A 73-year-old man is brought to the emergency department 30 minutes after the sudden onset of right-sided body weakness.

His wife reports that he does not seem to understand simple questions.

He has type 2 diabetes mellitus and has smoked 1 pack of cigarettes daily for 45 years.

The patient speaks fluently, but he answers questions with nonsensical phrases and cannot repeat single words.


This patient's skin finding is most likely associated with which of the following conditions?

A 23-year-old woman comes to physician for an annual health maintenance examination.

She is 155 cm (5 ft 1 in) tall and weighs 79 kg (174 lb); BMI is 33 kg/m2.

Examination shows a skin rash over both axillae.

A photograph of her left axilla is shown.


Which of the following is the most likely underlying cause of this patient's symptoms?

A 78-year-old man comes to the physician because of a 3-week history of dyspnea, chest pain, dry cough, painful swallowing, and a feeling of fullness in his head.

The symptoms were initially mild but have progressively worsened.

He has a history of gastroesophageal reflux disease treated with esomeprazole.

His pulse is 99/min and blood pressure is 95/66 mm Hg.

Examination shows swelling and redness of the face and neck.

There is distention of the superficial veins of his neck and upper thorax.

Plain radiographs of the chest show a widened mediastinum and unremarkable lung fields.


What is the most likely explanation for this patient’s symptoms?

Implantation of the embryo outside of the uterus

Mutation of the porphobilinogen deaminase enzyme

Obstruction of the cystic duct and subsequent inflammation of the gallbladder

A 37-year-old woman is brought to the emergency department for acute abdominal pain for the past 2 hours.

She reports that the pain is constant, 10/10, and is diffusely spread across her stomach.

She reports nausea and 1 episode of vomiting 1 hour ago, painless bloody urine, and paresthesias in her hands bilaterally.

The patient claims similar episodes in the past following the use of alcohol, though with milder pain.

Her past medical history is significant for pelvic inflammatory disease that was adequately treated 5 years ago.

She is currently sexually active with her husband without contraceptive use.

Her temperature is 98.6°F (37°C), blood pressure is 148/98 mmHg, pulse is 103/min, respirations are 18/min, and oxygen saturation is 99% on room air.

A physical examination demonstrates a patient in acute distress with diffuse abdominal tenderness and decreased sensation of the hands bilaterally.


Which of the following is the most likely cause of this patient's jaundice?"

Decreased hepatic uptake of unconjugated bilirubin
"

Ten days after a motor vehicle collision, a 28-year-old man develops jaundiced skin.

Upon initial presentation for his injuries, the patient was taken for an emergency laparotomy, which revealed significant internal hemorrhage from blunt abdominal trauma to the spleen.

He required rapid transfusion with a total of 7 units of packed red blood cells.

He has recovered well from the procedure until this morning, when he began to develop jaundiced skin and sclerae.

He has had no prior surgeries and takes no other medications.

He is sexually active with one female partner.

Prior to the accident, he drank 4 beers per day.

His vital signs are within normal limits.

Abdominal examination is limited due to pain.

There is a midline surgical scar with no erythema, purulence, or drainage.

He has healing abrasions on the upper left side of his face and bruises over the anterior abdomen.

CT scans show a resolving hematoma in the peritoneal cavity.

Laboratory studies show: | Hemoglobin 9.7 g/dL | Hematocrit 30% | Leukocyte count 7,000/mm3 | Platelet count 135,000/mm3 | Serum | Total bilirubin 3.9 mg/dL | Indirect bilirubin 3.7 mg/dL | Direct bilirubin 0.2 mg/dL | Aspartate aminotransferase (AST) 60 U/L | Alanine aminotransferase (ALT) 92 U/L | .


Which of the following is the most likely diagnosis?

A 69-year-old man comes to the physician because of a cough for the past 3 months.

The cough is mostly dry, but the patient recalls a recent episode in which he coughed up mucus with dark red streaks.

He has felt tired for the past few months.

Over the past month, he has had poor appetite and a 5-kg (11-lb) weight loss.

He also has occasional back pain, which has worsened in recent weeks.

He has smoked a pack of cigarettes daily for 30 years.

Auscultation of the lungs reveals wheezing in the lower right lung field.

The liver is of normal size, and the spleen is not palpable.

Laboratory studies show a hemoglobin concentration of 13.5 g/dL, serum calcium concentration of 12.3 mg/dL, and a total serum protein of 7.0 g/dL.


What is the most likely cause of this patient’s condition?

A 16-year-old boy is brought to the emergency department by his parents after collapsing at home.

He was resting at home after an uneventful dental procedure that involved the extraction of several teeth.

He became drowsy and then unconscious and was unrousable.

At the hospital, his temperature is 37.0° C (98.6° F), respiratory rate is 15/min, pulse rate is 67/min, and blood pressure is 122/98 mm Hg.

The patient is deeply cyanosed despite a good respiratory effort and a clear airway.

His lungs are clear to auscultation, bilaterally.

Even though an endotracheal tube is introduced and assisted ventilation is induced, his condition does not improve.

A review of dental records reveals the details of the procedure where the local anesthetic pilocarpine was administered.


Which of the following is the most likely underlying cause of this patient's upper extremity symptoms?

Damage to the upper trunk of the brachial plexus

Damage to the lower trunk of the brachial plexus

Reason for Visit/Chief Concern: brought in by her parents because “her arm looks funny”.

History of Present Illness: mother had no prenatal care.

labor was spontaneous with rupture of membranes yielding fluid with dark green streaks.

the infant was delivered vaginally 1 day ago at home at approximately 39 weeks' gestation.

the delivery was complicated by shoulder dystocia, which was managed with suprapubic pressure and the McRoberts maneuver.

father reports that the infant's right arm “just hangs by the side” and that she never bends her right elbow.

the infant is breastfeeding, stooling, and voiding without complication.

Family History: mother has type 2 diabetes mellitus.

Immunizations: has not received any routine vaccinations.

Social History: the infant lives with her mother, father, and paternal grandmother.

Temp Pulse Resp BP O2 Sat Ht Wt Head circumference.

Appearance: well-appearing; crying during the examination.

HEENT: red reflex is seen bilaterally; there is a fluctuant area over the left parietal bone that crosses suture lines.

Cardiac: regular rate and rhythm; normal S1 and S2; no murmurs, rubs, or gallops.

Abdominal: no tenderness, masses, or hepatosplenomegaly; bowel sounds normal; umbilical stump is intact and clamped.

Extremities: hips are stable bilaterally.

Musculoskeletal: clavicles are intact bilaterally; the right upper extremity hangs limply from the shoulder in full extension, adduction, and fixed internal rotation; the hand is pronated, and the wrist and fingers are flexed.

Neurologic: normal suck and grasp reflexes; the Moro reflex is normal in the left upper extremity and absent in the right upper extremity; deep tendon reflexes are 2+ bilaterally. Please refer to the summary above to answer this question.


A 70-year-old man is brought to the emergency department because of severe back pain that began when he was lifting a box 1 hour ago.

He also has a 2-year history of increasingly severe right hip pain.

Physical examination shows tenderness to palpation of the lower spine as well as erythema of the skin over the right hip.

Neurologic examination shows decreased hearing in the left ear; the Weber test localizes to the left side.

Serum studies show an alkaline phosphatase concentration of 410 U/L, calcium concentration of 9.5 mg/dL, and parathyroid hormone level of 322 pg/mL.

An x-ray of the spine shows a fracture of the L4 vertebra.


An 8-year-old African-American male is found to have a holosystolic, harsh-sounding murmur upon physical examination.

The murmur is best appreciated at the left sternal border, and is found to be louder when the patient squats.


A 55-year-old woman comes to the office complaining of a dry mouth for the past few months.

She has to drink water more frequently, as she finds it difficult to chew and swallow solid foods.

She has to wake up 3–5 times each night to drink due to intense thirst.

She also complains of a foreign body sensation in both the eyes for the past month.

She has had no joint pain, fever, weight loss, or urinary or bowel changes.

She does not smoke cigarettes but drinks alcohol socially.

Her mother has rheumatoid arthritis for which she takes methotrexate, and her father died of prostatic carcinoma 7 years ago.

Her temperature is 36.7°C (98°F), blood pressure is 130/75 mm Hg, pulse is 80/min, respirations are 14/min, and BMI is 28 kg/m2.

On examination, the eyes and oral cavity appear dry, and dental caries are present.

Cardiopulmonary and abdominal examinations are negative.

Laboratory results are shown below: | Complete blood count: | Hemoglobin 10 g/dL | Leukocytes   13,500/mm3 | Platelets 170,000/mm3 | ESR 65 mm/hr |    Antinuclear antibody Positive | Rheumatoid factor Positive | Anti dsDNA Negative | Anti Ro Positive | Anti-CCP Negative | Anti Jo 1   Negative | .


