There was no associated pain, facial numbness, or weakness.

On examination, the mass was freely mobile, the facial nerve was intact bilaterally, and there was no palpable lymphadenopathy.

Results from fine-needle aspiration 6 months prior to presentation were consistent with a cystic oncocytic neoplasm, and computed tomography revealed a 1.9 × 1.5 × 2.1-cm, well-circumscribed ovoid lesion in the anterior aspect of the superficial lobe of the left parotid gland (Figure, A).

Postoperative pathological analysis revealed a cystic, cribriform, papillary neoplastic growth with low-grade cytologic features and nearly complete myoepithelial rims (Figure, B and C).

Calponin and p63 staining were positive in the myoepithelium surrounding the tumor nests (Figure, D), S-100 was positive within the tumor, and the Ki-67 rate was approximately 5%.

Extracellular mucin and occasional mucous cells were also noted.

Margins were clear, and there was no evidence of perineural spread or lymphovascular invasion.

Multiple aspirations of the mass had been performed, which would temporarily decrease the size.

A left superficial parotidectomy was performed under general anesthesia with facial nerve dissection and facial nerve monitoring.

The patient tolerated the procedure well, recovered appropriately, and was free of complications at his first follow-up appointment.


She also reported no recent sick contacts, travel, illicit drug use, or use of oral contraceptive pills or other medications.

She reported a 2-day history of skin lesions on her ears without trauma.

Vital signs included a blood pressure of 140/70 mm Hg, heart rate of 168/min, respiratory rate of 30/min, temperature of 40.9°C, and oxygen saturation of 90% on room air.

Physical examination revealed decreased right basilar breath sounds with dullness to percussion, tachycardia without murmurs or rubs, lower extremity edema, and bilateral cutaneous necrosis of her pinnae (Figure 1).

Laboratory findings showed a white blood cell count of 7700 cells/µL (neutrophils, 82.4%; lymphocytes, 10.6%; monocytes, 6%; eosinophils, 0.2%; basophils, 0.8%), hemoglobin level of 9.0 g/dL (mean corpuscular volume, 84 fL), platelet count of 151 ×103/µL, partial thromboplastin time of 61 seconds (reference range, 22-32), and an international normalized ratio (INR) of 1.4.

An ultrasound showed a right above-the-knee deep venous thrombosis (DVT), and a chest computed tomography angiogram showed a large right pleural effusion without evidence of pulmonary embolism.

Pleural fluid was exudative; Gram stain and culture were negative.


The patient reported no alcohol or drug use and no personal or family history of liver disease.

Medications included diltiazem and furosemide.

She was anicteric with mild bilateral temporal wasting and jugular venous distension.

On physical examination, her blood pressure was 108/54 mm Hg and pulse was 64/min.

Further pertinent findings included regular heart rate and rhythm with an accentuated P2, grade 1/6 holosystolic murmur in the left lower sternal border, full bulging abdomen with flank dullness, and scattered spider angiomata on her chest.

Doppler ultrasound revealed an irregular liver echo pattern without lesions, normal common bile duct, patent portal and hepatic vasculature, splenomegaly, and ascites.

Laboratory results including diagnostic paracentesis are shown in Table 1.Her ascites is primarily due to chronically elevated pressures on the right side of the heart.Her ascites is primarily due to abdominal malignancy.Her ascites is primarily due to spontaneous bacterial peritonitis.


There were no other associated symptoms, including dyspnea, cough, urinary tract symptoms, or gastrointestinal symptoms.His medical history was significant for hypertension, poorly controlled type 2 diabetes, hypercholesterolemia, and biopsy-proven autoimmune hepatitis.

His other regular medications include aspirin, metformin, verapamil, and pravastatin.Examination was remarkable for a temperature of 38°C, an exquisitely tender erythematous papulonodular rash on the extensor surface of his lower and upper limbs, and bilaterally tender knees with a patellar tap (Figure 1).

Laboratory findings included a white blood cell count of 10 500/μL (with neutrophil level elevated at 9900/μL [94%]) and elevated inflammatory markers (C-reactive protein level, 76 mg/L [724 nmol/L]; erythrocyte sedimentation rate, 69 mm/h).

Rheumatoid factor was slightly elevated at 16 IU/mL, but testing for anti–citrullinated protein antibody was negative.

Tests for double-stranded DNA, extractable nuclear antibodies, and anti-antistreptolysin O were negative.

Renal function was normal, and there were no leukocytes, nitrates, red cells, or casts noted in the urine.

Synovial fluid analysis showed a noninflammatory picture with a leukocyte count of 700 cells/L; no bacteria or crystals were seen.Tender erythematous papulonodular lesions on extensor surfaces of lower (left) and upper (right) extremities.

He was started on azathioprine for autoimmune hepatitis at a dose of 75 mg 10 days prior to his presentation after thiopurine methyltransferase activity was found to be normal.


Endometrial carcinoma was found on dilation and curettage.

Family history was significant for prostate cancer in her father and paternal grandfather.Her tumor was screened for Lynch syndrome by immunohistochemistry staining for DNA mismatch repair proteins (mutL homolog 1 [MLH1]; MutS homologs 1 and 6 [MSH1, MSH6]; PMS 1 homolog 2 [PMS2]) and microsatellite instability testing (MSI)1 (Table 1).

Hysterectomy and surgical staging confirmed stage IA endometrial cancer.


The eruption presented abruptly over a 2-week course, and the patient attributed it to contact with plants while working in his yard.

He denied having similar lesions in the past.

He denied recent sexual intercourse, penile discharge, fevers, chills, nausea, or vomiting.

He had used over-the-counter topical antibiotic and antihistamine creams, which alleviated the pruritus.

Physical examination of the bilateral cheeks, glabella, dorsal nose, and chin revealed multiple skin-colored papules, nodules, and vegetative plaques ranging in size from 4 to 6 mm in diameter, with central umbilication, erosions, and overlying yellow crust (Figure, A).

There were similar lesions on the scrotum and inner thighs, with sparing of the penis (Figure, B).

There was also a 1-cm ulceration on the left lower lip.

One 4-mm punch biopsy specimen was obtained from a nodule on the left medial cheek, bisectioned, and sent for both hematoxylin–eosin staining and tissue cultures for bacteria, fungi, and atypical mycobacteria (Figure, C and D).A, Multiple crusted and ulcerative papules and nodules on the face.

B, Multiple ulcerated healing papules on the scrotum and umbilicated papules on the left inner thigh.

C, Low-power image (hematoxylin-eosin, original magnification ×40).

D, A Warthin-Starry silver impregnation stain (original magnification ×40).


She was taking no medications and had not had active gastrointestinal symptoms of CD in several years.

The growths had been present for 1 year.

Physical examination revealed a tender perianal plaque with a papular, cobblestone-like surface (Figure, A).

A biopsy specimen from the perianal plaque was obtained (Figure, B and C).A, Multiple flesh-colored perianal papules and a pebbly plaque in a middle-aged woman with Crohn disease.

B, A biopsy specimen of the perianal plaque was obtained, and histopathology revealed a dermal perivascular and nodular infiltrate composed of aggregates of histiocytes and (C) numerous multinucleate giant cells surrounded by mature lymphocytes.

Acid-fast and silver stains were negative (not shown).


The patient lived in a farm community that used well water for drinking.

He denied wading in local water sources and had not traveled abroad recently.

The patient denied systemic symptoms, and further physical examination results were unremarkable.

The lesion appeared 2 months prior and had been mildly itchy.

Recent laboratory blood work was notable for peripheral eosinophilia.

A punch biopsy was performed (Figure, B).A, Photograph shows a serpiginous erythematous plaque on the abdomen.

B, Histopathological analysis of a punch biopsy specimen demonstrates eosinophilic cuticle (external tegument) and calcareous bodies (basophilic internal structures).

C, Histopathological analysis of a punch biopsy specimen.


She had no alcohol abuse or smoking history.

Dyslipidemia and a transient ischemic attack 3 years before were her only medical history.

She had never had a colonoscopy or a gastroscopy.

Her medication included atorvastatin and acetylsalicylic acid.

The only past operation she reported was for treating carpal tunnel syndrome.

She was a sheep breeder and was in close contact with animals for 8 to 12 hours every day.During physical examination, the heart and the lungs were normal.

The pain was localized to the epigastrium and had acute onset  that prevented her from working for the rest of the day.

There was no association between the pain and eating.

Her abdomen was soft with tenderness in the epigastrium.

The laboratory evaluation results revealed normocytic anemia (hematocrit level of 28.1% [to convert to proportion of 1.0, multiply by 0.01] and hemoglobin level of  9.3 g/dL [to convert to grams per liter, multiply by 10]) including a normal white blood cell count of 7600/µL (to convert to ×109 per liter, multiply by 0.001).

Results from a complete metabolic panel were normal.

An abdominal radiography was performed (Figure 1).Anteroposterior abdominal x-ray film in erect position.

In the right side of the mid to lower quadrant, at the level of L5 vertebral body, there is a rounded radio-opaque lesion (arrowhead) that seems to contain a second smaller sclerotic lesion within its central part (a lesion-in-lesion appearance).


He had no history of abdominal trauma, infection, or surgery.

A physical examination revealed mild tenderness over the posterior left upper quadrant.

A precontrast computed tomographic scan revealed a large, well-defined, cystic mass (7 by 6 cm) with mural calcification in the spleen (Figure 1A), and the cyst was not enhanced in the arterial phase.

The laboratory test results, including carcinoembryonic antigen and carbohydrate antigen 19-9 levels, were all within normal ranges, except for a mild increase in the carbohydrate antigen 72-4 level, which was 11.89 U/mL (normal range, 0-6.9 U/mL).A, Computed tomography (CT) revealed a large, well-defined, cystic mass with mural calcification in the spleen.

B, The surface of the cyst was extremely dense and appeared to have an eggshell-like calcification; the cyst was filled with about 300 mL of yellowish opalescent fluid containing numerous cholesterol-like crystals (arrowhead).During a laparotomy, a huge hard mass, which was partially covered by fibrous and necrotic tissue, was found emerging from the lower pole of the spleen.

A total splenectomy was performed, and a gross pathologic examination revealed that the lower end of the spleen was occupied by a cyst, the surface of which was extremely dense and appeared to have an eggshell-like calcification (Figure 1B).

The cyst was filled with about 300 mL of yellowish opalescent fluid containing numerous cholesterol-like crystals (Figure 1B).


Two weeks prior to her visual symptoms, she had noticed intermittent painless, postmenopausal vaginal bleeding.Her medical and ocular history were unremarkable.

Ophthalmologic examination revealed a visual acuity of no light perception OU.

Her examination reults 2 years prior were normal, with a visual acuity of 20/20 OU.In the emergency department, the patient had a computed tomographic scan of the brain and orbits that was normal, and she was referred to Ophthalmology.

Pupils were unreactive to light but constricted to a near target.

Slitlamp examination of the anterior chamber revealed mild nuclear sclerosis in each eye and quiet anterior chambers.

A dilated fundus examination is shown  in the Figure.


The right eye was temporally hyperemic and mildly tender to touch.

Her visual acuity was 20/200 OD and 20/20 OS.

