateral shoulder pain.

The next day, he noticed weakness in the right arm and leg.

The day before hospitalization, the weakness progressed, and he lost the ability to ambulate.

On examination, he was afebrile, and his vital signs were normal.

He was fully alert but had weakness in the right distal arm (medical research council [MRC] grade 3/5) and the right leg (MRC grade 4/5) with sensory impairment to pinprick and temperature in the left leg.

Laboratory values revealed an elevated γ-glutamyltransferase level of 615 U/L (reference range, 10-71 U/L).

Analysis of the cerebrospinal fluid indicated xanthochromia; normal white blood cell count, 1 cell/µL; red blood cell count, 2.19 × 10/μL; increased glucose level at 133 mg/dL (reference range, 40-80 mg/dL); increased total protein level at 0.10 g/dL (reference range, 0.03-0.05 mg/dL); and increased lactate level at 28.8 mg/dL (reference range, 9.9-21.6 mg/dL).

Magnetic resonance imaging (MRI) of the brain and the entire spinal cord revealed patency of the intracranial vessels and multiple abnormal signals throughout the brain on T1-, T2-, and susceptibility-weighted sequences (SWIs) and 1 lesion at the level of the fifth cervical vertebra (Figure).A, Susceptibility-weighted magnetic resonance image (MRI) showing numerous intraparenchymal hypointensities (red arrowheads).


The rash did not bleed, ulcerate, weep, or appear itchy or painful, though the patient did seem uncomfortable when getting dressed.

The eruption continued to spread down his left thigh over the next 2 days, at which point the pediatrician advised the family to go to the emergency department.On examination, the boy was afebrile and well-appearing.

There were multiple discrete clusters of monomorphic pink to violaceous papulovesicles with surrounding erythema scattered across the left buttock, thigh, and upper calf, some with central punctate hemorrhagic crust (Figure).

His pediatrician prescribed cephalexin and mupirocin for a presumed cutaneous bacterial infection.

He was fully vaccinated, with routine vaccines at 12 months of age administered in the left thigh.

The influenza vaccine was administered 1 week prior to presentation in the left buttock, after which his parents noted a red patch near the site of vaccination.

The patch became raised and spread down the left thigh over the course of several days.

He had no known sick contacts at day care, exposure to  herpes simplex virus, or exposure to new topical products or clothing.

A test was performed.Discrete clusters of monomorphic pink to violaceous papulovesicles with surrounding erythema.


His social history was notable for a 15-pack-year smoking history and consumption of 6 to 8 beers per night.

He had no family history of malignant disease.

He described early satiety but had no nausea or vomiting.

He reported night sweats and a 9.1-kg weight loss over the prior 6 months.

On examination he had no fever, a reducible umbilical hernia, a palpable left-sided abdominal mass, and significant bilateral lower extremity edema.

Laboratory evaluation revealed anemia for which the patient received several blood transfusions, leukocytosis (white blood cell count, 57 000/μL), and hyponatremia (sodium level, 128 mEq/L).

He was first referred to the hematology service, and a blood smear showed 74% neutrophils.

A bone marrow biopsy specimen showed a hypercellular marrow with a myeloid left shift but no increase in blasts or dyspoiesis.

Tests for BCR/ABL and peripheral blood JAK2 mutation were negative.

Computed tomography revealed splenomegaly, hydronephrosis, and a 25-cm left-sided retroperitoneal mass (Figure).

A, A large retroperitoneal mass extending from the upper abdomen into the pelvis.

B, A mass encasement of the left kidney.

Surgical consultation was requested.Computed tomographic images.


She had no significant medical history and no prior abdominal surgery.

Results of her abdominal examination revealed a slight distension and diffuse tenderness to palpation, particularly in the epigastrium and right upper quadrant, but no peritoneal signs.

In the emergency department, she was afebrile with stable vital signs.

Results of laboratory tests were unremarkable except for mild leukocytosis.

Abdominopelvic computed tomography revealed a distended cecum in the left upper quadrant (Figure 1).Computed tomographic images of a dilated cecum in the left upper quadrant (arrowhead).


A review of systems as well as her medical, family, and social history were unremarkable.

She was not taking any medications and did not have any allergies.

She had no specific chief complaint other than intermittent floaters.