Which of the following is the most likely diagnosis in this patient?

A 35-year-old man presents with loose stools and left lower quadrant abdominal pain.

He says he passes 8–10 loose stools per day.

The volume of each bowel movement is small and appears mucoid with occasional blood.

The patient reports a 20-pack-year smoking history.

He also says he recently traveled abroad about 3 weeks ago to Egypt.

The vital signs include: blood pressure 120/76 mm Hg, pulse 74/min, and temperature 36.5°C (97.8°F).

On physical examination, mild to moderate tenderness to palpation in the left lower quadrant with no rebound or guarding is present.

Rectal examination shows the presence of perianal skin ulcers.


Which of the following is the most likely etiology of this patient’s condition?

A 50-year-old man presents for a routine examination.

Past medical history is significant for cirrhosis secondary to hepatitis C virus (HCV) infection diagnosed 4 years ago and complicated by ascites.

Current medications include furosemide 40 mg orally daily.

Laboratory findings are significant for the following: | Laboratory test | Aspartate Aminotransferase (AST) 80 U/L | Alanine Aminotransferase (ALT) 50 U/L | Total bilirubin 2.5 mg/dL | Direct bilirubin 1.8 mg/dL | Alkaline phosphatase (ALP) 140 U/L | International normalized ratido (INR) 1.9 | Serum creatinine 1 mg/dL | Urinalysis | Sodium 200 mmol/24h | Potassium 60 mmol/24h | Protein Nil | RBCs Nil | RBC casts Nil | WBCs Nil | Urea 13 g/24h | Creatinine 6 mmol/24h |   Abdominal and renal ultrasound reveals no interval change over the past 6 months.

Upper GI endoscopy reveals esophageal varices with a hepatic venous pressure gradient measuring 14 mm Hg.

Diagnostic paracentesis is performed and yields a clear liquid with an absolute polymorphonuclear neutrophil (PMN) count of 75 cells/mm3.


What is the most likely cause of this patient's menorrhagia?

A 25-year-old woman comes into your office with complaints of heavy bleeding.

She states that her mother also has heavy bleeding during her menstrual cycle.

She has had a heavy flow as long as she can remember and had her first menstrual cycle at age 12.

She states during her cycle, she has to change pads every 2 hours for at least 3 days.

She also states that she gets bruised easily just like her mother.

She denies any past medical history other than her heavy menstrual flow and denies taking any medications.

She also denies any medical history in her father and says he is "perfectly healthy." Her vitals are HR 85, T 98.8 F, RR 13, BP 125/75.

Her CBC is significant for Hgb 10.5, WBC 5.8, Plts 250, Hct 33.

On coagulation studies, her PT is 14 seconds and her PTT is 43 seconds.


Which of the following is the most likely underlying cause of this patient’s current condition?

A 14-year-old girl is brought to the emergency department because of occipital headache, nausea, and vomiting for the last 2 hours.

She has a multi-year history of frequent left ear infections and discharge, with poor response to antimicrobial therapy.

She has muffled hearing in the left ear.

Her blood pressure is 134/78 mm Hg, the pulse is 83/min, the respiratory rate is 16/min, and the temperature is 36.5°C (97.7°F).

Physical examination of the left ear shows perforation of the tympanic membrane, granulation tissue, and white keratinaceous debris in the posterosuperior quadrant of the tympanic membrane.

An MRI shows evidence of sigmoid sinus thrombosis on the left side and a hyperintense area in the middle ear on.


A 23-year-old woman comes to the physician for a 6-month history of dry cough, hoarseness, and chest pain.

She does not smoke and has not lost weight.

Laboratory studies show no abnormalities.

An x-ray of the chest shows a mass that projects across the right hilum.


A 32-year-old male presents to his primary care physician with complaints of chronic headaches that have developed and increased in frequency and severity over the last several months.

Additionally, the patient has noted he has been less coordinated over the last few weeks, stumbling and tripping often when he is walking.

Physical examination is significant for notably reduced hand grip strength bilaterally as well as decreased pain and temperature sensation along the upper back and down both arms to the hands.

A referral to the appropriate specialist is made, and an MRI of the brain and neck is obtained.

Results of the MRI are show in Figures A and B.


A 17-year-old girl comes to the primary care clinic with her father complaining of right knee pain.

She reports that the pain started about a month ago, and since then it has gotten progressively worse.

The knee pain is not constant but becomes most noticeable when going up or down the stairs.

She also endorses that her knee becomes uncomfortable towards the end of class.

Her father is worried because the pain is affecting her ability to play basketball, and she has college scouts coming to watch her play.

The patient has no chronic medical conditions.

She takes a multivitamin and uses ibuprofen as needed for the pain.

On physical examination, there is tenderness at the inferior pole of the patella, without swelling or overlying skin changes.


Which of the following is the most likely diagnosis?"

A 13-month-old boy is brought to the physician for the evaluation of rectal bleeding that occurred earlier that morning.

The patient has also had several itchy and red skin lesions that started on his scalp and spread downwards.

The parents report that their son has had six episodes of bilateral otitis media since birth.

He is at the 3rd percentile for height and weight.

His vital signs are within normal limits.

Examination shows several eczematous lesions over the scalp, neck, and upper and lower extremities, as well as multiple red spots that do not blanch on pressure.

The remainder of the physical examination shows no abnormalities.

Laboratory studies show: | Hemoglobin 9.4 g/dL | Leukocyte count 11,500/mm3 | Platelet count 30,000/mm3 | Prothrombin time 14 sec | Partial thromboplastin time 33 sec | .


A 28-year-old woman, gravida 3, para 2, at 34 weeks' gestation comes to the physician because of a 1-day history of dyspnea, dry cough, and chest pain.

Her pulse is 112/min, respirations are 24/min, and blood pressure is 108/78 mm Hg.

Pulse oximetry on room air shows an oxygen saturation of 90%.

Examination shows jugular venous distention and bilateral pitting edema below the knees that is worse on the right side.

There is dullness to percussion over the right lung base.


Which of the following is the most likely cause of this patient's symptoms?

A 55-year-old man is brought to the emergency department because of cough and poor appetite for the past week.

Since it began, he has been coughing up small amounts of malodorous phlegm.

During the past two nights, he has also had night sweats.

He was diagnosed with HIV infection 5 years ago.

He has hypertension, type 2 diabetes mellitus, and severe heartburn.

The patient is homeless and does not take any medication.

He has smoked a pack of cigarettes daily for 30 years.

His temperature is 38.9°C (102.0°F), pulse is 101/min, respirations are 25/min and blood pressure is 145/92 mm Hg.

Physical examination shows crackles and dullness to percussion at the right lung base.

Scattered expiratory wheezing is heard throughout both lung fields.

A grade 2/6 mid-systolic ejection murmur is heard along the upper right sternal border.

His CD4+T-lymphocyte count is 280/mm3 (Normal ≥ 500).

An x-ray of the chest shows a hazy infiltrate in the right lower lung field.


A 45-year-old man who underwent liver transplantation 3 months ago for chronic liver failure presents to the physician because of a backache following a fall from sitting.

He is currently on immunosuppressive therapy with glucocorticoids and cyclosporine.

On physical examination, his vitals are within normal limits.

He has tenderness over his lumbar spine.

An X-ray of the lumbar spine shows a wedge compression fracture of the L1 vertebra.

His serum testosterone and serum creatinine levels are normal.

Bone mineral densitometry shows a T-score of –3.0.


A 14-year-old girl is brought to the physician by her mother for evaluation of several bruises on her lower extremities.

She has had these bruises for about 6 weeks, and the mother is concerned that she might be bullied at school.

The patient has had increasing fatigue and paleness over the past several days.

She has a history of recurrent generalized tonic-clonic seizures treated with carbamazepine.

Her temperature is 37.8°C (100.1°F), pulse is 115/min, and blood pressure is 100/60 mm Hg.

Examination shows a soft, nontender abdomen with no organomegaly.

There are several subcutaneous purple spots on her legs bilaterally.

Her hemoglobin concentration is 8.4 g/dL, leukocyte count is 2,600/mm3, platelet count is 18,000/mm3, and reticulocyte count is 0.3%.

Serum electrolyte concentrations are within normal limits.


Which is the most likely diagnosis of this condition?

A 33-year-old man presents to his primary care practitioner, complaining about the presence of white spots in both of his hands.

He states that the white spots have expanded in the last few months; they are not tender nor ulcerated.

His past medical history is relevant for hypothyroidism.

Upon physical examination, the patient shows hypopigmented macules on both hands and on the back and shoulders adjacent to a patch of skin, with signs of excoriation and scratching.