Fundus examination revealed an elevated whitish lesion in the superotemporal quadrant of the right eye, associated with subretinal fluid at the fovea (Figure 1).

A- and B-scan ultrasonography demonstrated a choroidal mass with moderate internal reflectivity.An elevated whitish lesion is noted in the superotemporal quadrant of the right eye, associated with subretinal fluid at the fovea.


Her history consisted of non–small cell lung cancer treated with wedge resection 2 years prior, and she had been receiving chemotherapy until 1 year previously.

She was declared free of cancer 6 months earlier based on systemic surveillance.

Visual acuity was 20/80 OD and 20/20 OS.

Biomicroscopy showed trace anterior chamber cells in the right eye with mild vitritis.

Fundus examination demonstrated parafoveal whitening with hemorrhage and focal aggregations (Figure, A).

The lesion stained on fluorescein angiography (Figure, B).

There was no evidence of disc edema or vasculitis.

Optical coherence tomography showed disorganization of the retina.A, The fundus montage shows a white retinal hemorrhagic lesion on initial presentation, which subsequently gradually enlarged.

B, Wide-field fluorescein angiography demonstrates late staining of the lesion.

Aqueous polymerase chain reaction testing was negative for cytomegalovirus, herpes simplex virus, varicella zoster virus, and toxoplasmosis.

Systemic testing was negative for cytomegalovirus, herpes simplex virus, varicella zoster virus, Lyme disease, syphilis, Toxocara, toxoplasmosis, tuberculosis, and Bartonella.

The patient was treated with a vitreous tap and intravitreous injection of foscarnet sodium and ganciclovir sodium, and she began valgancyclovir hydrochloride therapy.

Sulfamethoxazole–trimethoprim sulfate was started to cover possible toxoplasmosis.

Doxycycline hyclate was initiated, but subsequent titers were negative.

C, The lesion is regressing after external beam radiation therapy with a radiosensitizing adjuvant.There was no vasculitis, but an atypical presentation of viral retinitis was suspected given her history of immunosuppression, vitritis, and retinal whitening.

The diagnosis was indeterminate, but treatment was recommended due to macular involvement.

Rickettsia retinitis was also suspected clinically.


She denied pain but described a pressure sensation behind her left eye.

There was moderate restriction to elevation of the left eye.

Uncorrected visual acuity measured 20/80 OD and 20/60 OS, improving to 20/40 OD and 20/30 OS with pinhole testing.

Pupils were round and reactive without an afferent pupillary defect.

Intraocular pressure was 18 mm Hg OU by applanation tonometry.

Hertel exophthalmometer measurements demonstrated 7 mm of proptosis OS.

Anterior segment and ophthalmoscopic examination results were unremarkable.Computed tomography of the orbits with and without contrast was performed (Figure).

A homogeneously enhancing, well-demarcated, ovoid mass was visualized within the medial intraconal space, measuring 2.0 × 2.2 × 2.6 cm.

The lesion displaced the globe inferiorly and anteriorly.

Smooth bony remodeling was present without erosive changes.

Magnetic resonance imaging of the orbits corroborated these findings.

Conventional angiography demonstrated a tortuous branch of the left ophthalmic artery supplying the medial aspect of the orbital mass.Computed tomography and conventional angiography of the orbital mass.

A, Computed tomography of the orbits demonstrates a homogeneously enhancing mass within the intraconal space (blue arrowhead).

B, Cerebral angiography demonstrated a branch of the left ophthalmic artery supplying the orbital mass (black arrowhead).


This condition had been present for 4 weeks and included areas of the scalp, face, chest, abdomen, back, axillae, arms, legs, perineum, and buttocks.

There were no aggravating factors, and no family member in contact with the patient had a rash with similar characteristics.

The physical examination findings were notable for a well-appearing infant with pink to brown nummular patches and plaques with thick white scale on the scalp, cheeks, trunk, back, axillae, inguinal crease, scrotum, and umbilicus and posterior to the ears (Figure 1).

The patient had no lymphadenopathy or hepatosplenomegaly.

B, Pink and brown scaly patches on the abdomen within the inguinal crease.

The parents tried moisturizers and topical antiyeast medications with no change in the rash.

He was up to date on all immunizations and was full term.A, Infant at presentation, with pink to brown round scaly patches and plaques widespread on the trunk including umbilicus and axillae.


She had undergone aortic valve replacement for aortic stenosis, 2-vessel coronary artery bypass, and permanent pacemaker placement 1 year before presentation.

She had a history of mantle chest radiotherapy for Hodgkin lymphoma at age 17 years, which was curative.

After successful intravenous diuresis, neck examination at approximately 60° (Figure 1A and Video) revealed a regular carotid pulsation and discernible internal jugular venous pulse.

Hemodynamic data at left and right heart catheterization are shown in Figure 1B.A, Neck examination at 60° shows the location of the carotid and internal jugular pulsations.

B, High-fidelity micromanometer simultaneous recording of the left ventricular (LV) and right atrial (RA) pressures.

After examination of the neck at rest, the patient took a small breath and held it for a few seconds.


He had no history of dysphagia and/or odynophagia, hoarseness, vision changes, dry mouth, decreased ipsilateral sweating, weight loss, fevers, chills, or a history of tobacco or alcohol use.

He did not have facial pain, weakness, numbness, trismus, or difficulty chewing.

Physical examination revealed a palpable nontender mass in the left parapharyngeal space.

Cervical lymphadenopathy was not palpable.

Nasopharyngoscopy revealed slight bulging of the lateral pharyngeal wall into the oropharynx with no discrete masses seen.

Computed tomography–guided fine-needle aspiration biopsies were nondiagnostic.

Axial T2-weighted MRI showed a lesion arising from the left parapharyngeal space, arising from the deep lobe of the parotid gland.

The lesion caused loss of the fat plane between the parapharyngeal space and masticator space with subsequent inflammation of the pterygoid space.

The pharyngeal mucosa of the left lateral pharyngeal wall was effaced owing to mass effect (Figure, A).

Hematoxylin-eosin staining showed sections of confluent amorphous eosinophilic masses with surrounding foreign-body giant cell reaction and lymphoplasmacytic infiltrates (Figure, B).

Sheets of plasma cells were positive for κ and CD138 and negative for λ, consistent with clonal κ light-chain restricted plasma cell infiltrates around amorphous tissue (Figure, C).

Congo red stain showed apple-green birefringence demonstrated on polarized microscopy (Figure, D).A, Magnetic resonance imaging (MRI) of a lesion in the left parapharyngeal space.

The mass was surgically resected in its entirety.


Twenty-nine months prior to presentation, the patient had been diagnosed as having myelodysplastic syndrome, which 7 months later progressed to AML.

His physical examination was notable for a “breathy” voice with no respiratory distress or cervical lymphadenopathy.

Flexible nasal endoscopy revealed an immobile right vocal fold with broad submucosal fullness on the right false vocal cord and arytenoid.

Computed tomography (CT) demonstrated a right-sided laryngeal mass extending from the level of the free margin of the epiglottis to the level of the glottis (Figure, A).

In the operating room, during direct laryngoscopy a polypoid mass was seen based on the right arytenoid, also involving the right false vocal cord (Figure, B).

Frozen section confirmed polypoid ulcerated tissue with granulation consistent with an inflammatory process, and histopathologic analysis demonstrated a mixed infiltrate containing numerous mononuclear myeloid cells positive for myeloperoxidase, CD34, CD68, and CD33 but negative for CD117 (Figure, C and D).

There are small numbers of scattered CD3+ T-cells and CD20+ B-cells.A, Right-sided laryngeal mass.

B, Polypoid mass on the right arytenoid.

C, Neoplastic cells admixed with lymphoplasmacytic inflammatory cells.

During treatment for AML with low-dose cytarabine, his course was complicated by neutropenic sepsis requiring intubation, resulting in his head and neck symptoms.


She denied pain and was an otherwise healthy nonsmoker.

The size now caused intermittent gagging and a foreign-body sensation in her throat.

Examination of the oral cavity revealed an approximately 1-cm, pedunculated mass of the posterior dorsal tongue just lateral to the circumvallate papillae.

The lesion was mobile and well-circumscribed without ulceration or surrounding mucosal abnormalities.

It was nontender to palpation without drainage or discharge.

Flexible fiber-optic laryngoscopy showed no other lesions.

Neck ultrasonography showed a normal thyroid gland.

Histopathologic examination revealed a discrete proliferation of mature bone with overlying mildly hyperplastic stratified squamous epithelium without surrounding inflammation (Figure, A).

The core was composed of dense lamellar osseous matrix with numerous Haversian canals and associated regularly dispersed osteocytes in lacunae (Figure, B).

Excisional biopsy was performed with the patient under local anesthesia, and the entire lesion was sent for pathologic examination.

Grossly, the firm, polypoid mass measured 0.5 × 0.5 × 0.8 cm.


Physical examination demonstrated no lymphadenopathy.

The oropharynx and oral cavity were clear.

Anterior rhinoscopy demonstrated thick, clear to yellow mucus bilaterally in the nasal cavities and bilateral inferior turbinate hypertrophy.

There was right to left nasal septal deviation.

Rigid nasal endoscopy demonstrated moderate mucosal edema responsive to topicalization.

Thick, clear to yellow mucus was evacuated with suction from the middle meatus and sphenoethmoidal recess, revealing a large, lobulated, flesh-colored mass filling the entire nasopharynx with an irregular surface and firm consistency.

The fossae of Rosenmuller were effaced bilaterally.

There was no overt surface ulceration or bleeding.

Computed tomography (CT) revealed a well-defined, round, 2-cm midline posterior nasopharyngeal mass, situated between the longus colli muscles (Figure, A).

The lesion appeared hypodense on CT, without calcification, enhancement, or bony erosion.

Magnetic resonance imaging (MRI) (Figure, B-D) demonstrated the lesion to be hypointense on T1 sequence and hyperintense on T2 sequence with mild heterogeneous enhancement.

Endoscopic biopsy of this mass was performed.


The patient had a history of hypertension and laminectomy, and he never smoked.

Family history was notable for prostate cancer in his father, but the patient’s prostate-specific antigen and digital rectal examination results were normal.Perform a computed tomographic scan to evaluate for source of hematuria.Perform microscopic urinalysis to confirm the presence of blood in the urine.Refer to urology for cystoscopy to evaluate the source of hematuria.


Her mother described an intermittent inflammatory reaction localized only to the patient’s abnormal skin lesions that was reproducible in febrile states and was concerned about asymptomatic, stable, dark brown lesions that had developed in areas of prior blistering and scarring on the patient’s right arm, left knee, and right upper back.

There was no history of developmental delay, eye abnormalities, or seizures.Examination revealed irregular, dark brown, well-demarcated macules with regular pigment networks on dermoscopy within prominent soft, yellow, papular protrusions in a Blaschkolinear arrangement on her upper arms, particularly her right arm.

The right flank demonstrated linear, atrophic pink plaques with focal erosions and crusting, evidencing recent inflammation described by the patient’s mother (Figure, A).

Additional irregular, well-demarcated, evenly pigmented dark brown macules were present within pink, atrophic scars located on the right side of the trunk, the right upper back, and the left knee.