Best-corrected visual acuity was measured 20/25 OD and 20/20 OS.

Extraocular movements were intact and pupils were round and reactive with no afferent pupillary defect.

Intraocular pressure by applanation was 18 mm Hg OU.

Her anterior segment examination was within normal limits.

Examination of her fundus revealed the optic nerves seen in Figure 1.


His medical history was significant for 8 months of bilateral recurrent gingivitis with persistent reactive lymphadenopathy of the head and neck unresponsive to treatment with systemic antibiotics.

He was referred to our clinic for worsening uveitis and ocular hypertension.

Gram stain and culture of aqueous and vitreous biopsy sample results were negative for infectious and cytologic studies, as were serum human leukocyte antigen B27, tuberculosis, syphilis, and sarcoidosis test results (Figure, C).Slitlamp examination findings of the left eye with hypopyon (A) and iris nodules (B).

His visual acuity was 20/40 OD and 20/200 OS, and intraocular pressures were 17 and 32 mm Hg, respectively.

On slitlamp examination, both eyes were markedly hyperemic with diffuse corneal stromal edema, and his anterior chambers were deep with 3+ OD and 4+ OS cell and mixed hypopyon and hyphema in each eye (Figure, A and B).

His dilated fundus examination findings were remarkable only for an area of retinal pigment epithelial stippling inferonasal to the macula in the left eye, without any vitritis in either eye.

He did not have any skin or mucocutaneous lesions, but he had extensive bilateral preauricular, submental, posterior occipital, and supraclavicular lymphadenopathy.

Note the scant cytoplasm containing small, irregular nuclei with inconspicuous nucleoli.


In 1998, he underwent mitral valve replacement with a St Jude mechanical prosthesis for severe mitral regurgitation owing to a flail leaflet, with 2-vessel coronary artery bypass grafting.

He reported compliance with warfarin therapy for many years.One year ago, he noted the onset of exertional dyspnea that culminated in multiple recurrent admissions for decompensated heart failure requiring intravenous diuretics.

While hospitalized, he underwent a cardiac catheterization that demonstrated patent bypass grafts but a profoundly low cardiac output and elevated pulmonary capillary wedge pressure.

A transthoracic echocardiogram was performed that suggested the presence of a significantly elevated gradient across his mitral prosthesis.

A plain radiography of the chest demonstrated a mechanical prosthesis (Figure 1A).

A transesophageal echocardiogram was then performed that demonstrated complete immobilization of 1 mechanical leaflet and impaired excursion of the other leaflet (Figure 1B), with subsequent severe mitral stenosis (Figure 1C) at a baseline heart rate of 82 bpm.

B, Transesophageal echocardiogram midesophageal view (90°) demonstrating bileaflet mechanical mitral valve prosthesis with immobilized leaflet (left) in systole and diastole.

He developed worsening hemodynamic instability and refractory pulmonary edema, and a TandemHeart left ventricular assist device was placed for cardiogenic shock.A, Chest radiograph with zoom of the mitral valve prosthesis, demonstrating opening of 1 leaflet from en face view and other leaflet not seen.


Her history of end-stage renal disease precluded postoperative radioactive iodine treatment.

Her compressive symptoms had also resolved.

Ultrasonography performed at her 3-month follow-up showed no evidence of disease recurrence or local metastasis.

Ultrasonography revealed a 1.3 × 1.1 × 1.0-cm hypoechoic mass in the right central compartment and a suspicious lymph node in the right carotid sheath.

Fine-needle aspiration confirmed recurrence of a malignant neoplasm in the central compartment; however, the lymph node was not amenable to sampling based on its anatomic location.

C and D, Histopathologic images of resected tissue specimen, hematoxylin-eosin.

The procedure was uneventful and well tolerated.

The patient decided to undergo a right central (level VI) lymph node dissection.

In the right central compartment several prelaryngeal and pretracheal nodes were identified and excised.

Intraoperatively, insular carcinoma was dissected away from the cricothyroid membrane and recurrent laryngeal nerve.

Histopathologic analysis revealed a cystic mass composed primarily of mucosal cells and sparse fibroconnective tissue (Figure, C and D).A, A large heterogeneously enhancing mass with central necrosis arising from the right thyroid fossa with leftward displacement of the trachea (t).