Under the Wood’s lamp, the skin lesions on the hands, back, and shoulders show fluorescence.

There are no signs of inflammation in any of the skin lesions.

The vital signs of the patient are within normal limits.


Which of the following congenital heart diseases does this infant most likely present with?

A 6-month-old infant is brought to a pediatrician for his scheduled immunizations.

The parents deny any specific current complaints, but his facial features differ from those of other children in the family.

During the physical examination, the pediatrician notes that the infant’s vital signs are stable.

His facial features include a medial epicanthic fold, a face that appears flat, and a flat occiput with low-set ears.

The pediatrician also notes a single transverse palmar crease on both hands.

An echocardiogram is performed which suggests that the infant has a congenital heart disease which is the most common form of congenital heart disease seen in children with this particular genetic disorder.


A 72-year-old man with a history of chronic kidney disease presents to his primary care physician complaining of recurrent chest pain with activity.

The patient used to have chest pain when he mowed his lawn.

Now he gets chest pain whenever he walks short distances such as to get his mail.

The pain resolves on its own when the patient sits and rests.

His temperature is 98.2°F (36.8°C), blood pressure is 157/98 mm Hg, pulse is 80/min, respirations are 15/min, and oxygen saturation is 98% on room air.

Physical exam is notable for an obese man who is in no distress.

An initial ECG is unchanged from a previous ECG.

The patient's first troponin is 0.06 ng/mL which is unchanged from previous troponins.


A 73-year-old male is brought to his family practitioner by his daughter with the complaints of a spinning sensation for the past 4 weeks.

He says that the room appears to be continuously spinning.

This has progressively worsened over the last 4 weeks to the point that he has become bed bound and cannot walk without support.

These spinning sensations are present throughout the day and do not change with position.

They are associated with nausea and vomiting.

He denies ear pain, ear discharge, ringing in the ear, hearing disturbances, ear fullness, head trauma, fever, or recent flu-like illness.

He has a blood pressure of 133/80 mm Hg, heart rate of 80/min, respiratory rate of 12/min, and temperature of 36.7°C (98.2°F).

His extraocular eye movements are normal in all directions, but a vertical nystagmus is present that does not disappear despite repetitive testing.


What is the most likely cause of the following findings?

A 45-year-old man presents to the outpatient unit with a complaint of lower extremity edema for the past few weeks.

He has also been observing puffiness of the face lately.

The serum creatinine is 3.5 mg/dL, and antinuclear antibodies are absent.

The biopsy findings are given in the picture.


A 49-year-old G4P4 woman comes to the clinic complaining of repeated leakage of fluid from her vagina for the past 5 months.

She noticed an increase in episodes following her cold last week when she was coughing and sneezing a lot.

Her past medical history is significant for Crohn disease, which is well controlled with sulfasalazine.

Her last menstrual period was 1 year ago.

She is currently sexually active with multiple partners with inconsistent condom use.

She denies any vaginal itching, abnormal discharge, pain, subpubic pressure, urinary urges, or odors.

Physical examination is significant for a bulge at the anterior vaginal wall.


What is the most likely etiology of this patient’s diagnosis?

A 14-year-old boy is brought to the office by his parents because he states that for the past 2 months he has been feeling constantly tired, and also noticed a dull pain in the pit of his stomach.

The patient has no relevant family history.

The vital signs include a heart rate of 105/min, a respiratory rate of 16/min, a temperature of 37.0°C (98.6°F), and a blood pressure of 111/66 mm Hg.

On physical exam, the abdomen is distended with hepatomegaly 5 cm underneath the xiphoid process.

The complete blood count results are as follows: | Hemoglobin 17.6 g/dL | Hematocrit 64% | RBC 6.02 x 1012/L | Leukocyte count | 26,300/mm3 | Neutrophils 55% | Bands 2% | Eosinophils 1% | Basophils 0% | Lymphocytes 29% | Monocytes 2% | Platelet count 480,000/mm³ | Erythropoietin < 1.0 mU/mL | The coagulation test results are as follows: | Partial thromboplastin time (activated) 30.9 s | Prothrombin time 14.0 s | The abdominal Doppler ultrasound imaging is shown in the picture.


A 25-year-old man comes to the emergency department because of left flank pain for 2 hours.

The pain is colicky in nature and he describes it as 8 out of 10 in intensity.

He had a similar episode 6 months ago for which he took naproxen.

There is no personal or family history of serious illness.

He is a second-year medical student and has been consuming more coffee and energy drinks than normal to stay awake and study for the past 2 days.

His temperature is 37.3°C (99.1°F), pulse is 98/min, and blood pressure is 124/78 mm Hg.

Examination of the back shows no costovertebral angle tenderness.

The remainder of the examination shows no abnormalities.


A 16-month-old boy is brought to the pediatrician after his parents noticed the appearance of a rash on his face, torso, and limbs.

The boy has been ill for almost 2 weeks, initiating with fever, malaise, coryza, headache, nausea, diarrhea, and a rash on both of his cheeks.

Physical examination is unremarkable except for an erythematous maculopapular rash on the face, trunk, and extremities with a reticular pattern (as shown in the photograph).


Which of the following congenital defects is the most likely cause of these findings?

A 4-year-old girl presents to the pediatrician’s office for a physical examination prior to starting preschool.

She was born at 39 weeks gestation via spontaneous vaginal delivery.

She is up to date on all vaccines and is meeting all developmental milestones.

Past medical history is noncontributory.

She takes no medications but does take a chewable vitamin daily.

She lives with her parents and older brother in a house.

Today, her blood pressure is 110/65 mm Hg, heart rate is 90/min, respiratory rate is 22/min, and temperature of 37.0°C (98.6°F).

On physical exam, she appears well developed and pleasant.

She sits listening to the conversation and follows directions.

Palpation of the heart reveals a mild parasternal heave.

Auscultation reveals a normal S1 but the S2 is split and remains split during inhalation and exhalation.

Additionally, there is a medium pitched midsystolic murmur that is loudest between ribs 2 and 3 on the left side and a very soft diastolic rumble.


What is the most likely cause of this patient’s visual loss?

A 67-year-old woman presents to the ophthalmologist with complaints of worsening visual loss.

Driving has become difficult, particularly at night, as she experiences substantial glare and sees halos around lights.

On physical examination, there is absence of a red reflex.


A healthy patient in their teens presented to the emergency department with high-grade fever (38.7 °C), neck pain, and a foreign body sensation in the throat after eating chicken 3 days prior

Laboratory investigations demonstrated an elevated leukocyte count (22 500/µL

to convert to ×109/L, multiply by 0.001)

Lateral soft tissue radiography showed a radio-opaque foreign body at the level of C6. Computed tomography (CT) scan confirmed a 2-cm radio-opaque osseous foreign body at the level of the cricopharyngeus muscle with signs of esophageal perforation, including air leak tracking toward the left lobe of the thyroid gland (Figure, A and B)

The patient was brought to the operating room, and the esophageal foreign body was successfully removed

Postoperatively, the patient continued to become febrile despite intravenous antibiotic therapy

A repeated CT scan of the neck and chest was done (Figure, C)

A, Axial cut of computed tomography (CT) scan of the neck demonstrating free air in the left lobe of the thyroid gland (arrowheads)

B, Sagittal cut of neck CT scan showing 2-cm radiodense osseous foreign body in the upper cervical esophagus at C6 level (arrowhead)

C, Coronal cut of CT scan showing gas in the thyroid gland (arrowheads)


A man in his 30s with AIDS presented with acute-onset painful scattered umbilicated papulopustules and ovoid ulcerated plaques with elevated, pink borders on the face, trunk, and extremities (Figure, A)

The patient also had a new-onset cough but was afebrile and denied other systemic symptoms

Due to his significant immunocompromise, the clinical presentation was highly suspicious for infection

For rapid bedside differentiation of multiple infectious etiologies, a Tzanck smear was performed by scraping the base of an ulcerated lesion and inner aspect of a pseudopustule and scraping its base with a #15 blade

These contents were placed on a glass slide, fixed, and stained with Wright-Giemsa and subsequently Papanicolaou staining to further characterize the changes seen

A, Clinical image demonstrating papulopustules and ovoid ulcerated plaques with elevated, pink borders on the elbows

B, Tzanck smear using Wright-Giemsa staining of specimen demonstrating ballooning of keratinocytes and peripheralization of nuclear material (original magnification ×20)


Perineural spread of cutaneous malignancy

A 68-year-old man presented with progressive right-sided facial numbness and weakness, vision changes, and headaches

His symptoms began 5 years earlier with right temple numbness, which expanded to include the entire right face and mouth

He then developed progressive right facial weakness with a droop, slurred speech, and eyelid ptosis

Finally, binocular vertical and horizontal diplopia and periorbital headaches began