She had well-healed surgical scars on the hands, syndactyly of the toes, and ventral pterygium and dystrophy of the fingernails and toenails (Figure, B).A, Irregular, dark brown, well-demarcated macules within prominent soft, yellow, papular protrusions in a Blaschkolinear arrangement on the upper arms; linear, atrophic pink plaques with focal erosions and crusting are seen on the right flank.

B, Extracutaneous features show syndactyly of the toes and ventral pterygium and dystrophy of the toe nails.

C, Hematoxylin-eosin staining of skin biopsy demonstrates close proximity of subcutaneous fat to the epidermis with attenuated collagen (original magnification × 20).Previous treatment had included surgical excision of a frequently symptomatic focus of affected skin with subsequent adjacent tissue transfer.

Additionally, she had received dental implantation of frontal upper incisors.

Histopathologic slides from the prior excision of affected skin were reviewed (Figure, C).


According to her mother, lesions had started 2 days prior.

The lesions appeared on the 13th day of life.

Physical examination revealed vesicles filled with clear fluid disposed in a linear arrangement forming whorls and swirls (Figure, A).A, Linear vesicles on the calf of the newborn patient.

C and D, A biopsy specimen of a vesicle was obtained.Herpes simplex and varicella zoster virus polymerase chain reactions were negative.

Syphilis, cytomegalovirus, and toxoplasma serologies were negative.

Bacterial and fungal cultures of the vesicles were negative.

B, After 15 months, the lesions had evolved into small hyperpigmented macules in a linear arrangement.

A biopsy sample of a vesicle was obtained.Over time, lesions healed leaving residual grayish hyperpigmentation (Figure, B).


The patient’s medical history included hypertension, and review of his family medical history was noncontributory.

He endorsed polyuria and polydipsia but was otherwise asymptomatic.

Recent extensive laboratory data revealed mildly elevated triglycerides (209 mg/dL [to convert to mmol/L, multiply by 0.0113]) but otherwise were all within normal limits.

On physical examination, numerous coalescing reddish-yellow pedunculated papules were present in both axillae (Figure, A and B).

No other cutaneous lesions were present.

Shave biopsy of one of the left axillary papules was performed (Figure, C and D) with additional immunohistochemistry positive for CD68 antibody staining.A and B, Numerous coalescing reddish-yellow pedunculated papules in the right and left axillae.

C, Histopathological analysis of a biopsy specimen taken from the left axilla shows histiocytes with foam cells, scattered lymphocytes, and few plasma cells; D, a classic wreath like Touton cell and foam cells with histiocytes.


The patient reported being an infrequent smoker, with about 2 cigarettes a month.The patient during the first pain episode, with a red left eye, lacrimation, and periorbital swelling.She had no history of impaired vision, diplopia, facial trauma, prior head or neck surgery, or allergies.

She reported a strong unilateral headache located in her left cheek, eye, and forehead that evolved during the evening hours, peaked at night, and decreased again until the morning, without any remarkable trigger.

This first episode went along with ipsilateral lacrimation, a red eye, and periorbital swelling but without ptosis, which she documented with her smartphone (Figure 1).

During a painful night, she could not sleep.

Pain remitted, but the swelling increased until the next morning and slowly decreased over approximately 36 hours.

Recurrent but less intense episodes of several hours’ duration occurred during the 2 subsequent nights with no symptoms during the following 14 days.

The patient reported no nasal congestion, nausea, vomiting, or phonophobia or photophobia.

Examination of her head and neck showed no particularities, periorbital edema or proptosis, or enlarged lymph nodes.

The neurological examination was unremarkable, with normal visual acuity, symmetrically reactive pupils, and unimpaired ocular movements, as was a differential hemogram, which showed normal white blood cell count of 5500/μL (to convert to ×109/L, multiply by 0.001).


The patient’s history revealed chronic constipation, relieved by the use of laxatives.

The pain radiated throughout the right leg and was related to nausea without vomiting.

Furthermore, results of an abdominal physical examination were also normal, and no neurological or vascular deficits were found.The abdominal computed tomographic scans performed in the emergency department are shown in the Figure.

The patient never noticed any inguinal protrusion or mass.


Physical examination showed no notable abnormalities.

Blood test results revealed anemia (hemoglobin 9.8 g/dL [to convert to grams per liter, multiply by 10]) but were otherwise unremarkable.

Upper gastrointestinal series showed subtotal occlusion in the second part of the duodenum (Figure 1 A).

Computed tomography of the abdomen confirmed intraluminal narrowing of the duodenum without signs of a mass (Figure 1 B, arrowhead).

Gastroduodenoscopy revealed a duodenal ulcer with pinpoint stenosis that could not be passed or dilated.

Multiple biopsies were negative for cancer.

Total parenteral nutrition was started and the patient was scheduled for surgery.A, Coronal upper gastrointestinal series demonstrate subtotal occlusion in the second part of the duodenum.

B, Axial computed tomography image shows intraluminal narrowing of the duodenum without signs of a mass (arrowhead).


He had no relevant medical history and his family history was negative for malignancy.Clinical examination revealed an area of ulceration centrally along the left upper eyelid margin, measuring approximately 8 mm × 2 mm (Figure 1A).

This was accompanied by foreign-body sensation and discomfort of the left eye and was preceded by a week of general malaise without fevers or chills.

He denied vision changes, light sensitivity, or drainage from the eye.

He denied previous similar episodes of eyelid swelling or recurrent chalazia.

There was mild madarosis in the area of ulceration.

There was also a tender and enlarged preauricular lymph node on the ipsilateral side.

The eyelid demonstrated mild boggy edema; however, there were no associated nodules, vesicles, or plaques on the surrounding skin or face.

The remainder of the clinical examination was unremarkable, including visual acuity, intraocular pressure, slitlamp biomicroscopy, and dilated fundus examination.

A photomicrograph of a hematoxylin-eosin–stained section from an incisional biopsy specimen is shown in Figure 1B.A, External photograph of the left eye demonstrating central upper eyelid margin ulceration with sparse lashes and surrounding edema.


Medical history was significant for a heart murmur with no known history of chickenpox.

He presented with a 2-day history of painful, left-sided vesicular rash around his eyelid and forehead.

Best-corrected visual acuity was 20/20 OD and 20/25-2 OS.

On examination, clear vesicles on erythematous bases were noted in the V1 distribution with left upper eyelid edema and erythema.

Pupils were equally reactive with no relative afferent pupillary defect.

Anterior examination was significant for left-sided conjunctivitis, dendritic keratitis (Figure), and anterior uveitis.

The findings of the remainder of the examination, including the anterior segment of the right eye and the dilated fundus examination of both eyes, were unremarkable.Slitlamp photograph showing branching corneal pseudodendrites with minimal fluorescein uptake.

His pediatrician prescribed oral acyclovir and ibuprofen as needed for pain.


He had a history of human immunodeficiency virus (HIV) infection with a CD4 count of 25 and retinal detachment of the left eye secondary to cytomegalovirus (CMV) retinitis.

He was taking trimethoprim-sulfamethoxazole for prophylaxis of Pneumocystis jiroveci pneumonia (formerly Pneumocystis carinii).

On examination, visual acuity was 20/30 OD and no light perception OS, which was phthisical with no view of the fundus.

In the right eye, the anterior segment was quiet, and there was 1+ nuclear sclerosis.

Dilated fundus examination showed no vitritis, multiple discrete hyperpigmented scars (Figure, A, black arrowhead), and an area of inferotemporal retinal whitening (Figure, A, white arrowhead) with associated hemorrhage.

On his return, his visual acuity had declined to 20/50 OD, there was mild vitritis, and there were new areas of retinitis along the inferior arcade (Figure, B, white arrowheads) and vascular sheathing (Figure, B, black arrowhead).Fundus photographs of the right eye.

He was treated with oral valgancyclovir, but 3 weeks later, the lesions had progressed, prompting admission for intravenous ganciclovir before being lost to follow-up for 1 month.

The patient was presumptively diagnosed as having CMV retinitis based on the history of CMV retinitis in the contralateral eye, and the findings of hemorrhagic retinitis adjacent to chorioretinal scars suggestive of prior, healed CMV retinitis.

A, Inferotemporal area of retinal whitening (white arrowhead) with intraretinal hemorrhage adjacent to chorioretinal scars (black arrowhead).

There is retinal vascular sheathing within the area of retinitis.


Findings from the examination were remarkable for reduced visual acuity in the left eye (20/80), proptosis with hypoglobus in the left eye, S-shaped left blepharoptosis, and left retinochoroidal folds (Figure 1A).

B, Results of coronal, T1-weighted, postcontrast, fat-suppressed magnetic resonance imaging (MRI) of the orbits showing a 2 × 3 x 2.8-cm well-defined lesion of the lacrimal gland with inferior displacement of the globe and lateral rectus muscle.When the patient was reevaluated 2 weeks later, the pain at rest had resolved, but pain on palpation of the superotemporal left orbit persisted.

Magnetic resonance imaging (MRI) of the orbits with contrast demonstrated a well-defined superotemporal mass with homogeneous enhancement (Figure 1B).

Results of a complete blood cell count, comprehensive metabolic panel, antineutrophil cytoplasmic antibody, antinuclear antibody, Lyme titer and serum angiotensin-converting enzyme levels, chest radiograph, erythrocyte sedimentation rate, C-reactive protein levels, urinalysis, and IgG4 index were normal.

The patient’s pain nearly resolved after 3 days of intravenous methylprednisolone treatment, and he was discharged with a regimen of 80 mg/d of oral prednisone.A, Left eye at initial presentation showed proptosis with hypoglobus, S-shaped blepharoptosis, and retinochoroidal folds.

Results of the examination were otherwise unchanged.

The patient was admitted for a trial of intravenous methylprednisolone, 1000 mg, for presumed inflammatory dacryoadenitis.


She had a history of mild atopic dermatitis and was not taking any medications.

She had no known history of drug allergy or contact allergy.

The patient lived with her 2 siblings and both parents, who were all asymptomatic.

She had no pets and no recent travel, and her vaccinations were up to date.On physical examination, the patient was actively scratching the involved skin.

The rash started as small pink bumps on her arms that spread to involve the axillae, neck, and upper chest.

Four days prior to arrival, she developed significant redness and pruritus of her face.

On arrival, her parents noted black streaks on her right arm, prompting an urgent emergency department evaluation.

The bilateral arms, axillae, anterior neck, and face had poorly defined, blanching pink-red patches and edematous plaques without epidermal change (Figure, A).

Jet black, well-defined, and slightly shiny linear streaks with peripheral erythema were noted on her right forearm, with smaller circular, black macules on the ipsilateral biceps and axilla (Figure, B).Widespread pruritic eruption.

A, Poorly defined, blanching pink-red patches and edematous plaques on the upper chest, neck, proximal arms, and face.

B, Jet black, well-defined, and slightly shiny linear streaks with peripheral erythema on the right forearm.

Treatment prior to presentation included diphenhydramine, cetirizine, and a 20-mg dose of prednisone without improvement.

The patient was otherwise healthy with normal development and growth.


Angiography demonstrated widely patent grafts to the left anterior descending and right coronary arteries and a totally occluded graft to the obtuse marginal branch, which was known from a prior cardiac catheterization.