After removal of the most distal of these nodes, a separate pale tan mass 2 cm in diameter was visualized at the level of the thoracic inlet.

The mass demonstrated respiratory expansion and retraction.

Large air-filled cavity (yellow arrowhead) measuring 20 × 18 × 15 mm and communicating with the right posterolateral aspect of the trachea.

Incidental small esophageal diverticulae noted (e).

B, Large air-filled cavity (red arrowhead) measuring 20 × 18 × 15 mm and communicating with the right posterolateral aspect of the trachea (t).

Respiratory mucosa with absence of thyroid, lung, smooth muscle, and cartilaginous tissues.


Her birth had been uncomplicated after a term pregnancy.

Her parents also reported loud snoring and some retractions during sleep.

Physical examination revealed mild inspiratory stridor and several light-brown macules approximately 1 cm in size on the patient’s skin.

On flexible fiberoptic laryngoscopy (Figure), a supraglottic mass could be seen, but visualization of the full extent of the larynx or the mass was difficult owing to lack of cooperation by this very young patient.

A submucosal lesion of the left aryepiglottic fold extending inferiorly to the level of the arytenoid, and displacing the epiglottis medially, was found.

C, T1-weighted sagittal magnetic resonance image (MRI) showing laryngeal lesion.

D, Bundles of spindle cells with angulated nuclei in a stroma of variable coarse collagen and loose myxoid matrix (hematoxylin-eosin, original magnification ×4).

The decision was made to obtain a better view under direct laryngoscopy in the operating room.

An incision was made through the mucosa, where a firm, whitish mass that bled minimally was noted, and a biopsy specimen was sent for permanent pathologic analysis.

There was no clinically significant medical or family history.

A magnetic resonance image was then obtained to assess the full extent of the mass and whether surrounding tissues were involved.A, View of lesion at the level of the epiglottis on direct laryngoscopy.

B, View of the vocal folds on direct laryngoscopy.


Four months after SMG excision, she developed a right floor of mouth lesion after “burning her mouth” with high-temperature food.

Computed tomography (CT) and ultrasonography showed normal-appearing submandibular glands and neck soft tissues.

A sialogram showed high-grade stricture approximately 3 to 4 cm into the right submandibular duct orifice.

Complete stricture prevented evaluation of the proximal duct and intraglandular portions.

Sialendoscopy showed a widely patent right submandibular duct proximally with a segmental takeoff, consistent with the Bartholin duct draining into the Wharton duct distal to a blind pouch identified 2.5 cm from the intraoral duct orifice (Figure, A).

Magnetic resonance imaging demonstrated a cystic lesion in the right sublingual space following the Wharton duct (Figure, B).

Recurrent floor of mouth swelling (Figure, C) was addressed by repeated marsupialization and eventual sublingual gland excision.

Pathologic analysis identified fragments of squamous mucosa with inflammation, cystic space, and compressed granulation tissues consistent with mucous extravasation (Figure, D).

Despite a Wharton ductoplasty to improve sublingual gland drainage through the Bartholin duct, the degree of scarring at the hilum and inability to further dilate warranted submandibular gland (SMG) excision.


Her medical history included ulcerative colitis (UC) controlled by oral azathioprine for 2 years.

Initially, she had been treated with oral amoxicillin–clavulanic acid with no improvement.

Initial clinical evaluation revealed respiratory distress with respiratory rate of 21 breaths per minute, with oxygen saturation 95% on room air.

Her heart rate was 95 beats per minute, her blood pressure was 135/91 mm Hg, and she was pyretic.

Further examination with flexible fiberoptic laryngoscopy (Figure, A) showed considerable erythema and edema of the epiglottitis with patchy necrosis partially obstructing the glottis and saliva pooling in the supraglottis.

Findings from the remainder of the otolaryngology examination and chest auscultation were normal.

Subsequently, oxygen saturation improved to 100% with 3 L per minute of oxygen via nasal prongs.

Her initial laboratory findings revealed a white blood cell count of 12 000/μL, absolute neutrophil count of 46%, hemoglobin count of 12.8 g/dL, and a platelet count of 175 000/μL.

Her C-reactive protein level was 5 mg/dL (normal level, <0.5 mg/dL).

Results from liver function tests and chest radiograph were normal.