Trials of gabapentin and steroids provided no relief

His history was significant for a left cheek melanoma completely excised years prior, numerous cutaneous squamous and basal cell carcinomas of the bilateral head and neck removed without issue, type 2 diabetes, and hyperlipidemia

His medications included metformin and simvastatin

he had no history of smoking or drinking

On examination, he had right-sided ptosis and severe right facial weakness in a lower motor neuron pattern with temporal wasting

The left pupil was appropriately sized and reactive

His right-sided vision was impaired (20/250) with blurriness

Vertical and horizontal binocular diplopia was present

Right extraocular movements were significantly limited in all directions and painful, and right-sided facial sensation was diminished to pinprick in all cranial nerve (CN) V distributions

Left-sided extraocular movements were intact

His left facial sensation was intact to pinprick, and his strength was without deficit

There were no other neurological abnormalities

Initial magnetic resonance images (MRIs) were unremarkable

Tests for infection and autoimmune disease had negative results

Cerebrospinal fluid (CSF) studies showed mildly elevated protein values

T1 turbo spin-echo fat-saturated magnetic resonance images of the brain through the skull base at presentation


T-cell acute lymphoblastic lymphoma/leukemia

Myeloid/lymphoid neoplasms with eosinophilia and tyrosine kinase gene fusions

A 31-year-old man presented with left cervical and left inguinal masses

He reported intermittent itching and night sweats for 2 years

He denied fever, weight loss, shortness of breath, rashes, diarrhea, and neurological symptoms

On a preemployment evaluation, the patient was told he had a high white blood cell count 2 years ago

On examination, there was left cervical and inguinal lymphadenopathy and no other organomegaly

Complete blood cell count and peripheral blood smear showed marked leukocytosis, with a white blood cell count of 22 340/μL, an absolute neutrophil count of 5360/μL, and 55% eosinophils with an absolute eosinophil count of 12 290/μL (to convert all to cells ×109/L, multiply by 0.001)

Vitamin B12 was markedly elevated at more than 4000 pg/mL (to convert to pmol/L, multiply by 0.7378)

The erythrocyte sedimentation rate was 5 mm/h

Lactate dehydrogenase was 180 U/L, and alkaline phosphatase was 81 U/L (to convert both to μkat/L, multiply by 0.0167)

Evaluations for HIV and hepatitis B and C were all negative

Serum creatinine was 0.76 mg/dL (to convert to μmol/L, multiply by 88.4)

alanine aminotransferase and aspartate aminotransferase were 11 U/L and 9.9 U/L, respectively (to convert to μkat/L, multiply by 0.0167)

and total bilirubin was 0.35 mg/dL (to convert to μmol/L, multiply by 17.104)

Bone marrow biopsy showed hypercellular marrow (cellularity of 100%), myeloid hyperplasia, increased eosinophils with some dysplasia, and a blast count of 2%

Positron emission tomographic–computed tomographic scan showed a left upper cervical lymph node of 2.6 cm and a left inguinal lymph node of 3.1 × 2.3 cm with an standardized uptake value max of 5.7 (Figure, A)

Left inguinal lymph node biopsy showed partial involvement by atypical cells with high proliferation index (Ki-67 >95%) that were positive for CD3, CD4, CD8, BCL2, and TDT, suggestive of T-cell lymphoblastic lymphoma/leukemia (Figure, B)

A, Positron emission tomographic–computed tomographic (PET/CT) scan of the head and neck at presentation showing a left upper cervical lymph node of 2.6 cm (arrowhead)

B, Lymph node biopsy immunohistochemical stain with terminal deoxynucleotidyl transferase

The inset shows interphase fluorescence in situ hybridization for FIP1L1::PDGFRA rearrangement (positive)

C, PET/CT 12 weeks after treatment initiation


A 28-year-old woman presented with a 5-day history of painful cervical lymphadenopathy along with fever, nausea, and vomiting

Treatment with antibiotics and corticosteroids was started, without improvement of symptoms

She denied recent travel, sick contacts, or autoimmune disease

Her medical history was significant for α-thalassemia but otherwise noncontributory

On examination, she had tender lymphadenopathy involving right level V

A computed tomography scan of the neck revealed a collection of enlarged, matted nodes without evidence of an abscess

Fine-needle aspiration (FNA) biopsy showed nondiagnostic reactive lymph nodes

She was subsequently admitted to the hospital, where the lymphadenopathy progressed to the left side of the neck and a skin eruption developed over her face, back, and trunk

Results of an infectious disease and rheumatological workup, including bone marrow biopsy, were negative

Given her persistent symptoms, the ear, nose, and throat service consulted for excisional biopsy

On pathologic examination, the lymph nodes showed extensive coagulative necrosis, apoptosis, and karyorrhectic debris (Figure 1)

Cellular components included histiocytes and dendritic cells, with absent neutrophils and hematoxylin bodies

Well-defined areas of fibrinoid necrosis in paracortical areas (hematoxylin-eosin, original magnification ×20)


A 27-year-old woman presented to her primary care physician with primary complaints of headaches and visual-spatial difficulties when driving at night

In addition, the patient described a progressively enlarging lesion on her left scalp, which she had initially noted in high school

She denied any overlying loss of hair, sharp pain, or bleeding from the scalp lesion, and she only reported pain with prolonged pressure

On her scalp examination, a 7-cm ill-defined spongy and pulsatile mass was noted with an overlying gray patch of hair

Magnetic resonance imaging (MRI) of the brain/orbit was initially performed (Figure, A and B), which demonstrated an ill-defined, diffuse, intensely enhancing scalp lesion in the left frontoparietal region with very prominent vascularity and underlying calvarial thickening

No osseous destruction or intracranial extension was present

Subsequent catheter angiography (Figure, C) confirmed the hypervascular mass, supplied via hypertrophied superficial temporal and occipital arteries with marked internal vascularity without direct involvement of the calvarium or intracranial structures

The patient underwent biopsy under general anesthesia

the specimen showed an ill-defined deep dermal/subcutaneous proliferation of spindle cells without significant nuclear pleomorphism or mitotic activity

The lesions stained positive for S100 and SOX10 immunostains

Coronal T2-weighted (A) and postcontrast T1-weighted (B) fat-saturated images show the large left T2 hyperintense scalp lesion (double arrowheads

A and B), with underlying calvarial thickening (asterisk

A and B) without invasion, prominent vascularity (single arrowhead

Note the preservation of the overlying skin

Catheter angiography (C) shows the hypervascular scalp lesion being supplied by hypertrophied branches arising from the left superficial temporal and occipital arteries, with marked internal vascularity (arrowheads)


A woman in her 30s presented for evaluation of asymptomatic erythematous scaly plaques over the face and proximal extremities

The lesions started as an erythematous papule on the face, which had progressed to larger plaques within 10 years

There was involvement of the upper respiratory tract, causing palatal perforation and stridor, necessitating a tracheostomy a year ago

In addition, there was history of wheezing and breathlessness

There was no history of preceding trauma, fever, malaise, joint pain, cough, epistaxis, hemoptysis, or hematuria

Examination revealed multiple well-defined brown erythematous scaly plaques with central scarring measuring 2 × 1 to 8 × 7 cm on the face, arm, and thighs

The patient also had saddle nose deformity, perforation of the hard palate, and necrotic plaques on the ear, causing destruction of ear cartilage (Figure, A and B)

There was cervical lymphadenopathy: multiple, nontender, discrete nodes, ranging from 1.5 to 2 cm without any surface changes

The results of sensory examination and peripheral nerve examination were within normal limits

Respiratory examination and chest radiography results were normal

Hemogram showed anemia (hemoglobin, 10.1 g/dL [to convert to g/L, multiply by 10.0])

the results of a kidney function test, a liver function test, a fasting blood glucose test, and routine urinalysis were within normal limits

Serologic analysis results for HIV-1/2, antinuclear antibody, and antineutrophil cytoplasmic antibodies were negative

Punch skin biopsy was obtained for histopathologic analysis and culture (Figure, C)

A, Multiple well-defined brown erythematous scaly plaques with central scarring, saddle nose deformity, and necrotic lesions on the ear with loss of ear cartilage

C, Infiltrate of foamy histiocytes and pigmented yeasts without multiaxial septation (Fontana-Masson)


Acute coalescent mastoiditis with sigmoid sinus thrombosis

A 64-year-old man with a 2-year history of seizure disorder presented to the emergency department (ED) at an outside hospital with breakthrough seizures

One month prior to his ED visit, he had been treated with oral antibiotics when he presented to an urgent care center with left-sided otorrhea, otalgia, and hearing loss