Disease in the obtuse marginal branch appeared stable, but there was worsening of disease in the ramus branch, where 2 serial lesions were identified.

Intravenous adenosine was administered at 140 µg/kg/min to induce maximal hyperemia, and fractional flow reserve (FFR) was measured in the ramus, revealing a nadir value of 0.43 across both lesions (Figure 1).

A slow, pull-back maneuver across the distal lesion demonstrated that the proximal lesion had an FFR value of 0.82.A, Angiography shows 2 serial lesions of intermediate severity in the ramus intermedius (red arrowheads).

B, Fractional flow reserve (FFR) measurement was performed after induction of maximal hyperemia, demonstrating a nadir value of 0.43.

HR indicates heart rate; iPa, instantaneous proximal (aortic) pressure; iPd, instantaneous distal (coronary) pressure; Pa, proximal (aortic) pressure; and Pd, distal (intracoronary) pressure.The proximal lesion is ischemic while the distal lesion is nonischemic.

Only the proximal lesion should have a stent placed.The distal lesion is ischemic while the proximal lesion is nonischemic.

Only the distal lesion should have a stent placed.Both lesions are ischemic and should have a stent placed.FFR with adenosine should be remeasured after placing a stent in the distal lesion to better assess the proximal lesion.


His medical history was notable for bilateral retinoblastoma when he was an infant, which was treated with right-eye enucleation and left-eye irradiation.

As a teenager, he developed an advanced malignant small blue cell tumor, which was interpreted as an olfactory neuroblastoma and treated with radiation and chemotherapy.

When the patient returned 2 months later with increasing pain, endoscopy revealed a polypoid lesion occupying the biopsied site.

On examination, the patient had a hard, fixed, rounded 2-cm lesion in the right supraorbital region.

Computed tomography (CT) showed an irregular mass in the right frontal, maxillary, and ethmoid sinuses with surrounding sclerotic and erosive bony changes.

A fine-needle aspirate (FNA) revealed atypical, mitotically active cells.

Bone shavings obtained operatively were interpreted as chronic sinusitis with sclerotic bone.

The surgical specimen showed multinucleated giant cells and bizarre stromal cells suggestive of treatment effect (Figure, A) and normal-appearing bone.

On repeated operative biopsy, histologic examination showed similar giant cells and atypical stromal cells (Figure, B).

However, other areas demonstrated osteoid formation with fine calcifications and severely atypical spindle cells with mitotic figures (Figure, C), as well as osteoid-entrapped, malignant-appearing cells (Figure, D).

A second opinion was reported as chronic sinusitis and atypical stromal cells.


His extensive medical history was notable for congestive heart failure, Ménière’s disease after multiple endolymphatic shunts, a benign parotid tumor (which had been excised), and chronic abdominal pain secondary to pancreatic steatorrhea.

His only symptom was frontal and ethmoid sinus pressure.

He denied difficulty breathing, postnasal drip, rhinorrhea, epistaxis, or anosmia and had no history of sinus or upper respiratory infections.

Flexible nasal endoscopy confirmed a central posterior bulge in the posterior nasal cavity with normal overlying mucosa and clear nasopharynx.

The noncontrast CT bone window showed an expansile anterior nasal septum mass measuring 1.9 × 2.4 cm with internal matrix mineralization (Figure, A-C).

A complete septectomy was performed, which involved near-complete resection of the quadrangular cartilage and resection of the adjacent anterior portion of the periosteum of the vomer bone.

Biopsy and magnetic resonance imaging (MRI) were recommended as baseline studies for follow-up, but were not completed.

A histological specimen is shown in the Figure, D.A-C, Computed tomographic images without contrast of the head using a bone algorithm.

D, Histopathological image from the nasal cavity mass.


She reported return of her symptoms after 11 months to include mouth breathing, snoring, and nasal obstruction with hyponasality and eustachian tube dysfunction.

She also began to experience transient upper extremity paraplegias.

On examination, she was noted to have abnormal asymmetry of the posterior nasopharyngeal wall by flexible fiber-optic laryngoscopy without any overlying mucosal changes.

Magnetic resonance imaging revealed a complete recurrence and enlargement of the retropharyngeal mass with dimensions of 4.2 (anterior to posterior) × 6.3 (transverse) × 5.1 (craniocaudal) cm.

The mass was heterogeneously hyperenhancing on T1-weighting and centered at the atlantoaxial joint causing erosion of the upper dens, anterior arch of C1, and the basion (Figure).A, T1-weighted magnetic resonance imaging (MRI).

She required hospital admission with steroid and anti-inflammatory treatment for partial resolution of symptoms.

Arrowhead indicates soft tissues; asterisk, thecal sac.

C, Fat-saturated T1-weighted postcontrast MRI.

D, Computed tomographic image (CT), bone windows.


Computed tomography (CT) of the temporal bone detected middle ear, mastoid, and infralabyrinthine involvement (Figure, A).

Gadolinium-enhanced magnetic resonance imaging (MRI) of the brain demonstrated meningitis and a large cerebellar lesion with rim enhancement.

Moreover, diffusion-weighted imaging (DWI) images and apparent diffusion coefficient (ADC) maps were consistent with cerebellar abscess (Figure, B-D).Imaging of the head.

She had undergone an emergency craniotomy for brain decompression the day before.

She was receiving intravenous antibiotics and steroids and was sedated and mechanically ventilated.

ADC indicates apparent diffusion coefficient; CT, computed tomography; FLAIR, fluid attenuated inversion recovery; MRI, magnetic resonance imaging.


She had no history of trauma, and there was no response to steroids.

She had not experienced headaches, disequilibrium, or tinnitus.

Findings from a physical examination were notable for left House-Brackmann grade III/VI facial palsy, hypoesthesia in left trigeminal nerve distribution, and no left audiologic responses to bone or air stimuli.

A non–contrast-enhanced computed tomographic (CT) scan showed a left petrous apex expansile lesion involving the internal auditory canal (Figure, A).

Magnetic resonance imaging (MRI) showed a well-circumscribed lesion (Figure, B) extending into the fundus of the internal auditory canal (Figure, C).

There was a fluid-fluid level in the lesion (Figure, B), consistent with presence of blood products.

The displaced adjacent cranial nerve V (Figure, C) extended along the surface of the lesion.

After the administration of an intravenous contrast agent, there was a nodular focus of enhancement at the anterior aspect of the lesion (Figure, D) and linear areas of enhancement at the periphery and of internal septations (Figure, D).

The lesion was completely excised via a middle fossa craniotomy.

Postoperatively, the patient regained full function of all cranial nerve deficits, including the hearing loss.


The symptoms started approximately 3 months prior and had progressively worsened with interval difficulty weight bearing and ambulating up and down stairs.

The patient noted night sweats 2 weeks prior to presentation but denied any associated fevers, unintentional weight loss, fatigue, or change in appetite.

Physical examination revealed limited range of motion of the left leg related to pain.

There was no cervical, axillary, or inguinal lymphadenopathy.

Laboratory evaluation demonstrated a hemoglobin level of 10.3 g/dL and a lactic acid dehydrogenase level of 599 u/L.

Serum protein electrophoresis testing did not detect a monoclonal protein.

A plain radiograph of the left femur showed a 15-cm area of moderate destruction in the proximal femoral shaft extending distally from the level of the lesser trochanter with concern for impending fracture (Figure 1A and B).

A computed tomography (CT) scan of the chest, abdomen, and pelvis did not visualize any metastatic disease, and results were negative for lymph node enlargement.

A nuclear medicine bone scan demonstrated increased uptake in the left femur with no other focal abnormalities.

The patient underwent a CT-guided needle biopsy of the left femur (Figure 1C).A and B, Radiographs of the left femur; C, biopsy specimen; original magnification × 400.


He underwent colostomy after unsuccessful surgical correction for an enterocutaneous fistula and had required total parenteral nutrition (TPN) for the past year.

He had not noticed any significant changes in ostomy output during this time.

The rash was accompanied by diffuse alopecia of the face, scalp, axillae, and groin (Figure).

Generalized alopecia and erythematous, scaly rash involving face and scalp.Dermatologic examination revealed widespread erythematous plaques on the face, scalp, and medial aspect of the thighs bilaterally and scattered papules with scaling on the face, scalp, chest, and back.

Diffuse, nonscarring alopecia was present on the face, scalp, axillae, and groin.

There was no improvement in the rash despite multiple rounds of oral fluconazole, topical antibiotic ointment, and intravenous antibiotics.

Prior to development of the rash, he completed neoadjuvant chemotherapy followed by surgical resection.


She reported night sweats and some excessive fatigue over the last few months.

Her physical examination revealed normal heart sounds and no splenomegaly (Table).The patient has a low-normal serum erythropoietin level.

She should be tested for JAK2V16F mutation status.The patient likely has familial polycythemia given her low-normal erythropoietin level.

She was unaware of any family history of blood disorders.

She should be referred for genetic counseling.The patient likely has secondary causes of her elevated hematocrit level.

No further workup is needed.These findings are likely due to dehydration.

Further testing would likely indicate a normal red blood cell mass with a low plasma volume suggesting dehydration.


He was cold to the touch; blood pressure was 85/55 mm Hg, heart rate was 36/min, respiratory rate was 14/min, and pulse oximetry was 100% on ambient air.

Multiple temperature measurements, including a core temperature assessment via the rectal method, did not yield a detectable temperature.

His chest examination was clear to bilateral auscultation.

Chest radiographs and computed tomography of the head were negative for any acute pathology.

His electrocardiogram (ECG) on presentation is shown in Figure 1.

Routine laboratory tests were ordered, and he was started on an intravenous fluid.


The lesions had repeatedly recurred despite topical steroid therapy.

Physical examination revealed papules and plaques ranging up to 1 cm in diameter on the lateral cheeks and behind the ears and neck along with a red-brown hue (Figure, A).

Complete blood cell count and comprehensive metabolic panel results were unremarkable.

However, serum protein electrophoresis revealed polyclonal hypergammaglobulinemia.

Antinuclear antibody and anti-Ro/SS-A and anti-La/SS-B antibodies were undetectable by indirect immunofluorescence or double immunodiffusion methods.

Computed tomography (CT) revealed bilateral parotid gland swelling together with mild lymphadenopathy in the mediastinum and hilus.

B and C, Hematoxylin-eosin–stained skin biopsy specimens taken from papules show nodular inflammatory cell infiltration consisting of dense lymphocytes admixed with plasma cells and eosinophils.

D, Papule specimen under IgG4 immunohistochemical stain.

A skin biopsy specimen was obtained from a papule on the neck for further analysis (Figure, B-D).A, Indurated papules and plaques are present on the lateral cheeks and behind the ears and neck along with a red-brown hue.


Dermatology was consulted for evaluation of 5-day history of new tender lesions on the scalp.

The patient did not have a history of recent travel or trauma.

Flow cytometry showed myeloid blasts, most of which (≥90%) expressed CD13, CD33, CD117, and partial CD11b (≥50%).

Blasts were also “highly positive” for myeloperoxidase.

Stains were weak for CD4 and were negative for CD34 and HLA-DR. Cytogenetic studies revealed a normal karyotype with NPM1 and FLT3-ITD mutations.