In the ED, he denied headaches, fever, otalgia, otorrhea, or hearing loss

Magnetic resonance imaging of the brain with contrast showed diffuse enhancement and possible coalescence in the left mastoid process with adjacent dural enhancement and sigmoid sinus occlusion

He received intravenous vancomycin and cefepime and was started on a heparin drip before being transferred to our facility

On presentation at our facility, the patient appeared well with normal vital signs

There was no periauricular swelling or erythema

Otoscopic examination showed no inflammation of the external auditory canals, and the tympanic membranes were translucent without evidence of retraction or effusion

Computed tomographic venogram confirmed occlusion of the left transverse and sigmoid sinuses, and computed tomography of the temporal bone showed coalescence of mastoid air cells with erosion into the sigmoid sinus

Mastoidectomy was performed for biopsy and culture specimens

Histologic findings from the specimens demonstrated cells with oval nuclei and abundant cytoplasm arranged in syncytial clusters with occasional whorls, pseudoinclusions, and psammomatous calcifications (Figure, A)

Immunohistochemical stains were positive for epithelial membrane antigen (EMA) and vimentin (Figure, B)

Hematoxylin-eosin stain (A) and immunohistochemical stain (B) after mastoidectomy for biopsy and culture specimens


A 62-year-old healthy man with no recent travel history or sick contacts presented with 2 weeks of midline sore throat without systemic signs, such as fever, myalgia, or rash

A physical examination, including flexible laryngoscopy, revealed cobbling, adherent, thick yellow mucus and ulceration of the uvula (Figure 1A)

A, Nasopharyngoscopic imaging revealed infected nasal surface of uvula

B, Physical examination showed recurrent skin lesions at incision site and in the surrounding skin

Empirical treatment with augmentin and nystatin oral suspension for possible bacterial or fungal pharyngitis was unsuccessful, with symptoms worsening during the subsequent 2 weeks

Results of laboratory testing for group A Streptococcus, COVID-19, and mononucleosis were negative

A tissue biopsy was performed, and results of pathology revealed epithelial hyperplasia with suppurative granulomatous inflammation without evidence of dysplasia or malignancy

There was no evidence of fungal or acid-fast organisms

Tissue culture revealed only normal respiratory flora

Additional treatment with fluconazole, augmentin, doxycycline, omeprazole, prednisone, and bactrim failed to improve symptoms

Further exploration of the patient’s history revealed that 5 years earlier, he had traveled to Guyana in South America

After that trip, he sought medical care for multiple neck subcutaneous nodules as well as for a 4-cm focus of neck cellulitis

After failed treatment with empirical antibiotics, the largest lesion was excised

Findings of pathologic testing revealed dense granulomatous inflammation

However, results of stains for bacteria, fungus, and acid-fast bacillus were negative (Figure 1B)

The remaining cutaneous and subcutaneous nodules had resolved spontaneously


A 53-year-old woman with a history of stage IVA1 (T4N1M0B2) mycosis fungoides presented with a new 1-month history of hyperpigmentation of the oral mucosa (Figure 1)

Examination of the mouth revealed multiple coalescing painless nonpruritic black macules and patches on the tongue, roof of the mouth, and buccal mucosa

Examination of the skin was notable for erythematous and hyperpigmented patches covering 90% of the body surface area, consistent with her known mycosis fungoides

Other notable findings on examination were 1- to 2-cm lymphadenopathy in the bilateral inguinal folds and axillae

Review of systems was notable for fatigue

The patient had previously received 5 cycles of romidepsin with progression of disease, followed by 4 doses of pegylated liposomal doxorubicin hydrochloride, which was followed by partial response

Black macules and patches on the tongue, roof of the mouth, and buccal mucosa


A woman in her 60s with long-standing thickening and induration of the legs presented with 1 week of left leg pain

The patient had previously been diagnosed with elephantiasis nostras verrucosa, and she was advised to elevate and compress the leg but was unavailable for follow-up

Her medical history was notable for morbid obesity, hypertension, diabetes, and stroke

She reported no fever, fatigue, weight changes, gastrointestinal symptoms, difficulty concentrating, anxiety, or hyperhidrosis

Physical examination results revealed multiple firm skin-colored papules and nodules coalescing to form a large plaque on the anterior aspect of the left lower leg, whereas the right lower leg had diffuse induration and hyperpigmentation (Figure 1)

Laboratory analysis revealed normal complete blood cell count, thyroid function, serum protein electrophoresis, and serum immunofixation

A biopsy of a left leg nodule was performed

Multiple firm skin-colored papules and nodules coalesce to form a large plaque on the anterior aspect of the left lower leg


Familial cerebral cavernous malformations

A female patient in her early 50s presented with multiple (approximately 10) bluish, partially keratotic papules and nodules on the lower legs (Figure, A)

Since the age of 24 years, the lesions had been growing very slowly in number and size and had been bleeding occasionally after a trauma

The patient used a wheelchair due to a right-sided hemiparesis and hemianopsia caused by an intracranial hemorrhage

At the age of 4 years, recurrent seizures had led to the diagnosis of cerebral venous malformations

Clinical examination also demonstrated a lipolymphedema of the lower legs that had been attributed to a functional venous insufficiency with insufficient venous pump activity

The patient had been wearing compression stockings for years

The family history was positive for cerebral venous malformations involving the patient’s father and aunt, the father having died of an intracranial hemorrhage in his early 70s

Sonography of the skin lesions demonstrated intradermal slow-flow vascular malformations with a maximum diameter of 1.2 cm

A biopsy of a lower-extremity papule was performed (Figure, B and C)

Mutational analysis resulted in the detection of a heterozygous pathogenic KRIT1 variant


Pretibial pruritic papular dermatitis (PPPD)

A woman in her 30s presented with progressive, multiple itchy erythematous to violaceous papules and plaques localized to the left shin for more than 10 years (Figure, A and B)

On close inspection, milia and a few erosions with partially detached epidermis were identified

There were no abnormalities of the mucous membranes, nails, hair, or teeth

No extracutaneous involvement was observed

A, Multiple pruritic erythematous to violaceous lichenified papules and plaques on the left shin

B, Close-up of the skin lesions showing an erosion with partially detached epidermis and multiple milia

C, Histopathologic examination revealed dermoepidermal separation (hematoxylin-eosin stain)

D, Histopathologic examination revealed several milia (hematoxylin-eosin stain)


An otherwise healthy 13-month-old male was scheduled to undergo excisional biopsy of a left anterior tongue submucosal intramuscular mass (Figure 1)

This firm-to-palpation mass was noted at birth and remained proportional in size with the child’s somatic growth, measuring approximately 1 cm in diameter on presentation

Preoperative magnetic resonance imaging (MRI) documented the mass to be uniformly solid and well circumscribed without cystic components or flow voids, hypointense on T2, and hyperintense with homogeneous contrast enhancement on T1. His head, neck, and general examination findings were otherwise normal with no cervical lymphadenopathy or additional masses


A 56-year-old woman with a history of multiple myeloma complicated by therapy-related acute myeloid leukemia was admitted for myeloablative conditioning in preparation for allogeneic hematopoietic cell transplant

Her course was complicated by severe mucositis, acute kidney injury, and neutropenic fever

During her admission, the patient also developed painful necrotic skin lesions, a nonproductive cough, and altered mental status

Physical examination findings demonstrated dusky, purpuric, and pink papulonodules, some with overlying eschar, scattered on the scalp, neck, trunk, and extremities (Figure, A)

Laboratory study results revealed a white blood cell count of 150/μL (reference range, 3500/μL to 10 500/μL), an absolute neutrophil count of 130/μL (reference range, 1500/μL to 7400/μL), and a platelet count of 19 ×103/μL (reference range, 150 ×103/μL to 400 ×103/μL)

(To convert the white blood cell and neutrophil counts to cells ×109/L, multiply by 0.001

conversion of the platelet count to cells ×109/L is 1:1.) Blood culture results were negative

The results of a computed tomography (CT) scan of the brain showed no acute intracranial hemorrhage, territorial infarction, or mass effect

The results of a CT scan of the chest demonstrated multifocal bilateral ground-glass patchy opacities, scattered solid bilateral pulmonary nodules, and a moderately sized pericardial effusion

A biopsy specimen from a skin nodule on the left lateral thigh was obtained for histopathologic examination and tissue culture, and wet mount preparation of a tissue culture colony was performed (Figure, B)

A, Physical examination findings demonstrate dusky and pink papulonodules, some with overlying eschar, scattered on the scalp, neck, and extremities

B, Lactophenol cotton blue staining of a tissue culture colony reveals septated hyphae and oval microconidia


A man in his 80s presented to the dermatology clinic with a 3-month history of a violaceous plaque with blackish papules and nodules on his left cheek, neck, and chest

He reported that the lesion had been asymptomatic but was increasing in size, with progression from his face to anterior chest