Physical examination revealed several tender purpuric papules and plaques with central necrosis and hemorrhagic crusting on the scalp (Figure, A).

Similar lesions were present on the right dorsal hand and left lower leg.

On the right forearm, there was a tender erythematous plaque with overlying vesicles and mild hemorrhagic crusting.

On the left medial thigh, there was a large indurated, tender red-brown plaque.

Wound cultures and polymerase chain reaction studies from the right forearm were negative for herpes simplex virus and varicella zoster virus infections.

Punch biopsy specimens from lesions on the scalp, right forearm, and left thigh were sent for histopathological and immunohistochemical assessment and tissue culture (Figure, B-D).A, Clinical image of tender purpuric papules and plaques with central necrosis on the scalp.

B-D, Histologic images of a punch biopsy specimen from a scalp lesion.


He had a past medical history significant for metastatic renal cell carcinoma, a lung transplant secondary to chronic obstructive pulmonary disease, end-stage kidney disease being treated with hemodialysis, and multiple nonmelanoma skin cancers.

A dermatologist was consulted during his admission to evaluate a friable, growing lesion on his right elbow that had appeared 2 months prior.

Physical exam revealed 2 closely adjacent dome-shaped erythematous nodules with edematous borders (Figure, A).

A 4-mm punch biopsy was performed.A, Two closely adjacent friable, erythematous nodules with rolled borders on the left elbow.

B, Pseudoepitheliomatous hyperplasia of the epidermis with focal ulceration and multiple collections of neutrophils.

C, Mixed inflammatory infiltrate containing neutrophils, plasma cells, histiocytic, and multinucleate giant cells containing refractile spore-like structures.

D, Numerous spores and pseudohyphae staining positively for pigment.


This was her first episode, and she denied any pain.

C, Fluorescein angiography demonstrates hyperfluorescence of an arterial midsegment with distal nonperfusion.The next day, the patient developed severe headaches and presented to the local emergency department.

Visual acuity was 20/25 + 2 OD and 20/20 OS.

Confrontation visual fields detected a superior field defect in the left eye.

Pupils, intraocular pressures, and anterior segment examination results were within normal limits.

Dilated fundus examination revealed whitening and attenuation of a small arteriole in the inferior arcade of the right eye (Figure, A).

Examination of the left eye revealed an inferior branch retinal artery occlusion and a patch of retinal whitening superior to the optic disc approximately one-third disc diameter in size (Figure, B).

Fluorescein angiography demonstrated segments of arteriole hyperfluorescence with distal nonperfusion in both eyes (Figure, C).

This hyperfluorescence and nonperfusion occurred within straight arteriole segments, not at arteriole bifurcations.

An outpatient workup composed of fluorescent treponemal antibody absorption, QuantiFERON–TB Gold (Quest Diagnostics), erythrocyte sedimentation rate, antinuclear antibody, complete blood cell count, prothrombin time, and partial thromboplastin time was ordered.A, Fundus photograph of the right eye shows subtle whitening and attenuation of an arteriole.

The differential diagnosis included systemic lupus erythematosis, polyarteritis nodosa, Behçet disease, granulomatosis with polyangiitis (Wegener disease), acute retinal necrosis, syphilis, and hypercoagulability.

B, Left eye illustrates an inferior branch retinal artery occlusion and retinal whitening superior to the optic disc.


She had been seen 7 months prior to presentation for a comprehensive eye evaluation.

She was found to have a bilateral posterior vitreous detachment, and the results of her retinal examination at that time were normal.Her best-corrected vision was 20/20 OU, and the intraocular pressure in each eye was normal.

An anterior segment examination of the left eye revealed trace keratic precipitates on the inferior cornea and 1+ cell.

A posterior segment examination revealed 1+ vitreous cell.

A photograph from a fundus examination is shown in Figure 1.


He had no other medical history or medication use.Ocular and skin findings.

He also noted red spots on his arms and legs.

He denied blurry vision, discharge, photophobia, fevers, allergies, or recent illnesses.

The patient had conjunctival hyperemia in his left eye.

A, Left eye with nasal hyperemia and conjunctival foreign body.

B, Elevated erythematous lesions on extremity.Physical examination demonstrated discrete papules 5 mm in diameter with dark centers, localized to his extremities and trunk (Figure, B).

His wife had picked him up after his hike and developed a similar lesion on her left arm.


She claimed to be in good health and denied any concurrent medical conditions.

She had undergone a weight loss procedure known as a duodenal switch the year prior.On ophthalmologic examination with a Snellen eye chart, her visual acuity was best corrected to 20/40 OD and 20/30 OS.

She had little to no improvement over the last few months with an artificial tear solution.

She had a marked uptake of lesions, which were revealed with lissamine green stain on the conjunctivae, and an overall appearance of diffuse punctate keratopathy.

She had bilateral conjunctival lesions with a foamy appearance both temporally and nasally (Figure).

Her pupils were normal without afferent pupillary defect.

The lenses were clear, and ophthalmoscopy revealed no abnormal findings.

Macular optical coherence tomography revealed normal contour with a bilateral cube volume of 9.4 mm3 and a central subfield thickness of 220 and 222 μm for the right and left eyes, respectively.

Visual field testing revealed general constriction bilaterally.Conjunctival lesion stained with lissamine green.


She underwent gastric surgery more than 25 years ago, of which no details were available.On general physical examination, her vital signs were stable.

The results from initial blood tests, including hemogram, liver and kidney function tests, and serum amylase, were unremarkable.

Contrast-enhanced abdominal computed tomography was performed (Figure 1).


On review of systems, she endorsed anorexia and malaise for the past several days.

She was in no acute distress but appeared languid.

She was found to have severely elevated blood pressure at an urgent care center, prompting her transfer to the hospital.

Physical examination demonstrated periorbital and 1+ bipedal pitting edema.

She had mild tenderness to percussion of both flanks.Serum studies revealed a creatinine level of 13 mg/dL (to convert to micromoles per liter, multiply by 88.4), potassium level of 6.6 mEq/L (to convert to millimoles per liter, multiply by 1.0), and bicarbonate level of 16 mEq/L (to convert to millimoles per liter, multiply by 1.0).

Her serum calcium level was 8.5 mg/dL (to convert to millimoles per liter, multiply by 0.25) and her serum phosphate level was elevated at 5 mg/dL (to convert to millimoles per liter, multiply by 0.323).

Computed tomography of the abdomen and pelvis revealed severe bilateral hydronephrosis with renal cortical thinning and obstructing calculi at both ureteropelvic junctions (Figure 1).

Interestingly, her serum calcium level elevated to 12 mg/dL and her serum phosphate level became low at 2.4 mg/dL.Computed tomographic scans of the abdomen and pelvis without contrast, showing obstructing calculi bilaterally at the ureteropelvic junctions (arrowheads) with resultant severe hydronephrosis and renal cortical thinning (A) and significant renal stone burden in the lower pole of the left kidney (B).

The patient underwent emergent dialysis followed by placement of bilateral percutaneous nephrostomy tubes.

Subsequently, her blood pressure normalized, edema improved, hyperkalemia and metabolic acidosis resolved, and serum creatinine level decreased to 1.6 mg/dL.


His parents said that he is a “picky eater,” with a diet consisting exclusively of orange soda, meat, and chili.

His mother reported that he was having difficulty grasping objects and recently started biting his fingers.

A physical examination revealed a very irritable, inconsolable child with diffuse muscle tenderness.

The results of an extremity examination were consistent with carpal spasm, with a fully extended first finger with hyperextended distal interphalangeal joint, thumb constantly adducted to the hand, and last 3 digits mildly flexed and adducted together.

The extremity examination also revealed twitching of facial muscles in response to tapping over area of facial nerve (ie, a positive Chvestok sign1).

Laboratory studies confirm hypocalcemia (with a calcium level of 5.5 mg/dL [to convert to millimoles per liter, multiply by 0.25]), and a diagnosis of tetany is made.

Additional physical examination findings include wrists that are unusually wide with prominent styloid processes of the radius and ulna; radiographs were abnormal (Figure, A and B).A, The patient’s wrist is unusually wide and notable for prominent styloid processes of the radius and ulna.

B, Wrist radiograph shows widening of the physes of the distal radius.

There is associated transverse splaying, fraying, and cupping of the adjacent metaphyses, with loss of the zone of peripheral calcification.Further investigation revealed a parathyroid hormone (PTH) level of 798 pg/mL (normal range, 15-87 pg/mL [to convert to nanograms per liter, multiply by 1.0]), a vitamin D level of 7 pg/mL (normal level, >25 pg/mL [to convert to picomoles per liter, multiply by 2.6]), and a normal phosphorous level of 4.5 mg/dL (to convert to millimoles per liter, multiply by 0.323).

Oral and intravenous supplementations of calcium were started, but the patient’s calcium levels remained refractory, even after correction of other electrolytes, including potassium and magnesium.

He was placed on a continuous intravenous calcium infusion for 3 days with eventual normalization of his calcium level.


His medical history was significant with hypertension, intracranial hemorrhage, and chronic kidney disease (CKD).

After admission, his chest discomfort resolved in 2 days, but he was still febrile, and pericardial effusion did not decrease.

On arrival, his blood pressure was 130/64 mm Hg, heart rate was 104 beats/min, respiratory rate was 32 breaths/min, body temperature was 37.1°C, and arterial oxygen saturation was 95% on room air.

There was no murmur, rub, or gallop in chest auscultation.

Electrocardiographic (ECG) findings were significant for diffuse ST-segment elevation (Figure 1, A).

Laboratory examination revealed leukocytosis with normal cardiac enzyme levels and mild renal dysfunction (serum creatinine, 1.4 mg/dL [to convert to micromoles per liter, multiply 88.4]).

The C-reactive protein level was also elevated.

Computed tomography (CT) of the chest was performed (Figure 1, B and C).

He was admitted to the hospital with the suspected diagnosis of acute pericarditis.


His family history was positive for a similar presentation involving his mother.

Physical examination revealed left orbital swelling and erythema with proptosis.

Cranial nerve function and ocular range of motion were intact.

Contrast-enhanced computed tomographic (Figure, A) and magnetic resonance imaging studies of the paranasal sinuses (Figure, B) disclosed extensive preseptal and postseptal orbital inflammation, bilateral frontal sinus opacification from a large left frontal osteoma occupying the inferior aspect of the left frontal sinus, and left epidural abscess.

Genetic analysis was positive for mutation in the APC gene at 5q21, resulting in a stop codon.A, Contrast-enhanced computed tomographic (CT) in bone windows.

The patient underwent a bicoronal approach osteoplastic flap and endoscopic frontal sinusotomy for access and drainage of left frontal sinus disease and obstructive osteoma excision.

The patient’s postoperative period progressed without incident, and he was discharged home with oral antibiotics following resolution of symptoms.

In addition to frontal sinus osteoma, multiple 3- to 5-mm osteomas covering the calvarium were encountered (Figure, C).

Histopathologic findings of operative specimens showed dense lamellar bone (Figure, D).

B, T1-weighted magnetic resonance image (MRI).

C, Reflected osteoplastic flap, frontal sinusotomy.

D, Hematoxylin-eosin, original magnification ×400.


His medical history was significant for hypertension, dyslipidemia, and cigarette smoking.

Flexible fiber-optic laryngoscopy revealed a 15-mm submucosal swelling in the right aryepiglottic fold.

The vocal folds were free of lesions and had intact motion.

Findings from the rest of the head and neck examination were unremarkable.

A computed tomographic (CT) scan of the neck revealed a submucosal hypodense supraglottic mass on the right side that was compressing the airway.

A magnetic resonance imaging study (MRI) of the neck demonstrated a soft-tissue mass measuring 27 × 17 mm and causing deviation of the right aryepiglottic fold medially and compressing the airway (Figure, A and B).

The patient underwent direct laryngoscopy, and a submucosal mass was observed in the right aryepiglottic fold (Figure, C).

Results from the S100 protein immunostain were positive.

The mass was dissected submucosally, excised completely, and sent for a histopathologic examination (Figure, D).

The postoperative course was uneventful, and after 5 months, the patient had no detectable laryngeal lesions and the vocal fold movement was intact.


Her medical history was notable for dyspepsia, hypertension, and diabetes.

She had no history of smoking, type B symptoms, angiotensin-converting enzyme-inhibitor use, or known cardiac pathologic abnormalities.

Initial treatment with proton pump inhibitors and evaluation by the gastroenterology unit did not improve her symptoms, so further workup was undertaken.

Findings from a complete head and neck examination were within normal limits aside from laryngeal findings.

Flexible laryngoscopy with videostroboscopy revealed right vocal fold polyp and left vocal fold desquamation, with bilateral linear bands along the vocal folds.

Noncontrast computed tomography (CT) revealed numerous calcified subglottic and tracheal nodules extending from the cricoid to the left mainstem bronchus (Figure, A).

Further examination revealed edema of the vocal folds with multiple submucosal masses in the subglottis and upper trachea (Figure, B-D).

The lesions were varying sizes, and not all were calcified.

They affected the anterior cartilaginous trachea and the posterior membranous portion.

Biopsy specimens of representative lesions revealed amorphous, eosinophilic material with calcification, metaplastic ossification, and multinucleated foreign-body giant cells.

She underwent direct laryngoscopy and bronchoscopy with biopsies.

The disease was treated with debulking of scattered lesions from the glottis through to both main stem bronchi and airway dilation, which provided symptomatic relief.A, Calcified subglottic, upper tracheal, and left mainstem bronchus lesions.

On induction, subglottic firm mass consistent with calcification prevented passage of an endotracheal tube, and the patient’s airway was secured with a rigid bronchoscope.

B, Waxy, friable deposits in the supraglottic airway.

C, Nodular lesions scattered circumferentially in the trachea.

D, Nodular lesions extending into bronchi and lower airways.


She mentioned that she had a cold sore once several years ago.

Two months after the last dose of radiation she developed pruritic pustules and papules on the skin of the ipsilateral right breast.

Physical examination findings showed pustules surrounding the areola on the right breast with an increasing number in the submammary fold (Figure 1).

Levoceterizine gave some relief of the itch.


Three years prior he had developed neutropenia and mild thrombocytopenia.

He noted fatigue, but otherwise felt well.

A bone marrow biopsy sample demonstrated refractory cytopenia with multilineage dysplasia.

Cytogenetic testing results were normal, and there were 5% bone marrow blasts.

After approximately 2 years, he developed more pronounced cytopenias and presented with a white blood cell count of 2100/μL(480 neutrophils), hemoglobin level of 8.4 g/dL, and platelet count of 92 000/μL.

It had been recently biopsied without definitive diagnosis, but since the biopsy, the mass had increased in size.An isolated polypoid mass affecting the right upper labial mucosa.

The patient was treated initially with cyanocobalamin 250 micrograms daily, then danazol 200 milligrams 3 times daily, then epoietin α 60,000 units weekly.

Filgrastim 300 micrograms weekly was added to epoietin α to attempt to augment response after 1 year of therapy.

Physical examination revealed an isolated, symptomatic polypoid mass involving his right upper labial mucosa (Figure).


The patient lived in North Carolina with no recent travel or known tick bite.

The lesion, initially attributed to a bug bite by her mother, enlarged and developed central clearing.

She had no symptoms other than mild local itching.

Examination results were normal except for a 2.0-cm annular lesion with central clearing and peripheral flaking (Figure).

After several weeks, the patient’s mother applied over-the-counter topical antifungal cream and the lesion faded.

The pediatrician ordered serologic testing for Lyme disease, prescribed 14 days of doxycycline based on the results (Table), and referred the patient to an infectious disease specialist for evaluation.Complete 14 days of antibiotic treatment for Lyme disease.

After 10 days of antifungal therapy, the patient was evaluated by her pediatrician.

No further testing is indicated.Complete 14 days of antibiotic treatment for Lyme disease.

Repeat Lyme disease serology in 1 month to evaluate response.


She was started on levothyroxine and takes no other medications.Currently, vital signs and physical examination are normal.

She was admitted recently for hypothyroidism, with a thyroid-stimulating hormone level of  209.1 µIU/mL (reference range, 0.4-4.0 µIU/mL) and free thyroxine level of 0.4 ng/dL (reference range, 0.7-1.5 ng/dL).

Complete blood cell count then revealed a leukocyte count of 3800/µL; platelet count, 158 ×103/L; hemoglobin level, 6.9 g/dL; and mean corpuscular volume, 107 μm3.

Laboratory values include a leukocyte count of 2600/µL; platelet count, 86 ×103µL; hemoglobin, 6.3 g/dL; reticulocyte count, 3.1%; and mean corpuscular volume, 106 μm3.

Levels of thyroid-stimulating hormone and free thyroxine are 31.4 µIU/mL and 0.7 ng/dL, respectively.

Lactate dehydrogenase (LDH) level is 3116 U/L (reference range, 0-271); haptoglobin, less than 8 mg/dL (reference range, 30-200); vitamin B12, 1313 pg/mL (reference range, 200-933), and serum folate, greater than 25 ng/mL (reference, >5.9).A, Peripheral blood smear showing microcytes and macrocytes, teardrop cells, absolute neutropenia, and thrombocytopenia (Wright-Giemsa, original magnification ×100).

The nucleated cell in the center is a polychromatophilic megaloblast containing a Howell-Jolly body.

A peripheral blood smear (Figure, panel A) and bone marrow biopsy (Figure, panel B) were obtained.


Three weeks prior, he had undergone coronary angiography and coronary artery bypass graft surgery after myocardial infarction.

On physical examination, he had exquisitely tender blue macules, patches, and papules on the plantar aspect of several toes (Figure 1A) and red, tender macules on his hands (Figure 1B).

Laboratory examination was notable for a white blood cell count of 9100/μL with 22% eosinophils, blood urea nitrogen level of 70 mg/dL (25 mmol/L), and creatinine level of 6.1 mg/dL (539.2 μmol/L).

Urinalysis was remarkable for 1+ blood and 2+ protein.A, Blue toes and associated skin changes over the lower extremity.


No other family members were similarly affected.

As an infant, her mother had taken her to their physician owing to dark discoloration of her diapers, but this was not investigated further.Examination revealed slate blue macular pigmentation within the antihelix and conchal bowls of both ears (Figure, A).

She had mild knee arthralgia that was treated with nonsteroidal anti-inflammatory drugs (NSAIDs).

There were prominent dark brown deposits on the conjunctivae medial and lateral to the iris of both eyes (Figure, B).

No other pigmentary changes were noted on the skin or mucosal surfaces.

A punch biopsy specimen from the pigmented area on the left ear was obtained (Figure, C).A, Examination of the left ear revealing slate blue macular discoloration, most prominent over the antihelix.

B, Examination of the left eye showing dark brown pigment deposition of the conjunctiva lateral to the iris.

Similar deposits were seen medial to the iris, bilaterally in both eyes.

C, Hematoxylin-eosin stain on punch biopsy specimen of pigmented area on left ear demonstrating large pigment deposit in the dermis and adjacent to the cartilage (original magnification ×150).

She was seeing an ophthalmologist annually regarding brown spots on both eyes diagnosed as conjunctival nevi.


The neonate was the product of a dichorionic, diamniotic twin pregnancy.

They were born to a previously healthy woman who had been hospitalized 12 days prior for tocolytic therapy.

One day prior to delivery, the mother was noted to have yeast on urinalysis.

The presence of epithelial cells in the sample suggested vaginal contamination.

Physical examination of the affected twin revealed an afebrile, 1200 g preterm infant.

Skin examination showed 100 to 200 pink papules, 0.05 to 0.2 cm in size, distributed on the trunk, extensor upper arms, and upper legs (Figure, A and B).

A subset of the lesions appeared vesiculopustular, but no fluid was evident after attempted unroofing.

The second twin had normal cutaneous examination findings.

Laboratory analysis revealed a white blood cell (WBC) count of 6000/μL, which was composed of 21% neutrophils, 38% lymphocytes, 24% monocytes, and 5% eosinophils.

Gram stain of lesional scrapings did not reveal bacteria nor WBCs, though potassium hydroxide staining showed numerous yeast forms.

Lesional polymerase chain reaction was negative for herpes simplex virus.

Bacterial cultures from the skin and the blood were ultimately negative.

The placentas from both neonates were sent for pathologic analysis (Figure, C and D).A, Pink papules and vesicopustules were noted on the trunk, arms, and extensor thighs, but spared the diaper area; B, morphology of lesions; C and D, histopathologic images of umbilical cord section.

Despite tocolytic therapy, labor ultimately progressed the following day and cesarean delivery was performed owing to breech presentation of the unaffected twin.

The other twin did not have any dermatalogic abnormalities.

The diaper area, hands, feet, nails, and oral mucosa were unaffected.


Social, family, and past medical history were noncontributory.

Physical examination revealed reddish-brown hyperkeratotic papules on the neck, trunk, and extremities and diffuse brown macules, many in a reticulated pattern, on the face, neck, trunk, and extremities.

A punch biopsy specimen was collected from both the arm and trunk and histologic examination was performed (Figure, C and D).Photographs of clinical findings of reticulate hyperpigmentation of the (A) neck and chest and (B) lower extremities.

Hematoxylin-eosin of (C) hyperorthokeratosis, epidermal acanthosis and elongated epidermal rete ridges with bud-like filiform projections and suprapapillary plate thinning (original magnification ×4) and (D) focal acantholysis without dyskeratosis (original magnification ×10).

She was treated with fluorouracil and tazarotene, 0.1%, cream with mild improvement, but she continued to develop new lesions.

She was previously diagnosed with Darier disease after biopsy revealed acantholytic dyskeratosis.


Two weeks prior to presentation, she reported a gradual increase in blurred vision with intermittent floaters in the right eye but denied pain or inciting events.

There were large keratic precipitates, 1+ anterior chamber cell and flare, and no hypopyon.

Numerous white precipitates were noted on the anterior and posterior surface of a 3-piece posterior chamber intraocular lens (Figure, A).

There were 2+ to 3+ anterior vitreous cells and a 2–disc area chorioretinal scar along the superior retinal arcades with extramacular drusen, but no areas of chorioretinitis (Figure, B).

B-scan ultrasonography showed a posterior vitreous detachment and moderate vitreous opacities.