He received a diagnosis of primary salivary duct carcinoma of the hard palate 1 year prior and had been receiving treatment with oral bicalutamide and radiotherapy

A partially regressive change of the palatal tumor was noted radiographically during regular follow-up 3 months previously

On clinical examination, a hemorrhagic and erythematous plaque with multiple purpuric-to-blackish infiltrative papules and nodules extending from cheek, lateral neck, to the interclavicular area was found (Figure 1)

An incisional biopsy from the infiltrative nodule on cheek was performed and submitted for histopathologic analysis

Hemorrhagic and erythematous plaque with multiple infiltrative purpuric-to-blackish papules and nodules with varying sizes extending from left cheek, neck, to chest


A previously healthy 16-year-old girl presented to our department with a red tumor on her right upper arm that enlarged over 6 months

The lesion initially started as a small cutaneous nodule without obvious triggers that gradually developed and enlarged to form a red-colored tumor with a hard nodule inside

There was no history of local trauma or insect bite

Her personal, past, and family histories were unremarkable

On physical examination, there was a 6 × 6-cm protuberant, thick-walled, and well-defined red bullalike tumor on the right upper arm, which extended 1 to 3 cm from the epidermal surface (Figure, A)

Inside the tumor, a nontender, firm-to-hard nodule was palpated

There was no regional lymphadenopathy, and results of the rest of the physical and systemic examinations were normal

Laboratory examinations of hematologic, biochemical, and urinalysis tests were normal

The tumor was excised completely under local anesthesia, and part of the tissue was sent for pathological examination

A, A tumor on the patient’s right shoulder

B, The excisional tumor showed multilocular spaces below the epidermis and a solid tumor present in the deep dermis with a chalky-white cut surface and lobulated appearance

C and D, Histological examination revealed basophilic cells and shadow cells as well as dilated lymphatic vessels (hematoxylin-eosin


A 15-year-old male presented to the pediatric otolaryngology clinic with dysphagia to solid foods, and dyspnea when lying on the right side that was progressive during 8 months

He denied pain, aspiration, noisy breathing, obstructive episodes, dysphonia, and weight loss

A physical examination revealed fullness in the left oropharynx and left level 3 of the neck

Findings of flexible laryngoscopy showed a submucosal mass in the left oropharynx extending inferiorly into the hypopharynx, obliterating the left pyriform sinus, abutting the base of tongue with hooding over the epiglottis

The supraglottis and glottis were unremarkable

Computed tomography imaging with contrast of the neck revealed a 4.6 × 4.0 × 8.0-cm heterogeneously enhancing mass in the left oropharynx and hypopharynx with cystic components and clear planes separating the mass from the spinal musculature and carotid sheath (Figure 1)

Computed tomography imaging with contrast of the neck with the coronal cut demonstrating the well-circumscribed heterogeneous mass involving the retropharyngeal space, abutting the great vessels, and extending from superiorly in the oropharynx to inferiorly in the hypopharynx


A 46-year-old man presented with a left shoulder mass

It began with a quarter-size maculopapular lesion and continued to progress during the next 3 years

He reported limited shoulder movements and denied other symptoms

There was no medical or surgical history, and he had a negative personal or familial oncologic history

The patient was a construction worker and used tobacco daily

On physical examination, there was a 24 × 15-cm fungating, necrotic, and ulcerated left shoulder mass (Figure 1A)

A punch biopsy showed sheets of highly pleomorphic atypical spindle and epithelial cells within the superficial and deep dermis, extending into the subcutaneous tissue, and with 18 mitoses/mm2. Immunohistochemistry demonstrated SOX10-positive and pankeratin-negative tumor cells

A whole-body positron emission tomographic and computed tomographic scan revealed an intensely hypermetabolic left shoulder mass and multiple hypermetabolic enlarged left axillary lymph nodes, with no distant metastases (Figure 1B)

Brain magnetic resonance imaging was negative for intracranial metastasis

A, Left shoulder giant fungating mass in a 46-year-old man

B, Positron emission tomographic and computed tomographic (PET-CT) scan shows the hypermetabolic left shoulder mass and no distant metastases


A man in his 60s presented to our clinic with a 5-year history of diffuse erythematous, edematous annular plaques (Figure 1)

The result of a skin biopsy performed 4 years prior was interpreted as subacute cutaneous lupus erythematosus (SCLE)

Treatments included hydroxychloroquine, 400 mg/d, for 4 years

mycophenolate mofetil, 1000 mg/d, for 6 months

and oral prednisone tapers, starting at 40 mg/d

Of these, only prednisone appeared to elicit a response

Review of symptoms was negative for fatigue, weight loss, cough, shortness of breath, joint pain, ocular symptoms, mucosal ulcerations, history of blood clots, photosensitivity, pleurisy, urine changes, and diarrhea

Medical history included hypertension, for which he took losartan, and seasonal allergies, for which he used fexofenadine and fluticasone

Laboratory evaluation revealed mild neutropenia and lymphopenia with normal hemoglobin and platelet counts

Antinuclear antibody screening results were negative

The results of a comprehensive metabolic panel, C3, C4, serum protein electrophoresis, serum free light chains, methylmalonic acid, homocysteine, ferritin, copper, zinc, haptoglobin, HIV, hepatitis B and C serologic tests, and rapid plasma reagin were negative

Lactate dehydrogenase was mildly elevated

Erythrocyte sedimentation rate was elevated at 41 mm/h

Therapeutic trials of dapsone and dapsone in combination with colchicine were not effective


Pseudoxanthoma elasticum–like disorder with coagulation deficiency

Pseudoxanthoma elasticum/pseudoxanthoma elasticum–like overlap syndrome

A Chinese woman in her late 20s presented with a 20-year history of progressive skin laxity

In early childhood, she had developed asymptomatic yellowish coalesced papules confined to flexural areas

Subsequently, severe skin sagging occurred on her abdomen with loss of elasticity, then spread extensively with developmental growth

The results of a physical examination showed thick and leathery skinfolds on the neck, axillae, inguinal regions, abdomen, and limbs (Figure, A), with yellowish papules scattered on the dorsal neck

The patient had no extracutaneous involvement and was born to healthy nonconsanguineous parents

However, her younger sister had developed the same skin symptoms

Coagulation-related tests indicated a low clotting activity of factor X (52.2%

reference range, 77%-131%) and a prolonged prothrombin time (12.8 seconds

The results of other laboratory investigations were unremarkable

Echocardiographic and fundoscopic examination results were normal

A skin biopsy specimen was obtained from her neck for histopathologic examination (Figure, B)

A, Clinical image of the neck, axillae, and abdomen

Severe skin laxity with deep cutaneous folds on the cervical, axillary, and abdominal regions

B, Skin biopsy specimen taken from the neck shows excessive calcification of elastic fiber fragments scattered in the reticular dermis (von Kossa)


A 71-year-old woman presented to the otolaryngology clinic for evaluation of a parapharyngeal space (PPS) mass that was incidentally found on magnetic resonance imaging (MRI) of the face

The patient experienced left jaw tightness, aural fullness, and facial discomfort

The MRI results demonstrated a well-circumscribed, ovoid mass in the left prestyloid PPS that measured 12 × 15 × 22 mm

The lesion was isointense on T1-weighted imaging, was hyperintense on T2-weighted imaging, and demonstrated heterogeneous enhancement following gadolinium administration (Figure 1)

A computed tomography–guided fine-needle aspiration was performed, which demonstrated blood, fibrin, and rare mesenchymal cells

Given her ongoing symptoms, the patient requested surgical resection

The patient underwent transoral robotic surgery for resection of her PPS mass to achieve definitive diagnosis and treatment

Histopathologic evaluation demonstrated a well-circumscribed, 12-mm mass

A, Coronal section from a T2-weighted image demonstrates a hyperintense mass in the left parapharyngeal space (PPS)

B, Axial section from a postcontrast T1-weighted image shows a heterogeneously enhancing mass in the prestyloid PPS


Maxillary carcinoma with perineural spread

Invasive fungal sinusitis (mucormycosis) with perineural spread

A 47-year-old woman was seen with left-sided facial weakness and swelling for 2 months

She was diagnosed as having recurrent high-grade serous cystadenocarcinoma of the ovary and developed these symptoms after cycle 4 of chemotherapy

She was receiving oral antidiabetic agents, and her blood glucose level was 110 mg/dL (to convert to millimoles per liter, multiply by 0.0555)

Physical examination findings were consistent with left lower-motor-neuron type of facial nerve palsy

She was conscious and well oriented, with no other relevant clinical signs or focal neurological deficits

There was no history of prior COVID-19 infection

Magnetic resonance imaging (MRI) of the brain was requested, with clinical suspicion of metastasis in view of mildly elevated CA125 levels