The contralateral phakic eye showed similar drusen but was otherwise unremarkable.Chronic pseudophakic endophthalmitis.

A, Slitlamp photograph showing large keratic precipitates, anterior chamber cell and flare, and an intraocular lens with white precipitates on the anterior and posterior surface.

B, Fundus photograph showing no areas of active chorioretinitis and an inactive 2–disc area chorioretinal scar along the superior retinal arcades with extramacular drusen.The patient underwent a vitreous tap, which was negative for organisms, as well as injection of intravitreal vancomycin hydrochloride and ceftazidime in the right eye.

Topical prednisolone acetate and ofloxacin were started 4 times daily, and steroids were increased to every 2 hours while awake at follow-up.

Her BCVA improved to 20/30 OD 4 days later.

Topical antibiotics were stopped and topical steroids were gradually tapered.

The BCVA remained excellent and keratic precipitates dissipated by 1 month of follow-up, with stable findings in the posterior pole.


Her ophthalmic history was significant for mixed-mechanism glaucoma due to uveitis and chronic angle closure with prior implantation of bilateral superotemporal Baerveldt drainage implants.

In addition, she had Fuchs corneal dystrophy.

Her visual acuity worsened from counting fingers to hand motions.

At that time, visual acuity was counting fingers at 1 ft OD.

On the first postoperative visit, IOP was 14 mm Hg OD by Goldmann applanation.

On postoperative day 11, IOP remained stable at 7 mm Hg OD by Goldmann applanation and topical atropine was discontinued.

At presentation 3 weeks postoperatively, IOP was 30 mm Hg OD by Goldmann applanation.

Slitlamp examination of the right eye revealed a superotemporal Baerveldt drainage implant with fluid over the plate.

Her intraocular pressure (IOP) was controlled with topical brinzolamide.

A month earlier, she had undergone Baerveldt implant repositioning from the anterior chamber to the sulcus in the right eye in preparation for future Descemet stripping endothelial keratoplasty owing to severe corneal decompensation.

She was discharged with therapy including topical steroids, antibiotics, and atropine sulfate.

High-resolution ultrasonography was performed (Figure).High-resolution B-scan ultrasonographic image of the right eye.


She denied any history of serious infections, including tuberculosis and syphilis, and denied having family members with visual difficulties.

Her ocular history was remarkable only for bilateral cataract surgery by phacoemulsification many years prior.

The patient had no visual complaints but stated that it was difficult for her to see at night.

Visual acuity was 20/50 OU and pupillary responses, external examination, extraocular motility, and intraocular pressures were normal.

Confrontation visual field testing showed peripheral constriction in each eye.

Slitlamp examination showed the absence of anterior chamber inflammation and the presence of posterior chamber intraocular lenses and liquefied vitreous with a few vitreous strands bilaterally.

Fundus examination (Figure, A) revealed poor foveal light reflexes, an annular pattern of outer retinal hypopigmentation approximating the border of the temporal arcades, and inferotemporal clumped pigment deposits in each eye.

Spectral-domain optical coherence tomography (Figure, B) showed outer retinal foveoschisis as well as outer retinal thinning with interruptions in the ellipsoid in the peripheral macula in each eye.

Fluorescein angiography showed no disc leakage or angiographic macular edema (Figure, C); there was faint staining of the annular area of hypopigmentation seen on fundus examination.

Fundus autofluorescence showed a ring of abnormal hyperautofluorescence.A, Fundus photograph of the right eye shows poor foveal light reflex, an annular pattern of outer retinal hypopigmentation approximating the border of the temporal arcade, and inferotemporal clumped pigment deposits (seen in both eyes).

B, Spectral-domain optical coherence tomographic (SD-OCT) image of the right eye shows outer retinal foveoschisis and outer retinal thinning with interruptions of the ellipsoid in the peripheral macula (seen in both eyes).


He reported fatigue, muscle stiffness, joint pain, and a 10-lb unintentional weight loss.

On ocular examination, his best-corrected visual acuity was 20/25 OD and 20/60 OS.

Anterior segment examination findings were unremarkable.Fundus examination revealed bilateral microaneurysms and dot hemorrhages throughout the posterior pole, along with bilateral macular edema and serous neurosensory detachment in the left eye (Figure 1A).

Optical coherence tomography of the right eye revealed severe cystoid macular edema, and fluorescein angiography showed normal filling with multiple microaneurysms.

An optical coherence tomographic image of the left eye showed a large neurosensory detachment with overlying and surrounding cystoid macular edema.

Fluorescein angiography of the left eye demonstrated hypofluorescence without pooling or leakage of fluorescein dye over the area of neurosensory detachment, which was persistent in later frames (Figure 1B).A, Fundus photograph of left eye showing neurosensory detachment.


She denied any antecedent trauma or neck manipulation.

She had no known history of renal dysfunction or poorly controlled hypertension and was a lifelong nonsmoker.

She was seen in the emergency department and underwent brain magnetic resonance imaging, which demonstrated patchy enhancement in the left basal ganglia and corona radiata concerning for demyelinating lesions.

Pertinent laboratory results included C-reactive protein level of 0.01 mg/L (to convert to nanomoles per liter, multiply by 9.524), erythrocyte sedimentation rate of 6 mm/h (to convert to millimeters per hour, multiply by 1), and normal cholesterol panel.

She underwent an extensive imaging workup consisting of bilateral carotid duplex, computed tomographic neck angiography (Figure 1A) and cerebral arteriogram (Figure 1B).A, Computed tomographic arteriogram of the neck demonstrating near occlusion of the proximal left internal carotid artery (arrowhead).

B, Cerebral arteriogram demonstrating a string sign in the proximal left internal carotid artery (arrowhead).

She was admitted and underwent further workup.


The product of a full-term normal delivery, she had had occasional mouth ulcers and recurrent tonsillitis treated with oral antibiotics by her family physician.

Her vaccinations were all up to date including the measles, mumps, and rubella vaccine given 2 days before hospitalization, the day of fever onset.Admission examination revealed severe exudative pharyngitis and oral candidiasis.

Admission blood and throat culture results were negative.Two days later, at approximately 8:00 am, her mother noticed 2 purple spots in the diaper area.

By 4:00 pm, the lesions were described as firm, nontender purpuric nodules.

By 9:00 pm, the lesions had progressed, with the emergence of multiple discrete ulcers involving the labial, perineal, and gluteal areas.

Laboratory parameters included a hemoglobin level of 11.4 g/dL (to convert to grams per liter, multiply by 10), white blood cell count of 5600/μL (to convert to × 109 perliter, multiply by 0.001), absolute lymphocyte count of 5600/μL (to convert to × 109 perliter, multiply by 0.001), absolute neutrophil count of 300/μL (to convert to × 109 perliter, multiply by 0.001), platelet count of 327 × 103/μ (to convert to × 109 per liter, multiply by 1), and C-reactive protein level of 183 mg/L (normal range, <10mg/L; to convert to micromoles per liter, multiply by 9.524).

She rapidly deteriorated with onset of septic shock, her white blood cell count fell to 0/μL, and her C-reactive protein level increased to 404 mg/L.Following stabilization and initiation of broad-spectrum antibiotics, she was transferred to a pediatric intensive care unit where Figure 1 was obtained.Multiple ulcers with an erythematous rim and notable absence of pus.

Intravenous benzylpenicillin was initiated and was followed by defervescence and improvement in her condition.

She remained afebrile and clinically stable.


The resting 12-lead electrocardiogram showed sinus rhythm with no preexcitation or repolarization abnormalities.

There were incessant multiple runs of nonsustained ventricular tachycardia with right bundle branch block morphologic features.

QRS complexes were negative in the rest of the precordial leads (V2-V6) and in leads I and avL, but positive in III, avR, and avF, suggesting a left (lateral) ventricular apical origin (Figure 1).

The echocardiogram showed no structural abnormalities.

Invasive electrophysiological mapping of the left ventricle endocardium demonstrated that the ventricular tachycardia probably originated from the epicardial surface of left ventricular apex.Twelve-lead surface electrocardiogram during tachycardia.

The patient was prescribed sotalol but her symptoms did not improve.


He reported labyrinthitis 2 years previously with possible reduced hearing afterward.

He took no regular medications, had no allergies or clinically significant family history, and was an occasional smoker and alcohol drinker.

It was associated with a whistling tinnitus and occasional sharp otalgia lasting for a few minutes, approximately twice a week, without otorrhea.

Examination showed a smooth mass behind the left tympanic membrane.

Findings from flexible nasoendoscopy were unremarkable.

Pure-tone audiometry demonstrated a mixed hearing loss on the left side.

Results from tympanometry of the left ear were normal.

A computed tomographic scan showed an expansile ground-glass density lesion arising within the lateral aspect of the otic capsule on the left (Figure, A and B).

The lesion eroded into the lateral aspect of the basal and second turns of the cochlea as well as the tympanic portion of the facial nerve canal.

Magnetic resonance imaging demonstrated a 7 × 8-mm lesion arising from the otic capsule, with mild uniform enhancement following gadolinium.

A technetium bone scan showed a lesion in the temporal bone, and a focus of increased uptake in the left lateral eighth rib (no fracture was demonstrated) (Figure, C).

To obtain a diagnosis, a bony sample was obtained, which consisted of small fragments of woven bony tissue with mild osteocyte nuclei enlargement and hyperchromasia, with no sign of neoplasia.A and B, Computed tomographic (CT) images showing left otic capsule lesion.


Her medical history was significant for gastroesophageal reflux disease, hypertension, stable angina, and obstructive sleep apnea for which she was using continuous positive airway pressure therapy at night.

A repeated computed tomographic (CT) scan, when compared with a study 3 years prior, indicated clinically significant growth.

A positron emission tomographic/CT scan was significant for increased metabolic activity in the mass.

Gross examination revealed an encapsulated, 11.0 × 8.4 × 8.2-cm, tan-orange, predominantly hemorrhagic, necrotic nodule without grossly identifiable thyroid parenchyma (Figure, B).

Extensive sampling revealed pleomorphic epithelioid cells with enlarged, hyperchromatic nuclei, irregular nuclear membranes, and increased mitotic activity (Figure, C).

There was evidence of angiogenesis and formation of nests around areas with organizing hemorrhage.

Tumor cells showed strong membrane reactivity for CD31, confirming an endothelial origin of the lesion (Figure, D).

Adjacent thyroid parenchyma showed multinodular goiter and chronic lymphocytic thyroiditis.

The neck dissection revealed normal lymphatic contents.A, Intraoperative view of the mass.

Surgical intervention was recommended for alleviation of symptoms and definitive diagnosis.

A left thyroidectomy with partial right thyroidectomy (Figure, A) and a left modified neck dissection (levels II-IV) were performed.

C, Enlarged, hyperchromatic nuclei and mitoses.

D, Neoplastic cells showing immunoreactivity to CD31.


She had not experienced shortness of breath, gastric reflux, smoking, cough, dysphagia, odynophagia, fevers, or chills.

Findings from a physical examination were remarkable for a rough voice.