The results of MRI of the brain did not reveal any neuroparenchymal or meningeal lesions

However, both maxillary sinuses showed mucosal thickening, which was intermediate to hypointense on T2-weighted imaging and hypointense on T1-weighted imaging with postcontrast enhancement

Computed tomography sections confirmed bony destruction with foci of remodeling involving the walls of the maxillary sinuses (Figure 1)

A, Axial T2-weighted magnetic resonance imaging (MRI) shows T2 heterogeneous soft tissue in the maxillary sinus with perimaxillary inflammatory changes (yellow arrowhead)

B, In this axial contrast-enhanced, T1-weighted (T1 + C) image, linear enhancement corresponds to expected course of buccal branch of facial nerve (VII) (white arrow)

The enhancement extends medially and involves the mandibular branch of the trigeminal nerve (V3)

P indicates parotid gland, and the yellow dashed line outlines the edge of the parotid gland

B and C, The white arrow shows the intercommunication between the auriculotemporal branch of V3 and the intraparotid facial nerve (green marker)

C, Additionally, involvement of enhanced thickening is seen up to the left foramen ovale, without involvement of the trigeminal ganglion

D, In oblique sagittal images, the thick enhancing branches of the facial nerves within the left parotid gland are seen (orange arrows)

The enhancement of facial nerve extends up to the stylomastoid foramen (SMF) (blue arrow), and there is subtle enhancement along its mastoid segments (MSs)

Fat stranding and enhancing soft tissue was seen in the retroantral spaces bilaterally as well as the pterygopalatine fossae

Anteriorly, enhancing soft tissue was present in premaxillary space, from which contiguous linear enhancement was seen extending to left parotid gland—along the expected course of buccal branch of left facial nerve

The entire extracranial left facial nerve proximal to this showed diffuse enhancement and thickening

Associated stylomastoid foramen widening was present with subtle enhancement along its mastoid and tympanic segments

These features were compatible with perineural extension of disease (Figure 1A and B)

Additionally, there was involvement of the mandibular branch of the trigeminal nerve via intercommunication between the auriculotemporal branch of trigeminal nerve and the intraparotid facial nerve

Diffuse enhancement and thickening was seen up to the left foramen ovale, without involvement of the trigeminal ganglion (Figure 1C and D)


Median rhomboid glossitis (nodular variant)

A 46-year-old man presented for an evaluation of a lesion on the dorsal tongue

The patient was asymptomatic and unaware of the lesion prior to it being discovered by his dentist

He underwent incisional biopsy at another institution and presented for a second opinion on the diagnosis

Overall, he was in good health with no underlying systemic diseases

Results of the most recent serologic analysis and urinalysis were also reportedly within normal limits

The extraoral examination was unremarkable, with no skin lesions, asymmetry, redness, swelling, or lymphadenopathy observed

The intraoral examination revealed an erythematous denuded area of the midline posterior dorsal tongue, with a central nodular component (Figure 1A)

The nodule was firm to palpation and nontender

The remaining oral soft tissue was unremarkable

Because a biopsy specimen was already obtained, blank slides were requested from the aforementioned institution, and staining was performed in house

A, Clinical examination revealed a pink nodule in the midline dorsal tongue with surrounding atrophic papillae

B, Hematoxylin-eosin stain of the biopsy specimen showed acellular, amorphous, and eosinophilic material arranged in lobules

Histologic evaluation revealed a piece of soft tissue with normal overlying epithelium

In the connective tissue, acellular, amorphous, and eosinophilic material arranged in lobules was noted (Figure 1B)


Blastic plasmacytoid dendritic cell neoplasm

A man in his 80s presented to the emergency department with a 4-week history of progressive weakness and fatigue, with associated development of purple bruiselike lesions on his head

He denied any history of trauma, falls, or occlusive headgear use

His medical history was significant for atrial fibrillation, receiving anticoagulation medication, and heart failure

On physical examination, numerous nontender violaceous plaques were seen on the forehead and scalp of the patient (Figure, A)

On full skin examination, nonspecific skin-colored plaques were noted on the chest, and a 1.8 × 2.5-cm erythematous plaque was noted on the left lower back

There was no appreciable lymphadenopathy or hepatosplenomegaly

Peripheral blood test results revealed a hemoglobin level of 9.1 g/dL, a platelet count of 68 × 103/μL, a white blood cell count of 2800/μL, and an absolute neutrophil count of 1.0/μL

(To convert hemoglobin to g/L, multiply by 10.0

white blood cell and neutrophil counts to ×109/L, by 0.001

and lactate dehydrogenase to μkat/L, by 0.0167.) A 4-mm punch biopsy was performed on the erythematous plaque on the left lower back (Figure, B and C)

B, Punch biopsy showing a grenz zone and infiltrate extending into deeper dermis (hematoxylin-eosin)

C, Punch biopsy showing cellular infiltrate of basophilic blastoid cells (hematoxylin-eosin)

D, Punch biopsy showing cellular infiltrate with CD123 staining


Cutaneous involvement of multiple myeloma

A man in his late 60s was referred to the dermatologic clinic with a 10-day history of fever up to 38.5 °C and tender lesions on the neck and trunk

In the previous month, he had received the first cycle of a chemotherapeutic regimen of ixazomib, lenalidomide, and dexamethasone to treat a recurrence of IgG-κ multiple myeloma with a 10-year history

Physical examination showed annular erythematous plaques of up to 2 cm in diameter, with violaceous and crusted centers on the neck and trunk

On the right chest, an annular erythematous lesion with 2 zones of color—a central area of purpura and an outer erythematous and edematous ring—was present (Figure, A)

Laboratory investigations found pancytopenia: a white blood cell count of 2.3 × 103/μL (reference range, 3.5-9.8 × 103/μL), hemoglobin levels of 12.1 g/dL (reference range, 13.5-17.6 g/dL), platelet count of 29 × 103/μL (reference range, 130-400 × 103μL), and elevated serum C-reactive protein levels of 3.31 mg/dL (reference range, <0.30 mg/dL)

Granulocyte colony-stimulating factor analogs had not been prescribed to the patient

Skin biopsy from a lesion on the left chest was performed for histopathological and immunohistochemical analyses (Figure, B-D)

A, Clinical image of annular erythematous plaques on the chest

B, A histopathologic image revealing inflammatory infiltrate of predominantly mononuclear cells in the superficial and mid dermis (hematoxylin-eosin stain)

C, The predominantly infiltrated cells showing large, elongated, vesicular nuclei, single inconspicuous nucleoli, and scant, slightly eosinophilic cytoplasm (hematoxylin-eosin stain)

D, An immunohistochemical image showing myeloperoxidase-positive cells


Moyamoya disease with renovascular hypertension

A 6-year child presented with headache for 1 month and seizures followed by altered consciousness for 1 day

He had new-onset daily occipital headache for 1 month of mild to moderate severity and pulsating character

Occasionally, the headache became severe accompanied by vomiting, photophobia, and phonophobia

The child had 4 to 5 episodes of generalized tonic-clonic seizures 1 day prior to admission

He remained unconscious for several hours and spontaneously regained consciousness over 24 hours

There was no history of fever, weight loss, limb weakness, vision impairment, or any strokelike illness

His birth and developmental history were unremarkable

On examination, his pulse rate was 80 beats per minute (regular) with all peripheral pulses palpable without any radio-femoral delay

His blood pressure was 170/110 mm Hg with less than 10 mm Hg difference of systolic and diastolic blood pressure in other limbs

Abdominal examination revealed abdominal bruit

Fundus examination revealed papilledema with changes of hypertensive retinopathy

The cranial nerves, motor, sensory, and autonomic system examination were unremarkable

There was no evidence of neurocutaneous markers

His cranial magnetic resonance imaging revealed bilateral white matter hyperintensity that was more marked in the bilateral parieto-occipital region

Ultrasound of the abdomen revealed a small right kidney and renal Doppler was suggestive of right renal-artery stenosis

His computed tomography angiography revealed short segment (3 cm) and short circumferential thickening of the descending aorta with right renal artery narrowing (Figure 1)

His repeat cranial magnetic resonance imaging showed significant reduction in the white matter hyperintensities

Maximum-intensity projection computed tomography (CT) angiography abdomen axial section (thickness 7 mm) (A) showing narrowing of whole length of right renal artery and thickened abdominal aortal wall (black arrowhead) and coronal section (B) showing small asymmetrical right kidney and compensatory enlarged left kidney


A 7-year-old girl was referred to the otolaryngology clinic by her pediatrician for a left pharyngeal lesion