Flexible nasolaryngoscopy revealed normal abduction and adduction of the vocal folds with a white sessile lesion starting at the right midfold on the superior and medial surfaces that crossed the midline to involve the entire left vocal fold as shown in the Figure, A. Histopathologic images of the biopsied lesion are shown in the Figure, B-D.Laryngeal lesion.

A, Gross appearance of vocal folds by flexible nasolaryngoscopy.

B, Hematoxylin-eosin, original magnification ×400.

C, Gram stain, original magnification ×400.

D, Gomori methenamine silver stain, original magnification  × 400.


Her surgical history was significant for having undergone a cesarean section.

The incidental finding of a mediastinal mass prompted a referral to her primary care physician, who elicited from the patient some complaints of mild but worsening orthopnea that was exacerbated when she would lie down.

She denied any recent weight loss, muscle weakness, or diplopia.

A computed tomographic scan of the chest was obtained (Figure, A), and the patient was referred to surgery for evaluation.A, Computed tomographic scan of the chest revealing a mediastinal mass.

B, Intraoperative dissection following median sternotomy.A physical examination identified a midline trachea, no significant thyromegaly in the neck, no significant lymphadenopathy, and no additional significant findings.

The patient was taken to the operating room for surgery to remove the mass (with gross findings as seen in Figure, B).


A clinically significant cardiac history precluded the option of surgery.

Approximately 5 months after treatment, he awakened with abrupt onset of severe right shoulder pain.

The pain was sharp, constant, and localized to his right scapula and ultimately progressed to the point of radiating down the extensor surface of his arm ending at his elbow.

He denied paresthesias, weakness, or any inciting events that could have led to its onset.

Chest radiography identified a right lung nodule, with complete workup revealing a T2aN0M0 right upper lobe non–small-cell lung cancer (NSCLC) measuring 4.3 × 2.9 cm.

A surveillance computed tomographic (CT) scan obtained at 3 months demonstrated that the right upper lobe tumor had decreased by 1 cm in all dimensions, and at this time the patient denied any new symptoms, including pain.

His primary care physician obtained a CT scan, followed 2 days later by a magnetic resonance image (MRI) of his right shoulder (Figure 1).Right shoulder diagnostic imaging 5 months after treatment.

He was treated with a definitive accelerated hypofractionated course of radiotherapy to a total of dose 60 Gy in 8 fractions.

Acute toxic effects were not observed, with the exception of minor fatigue that resolved 1 month after treatment.

CT indicates computed tomography; MRI, magnetic resonance imaging.


Diagnostic mammography revealed 2 separate masses: a 1.4-cm mass at the 3 o’clock position, 10 cm from the nipple; and a 1.3-cm mass in the lateral breast, 4 cm from the nipple.

Ultrasound of the axilla revealed an enlarged lymph node found to be malignant on fine-needle aspiration cytology.

Image-guided core biopsies of both masses revealed a poorly differentiated invasive ductal adenocarcinoma.

Estrogen receptor (ER) was strongly positive in 95% of tumor cells, progesterone receptor (PR) was positive in less than 5% of tumor cells, and human epidermal growth factor receptor 2 (ERBB2 [HER2]) by immunohistochemistry (IHC) was scored as 1+ (Table).The result is equivocal and repeat testing is needed.


He denied any prior history of Raynaud phenomenon; heart, kidney, or liver disease; diabetic retinopathy; nephropathy; or neuropathy.

He had quit smoking 24 years ago and reported currently drinking 3 glasses of wine per week.

His medications included rosiglitazone, glimepiride, and levothyroxine.On examination, he weighed 131.5 kg (body mass index, 40.57); his heart rate was 82/min and blood pressure was 161/89 mm Hg.

Acetaminophen and meloxicam provided only partial relief.

He also noted progressive thickening of skin in the nape of his neck.

He had facial swelling and brawny periorbital edema (Figure 1A).

He also had a large area of diffuse, slightly erythematous, nonpitting woody induration extending from the nape of his neck (Figure 1B), to the upper back, shoulders, and upper arms.

This resulted in limitation of range of motion of his neck.

Examination of his heart, lungs, abdomen, and lower extremities was unremarkable.A, Facial swelling and periorbital edema.

B, Woody induration involving the nape of neck and upper back.Laboratory testing showed a fasting blood glucose level of 178 mg/dL (9.9 mmol/L).

Complete blood cell count, levels of thyroid-stimulating hormone and albumin, and the remainder of his comprehensive metabolic panel were normal.

Urinalysis showed trace protein without blood.

Erythrocyte sedimentation rate was 34 mm/h, C-reactive protein level was 0.3 mg/dL (2.9 nmol/L), and hemoglobin A1C level was 7.5%.

An antinuclear antibody panel was negative.


His medical history included type 2 diabetes mellitus, hypertension and secondary chronic renal insufficiency, cardiovascular disease, and gout.

His long-term medications included lisinopril, glipizide, terazosin, insulin glargine, labetalol, allopurinol, hydrochlorothiazide, and erythropoietin.

Examination of the head and neck revealed the incidental finding of bilateral auricular rigidity and immobility.

The patient recalled that his barber had noted an increase in difficulty trimming hair in the areas around his auricles owing to their inflexibility.

The only other notable finding was increased rigidity of his nasal cartilage.

Both ears appeared normal and were nontender and asymptomatic (Figure, A).

Hematoxylin-eosin staining of the biopsy specimen showed regions of ossification and calcification (Figure, B).

A computed tomographic (CT) scan of the head was performed within 2 weeks of his original biopsy and showed extensive calcification in the area of the auricles (Figure, C).

The patient’s laboratory workup showed an elevated creatinine level of 1.8 mg/dL (reference range, 0.6-1.2 mg/dL), which had been his baseline level for the past 2 years.

He had a hemoglobin level of 11.1 g/dL (reference range, 14-18 g/dL), which was felt to be secondary to his chronic renal failure.

His serum calcium level  had been normal with the exception of an elevated value noted of 10.4 mg/dL noted 1 year ago (reference range, 8.7-10.2 mg/dL).

An incisional biopsy of his left auricle was performed.

The patient denied any decrease in auditory acuity, frostbite, or any previous trauma to his ears.

His phosphorus, alkaline phosphorus, electrolyte, and AM cortisol levels; parathyroid function; and thyroid function were within normal limits.A, Photograph of ear.


It had started as swelling of the feet, followed by erythematous acral lesions.

Numbness of the hands and feet had begun 1½ years previously, spreading gradually to the forearm and legs.

Black eschar and ulceration with purulent discharge was seen over the soles and elbows.

There were triangular and bizarre purpuric and necrotic lesions over the legs, thighs, and dorsum of the hands (Figure, A) with necrosis of pinnae and nasolabial folds.

There was diffuse infiltration of the face and madarosis of the lateral half of the eyebrows.

He also had hypoesthesia over the palms, proximal forearms, feet, and legs.

Peripheral nerve examination revealed thickening of the right radial cutaneous and both ulnar nerves.

Bilateral inguinal lymphadenopathy was present.

Findings from the rest of the general physical and systemic examination were normal.

Punch biopsy specimens were obtained (Figure, B-D).A, Multiple bizarrely shaped purpuric and necrotic plaques on the hands and lower limbs.

He had no history of fever, joint pains, or epistaxis.


The patient admitted to occasional burning sensation of new lesions.

On review of systems, the patient denied fevers, fatigue, myalgias, arthritis, shortness of breath, oral ulcers, and recent infections.

Clinical examination revealed several erythematous patches and plaques on the arms and lower legs, and nontender violaceous firm nodules on the elbows, wrist, ankle, interphalangeal, and metatarsophalangeal joints (Figure 1A and B).

A punch biopsy specimen from a plaque on the right forearm was obtained (Figure 1C and D).


He denied headaches, fevers, cough, risk factors for immunosuppression, or travel outside of the northwestern United States.His medical history was notable for hypertension and a 50-packs-per-year smoking history.

Despite this, his leg weakness worsened.

Two months prior he had undergone lumbar vertebra 3–sacra vertebra 1 lumbar laminectomies performed for symptomatic spinal stenosis.

Physical examination was notable for a thin, frail-appearing man with normal vital signs and intact mentation.

Cranial nerve examination revealed visual acuity of 20/400 OS, with an afferent pupillary defect and left-sided facial weakness including eye closure.

He demonstrated profoundly decreased bulk and hypotonia throughout all extremities, with fasciculations apparent across his chest and lower extremities.

Lower extremity muscle strength was graded 2/5 throughout with absent reflexes and intact sensation.Initial laboratories revealed a normal complete blood cell count and chest radiography.

Magnetic resonance imaging of the neuroaxis revealed leptomeningeal enhancement around the cerebral convexities, cerebellum, and spinal cord including the cauda equina (Figure 1).

Cerebrospinal fluid (CSF) studies revealed a white blood cell count of 29/μL (to convert to × 109 per liter, multiply by .001) with a near-complete lymphocytic predominance, a red blood cell count of 363 × 106/μL (to convert to × 1012 per liter, multiply by 1), a protein level of 0.142 g/dL (to convert to grams per liter, multiply by 10), and glucose level of 19 mg/dL (to convert to millimoles per liter, multiply by 0.0555).

Cerebrospinal fluid cytology analysis did not identify malignant cells, with negative assay results for tuberculosis and fungal infections.

Repeated flow cytometry did not identify abnormal B-cell or T-cell population.

Whole-body positron emission tomography–computed tomography showed extensive leptomeningeal and nerve root hypermetabolism without evidence of a primary malignancy.Sagittal T1-weighted MRI of the lumbar spine without (A) and with (B) contrast showing leptomeningeal enhancement of the lumbar spinal cord, cauda equina, and multilevel postsurgical changes.


B, Optical coherence tomography demonstrates neurosensory retinal detachment overlying irregular retinal pigment epithelium and thickened choroid.The patient denied recent corticosteroid use, joint pain, weight loss, myalgias, or cardiovascular, neurologic, or gastrointestinal symptoms.

He had fevers and mild upper respiratory tract symptoms 2 weeks before his presentation, closely followed by a rash on his hands and thighs.

His visual acuity was 20/200 OD and 20/20 OS.

There was no afferent pupillary defect, his color vision was intact bilaterally, and his intraocular pressures were normal.

No anterior chamber or vitreal inflammation was present.

Ophthalmoscopic examination of the right macula revealed a 2–disc diameter area of heterogeneous yellow retinal pigment epithelial (RPE) discoloration with overlying neurosensory retinal detachment and several small retinal hemorrhages (Figure 1A).

The optic disc and retinal vessels were unremarkable, and ophthalmoscopic examination of the left eye revealed no abnormalities.A, Fundus photography of the right eye shows retinal pigment epithelial discoloration and retinal hemorrhages.

A young family member had similar symptoms.Spectral-domain optical coherence tomography (SD-OCT) revealed a fovea-involving neurosensory retinal detachment, outer photoreceptor thickening, and an intact external limiting membrane overlying a zone of mildly thickened, irregular RPE.

The submacular choroid was relatively thick bilaterally on enhanced depth imaging OCT (Figure 1B).

Combined fluorescein and indocyanine green angiography demonstrated mottled early hyperfluorescence with late staining and minimal pooling.

There was no leakage from the retinal vessels or optic discs bilaterally, and uninvolved areas of RPE and choroidal vasculature were unremarkable.