Her parents noted the lesion 18 months prior

It remained stable in size and was asymptomatic

She denied sore throat, dysphagia, and dyspnea

She was otherwise healthy with no history of recurrent pharyngitis

On examination, her vital signs were within normal limits

She was comfortable with no noted stridor and tolerated her oral secretions

Her oropharyngeal examination was notable for size 2+ palatine tonsils without exudate and a white mass on the posterior tonsillar pillar

She did not tolerate palpation of the lesion

There was no palpable cervical adenopathy, and her lungs were clear to auscultation

No laboratory work or imaging were performed

The patient was taken to the operating room for an excisional biopsy

There, a more thorough gross examination of the lesion was notable for a pedunculated, white rubbery mass arising from the left posterior tonsillar pillar with a narrow mucosal stalk (Figure, A)

A, Pedunculated, white rubbery mass arising from the left posterior tonsillar pillar with a narrow mucosal stalk

B and C, A keratinizing squamous epithelium with Actinomyces colonization was found overlying pilosebaceous units including hair follicles, arrector pili muscles, and sebaceous glands (hematoxylin-eosin stains)

Gross examination revealed a 2.2 × 1.1 × 0.7-cm, pink-tan to purple, rubbery lesion

Histologic examination demonstrated both ectodermal and mesodermal components

A keratinizing squamous epithelium with Actinomyces colonization was found overlying pilosebaceous units including hair follicles, arrector pili muscles, and sebaceous glands (Figure, B and C)

The core of the lesion comprised adipose and fibrous tissue

The stalk was covered with acanthotic squamous epithelium, and the base contained embedded salivary glands


A 75-year-old man with myelodysplastic syndrome (MDS) with isolated deletion 5q (del5q) presented with multiple nonhealing skin ulcers on the chest and upper extremities

Four years earlier, he started taking lenalidomide, 10 mg/d (28-day cycle), to treat worsening anemia

after 2 cycles of treatment, he became transfusion independent

Since then, he has remained stable with the same dose and schedule of lenalidomide without significant adverse effects

The presenting skin lesions developed 1 month before presentation and began as small pustules that rapidly transformed into painful ulcers

He was treated with valacyclovir for a presumed diagnosis of shingles, but the lesions did not improve

Because of suspected necrotizing fasciitis, he underwent incision and drainage of the lesions

Cultures from the surgical specimen grew methicillin-resistant Staphylococcus hemolyticus

However, the lesions continued to worsen despite vancomycin and additional debridement

Thus, he was transferred to our facility

Physical examination showed multiple cutaneous ulcers with gray undermined borders and red papules of varying depths involving the chest, back, and upper extremity

A complete blood cell count showed a white blood cell count of 16.2 × 103/μL (to convert to 109/L, multiply by 1.0) with an absolute neutrophil count of 12.7 × 103/μL, hemoglobin level of 9.4 g/dL (to convert to g/L, multiply by 10.0), and platelet count of 262 × 103/μL with no circulating blasts

Results of additional laboratory studies, including blood culture and tests for herpes zoster virus (via polymerase chain reaction), antinuclear antibody, rheumatoid factor, and antineutrophil cytoplasmic antibody were all negative

Additionally, the patient also had hypertension and hypercholesterolemia for which he was taking lisinopril and simvastatin, respectively

Pathologic sampling from skin lesions demonstrated a dense dermal infiltrate with predominantly neutrophils along with reactive histiocytes, fibroblasts, and angioplasia (Figure, A)

Immunohistochemical staining showed myeloperoxidase-positive neutrophils (Figure, B), histiocytic markers (CD43, CD63, CD168), and CD117-positive mast cells, but no immature myeloid precursors

Results of Grocott-Gomori methenamine silver staining were unremarkable

A, Hematoxylin-eosin staining shows polymorphonuclear neutrophils admixed with scattered histiocytes and vasculatures with a prominent endothelial lining

B, Immunohistochemical staining shows myeloperoxidase-positive neutrophils peripherally and in aggregates


A 74-year-old man with a history of multiple squamous cell carcinomas that were treated with Mohs micrographic surgery presented with a tender, enlarging scalp lesion of 6 months’ duration

Skin examination revealed a 5-mm bright pink, dome-shaped papule with overlying vessels located at the crown of the scalp (Figure, A)

There was no purulent discharge or overlying skin changes

A shave skin biopsy was performed and submitted for histopathologic analysis (Figure, B-D)

A, Bright pink, dome-shaped papule of the scalp crown with overlying vessels measuring 5 mm

B, Hematoxylin-eosin staining of the lesion from biopsy

Scanning magnification demonstrates dermal-based tumor cells arranged in a vague nodular configuration

C, Proliferation of tumor cells is present around small vessels with scattered mitoses (black arrowhead) and necrosis (yellow arrowheads) (hematoxylin-eosin)


An otherwise healthy female patient in her late 20s was seen with a 2-year history of multiple, slowly growing, infiltrated erythematous-violaceous patches and plaques, with occasional slight pain extending from her left upper arm to the left side of her chest

No triggers, such as local infection, insect bite, or trauma, occurred before the appearance of the lesions

She denied fever, weight loss, or fatigue

Physical examination showed multiple firm, nonscaly, purple erythematous patches and plaques with irregular borders on her left axilla, medial upper arm, and lateral thoracic region (Figure, A)

No extracutaneous involvement was observed

A skin biopsy specimen was obtained from the left side of her chest for histopathological examination (Figure, B and C)

A, Multiple nonscaly, erythematous-violaceous patches and plaques with irregular borders on the patient’s left axilla, medial upper arm, and lateral thoracic region

B, Irregularly branched, thin-walled blood vessels dissecting through collagen bundles within the superficial to mid dermis (hematoxylin-eosin)

C, Irregularly branched, thin-walled blood vessels with plump endothelial cells dispersed between thickened collagen bundles without signs of atypia (hematoxylin-eosin)

D, Endothelial cells positive for cluster of differentiation (CD) 31 (CD31)


High-grade serous carcinoma of gynecological origin

High-grade carcinoma of the breast with occult primary

High-grade papillary carcinoma of unknown primary

A 76-year-old woman presented with a palpable left axillary mass

When she was 36 years old, she underwent a hysterectomy due to abnormal uterine bleeding and was taking estradiol to control postmenopausal vasomotor symptoms for the past 20 years

Bilateral diagnostic mammography revealed a high-density irregular mass in the left axilla with no nodules, architectural distortions, or microcalcifications in the breasts

Ultrasonography of the left breast showed a 4.6-cm lobulated mixed cystic and solid left axillary mass

Contrast-enhanced magnetic resonance imaging showed an enhancing 5.2-cm left axillary mass with no breast lesions (Figure 1A)

The biopsy examination indicated high-grade adenocarcinoma with papillary features (estrogen receptor– and progesterone receptor–positive, ERBB2 [formerly HER2]–negative, and a high Ki67 index)

Positron emission tomography/computed tomography revealed only high uptake in the left axilla region

The patient was treated at an outside hospital with neoadjuvant dose-dense doxorubicin and cyclophosphamide, followed by weekly paclitaxel for 10 weeks

She then transferred her care to our center for surgical management

We requested the pathology slides for review at our laboratory and additional immunostaining was performed, which revealed high-grade adenocarcinoma with tumor cells positive for PAX8 (Figure 1B), p53, and WT1 and negative for GATA3.A, Magnetic resonance image of the breasts shows an enhancing 5.2-cm left axillary mass (arrowhead) but no breast lesions

B, Immunohistochemical staining revealed tumor cells positive for PAX8 (original magnification ×100)


Cervical chondrocutaneous branchial remnant

A 7-year-old boy presented with a slowly growing, asymptomatic lump on his left lower neck since birth

His parents denied any medical history of trauma, infection, or surgery

Physical examination showed a 1.3 × 0.7 × 0.6 cm, yellowish, hump-like mass with hairy surface and cartilage-like consistency on the anterior border of the lower third of the left sternocleidomastoid muscle (SCM) (Figure, A)

Ultrasonography revealed a 1.2 × 1.0 × 0.9 cm, hypoechoic, avascular, bulging nodule with an anechoic tubular structure extending to the subcutaneous fat and reaching the surface of the muscular layer

Magnetic resonance imaging (MRI) demonstrated a protuberant nodule with diffuse, slight hyperintensity on T1WI

and 2 mild hyperintense foci surrounded by hypointense tissue on fat-suppressed T2WI (Figure, B)

The neck mass was completely excised under general anesthesia in the department of pediatric surgery

Intraoperatively, the mass extended into the anteromedial fascia of the left SCM and did not involve the deeper neck structures

Postoperative histopathologic analysis showed normal-appearing epidermis, multiple pilosebaceous follicles, abundant adipose tissue, and 2 centrally circumscribed segments of cartilage in the subcutis (Figure, C)

Van Gieson stain highlighted elastic fibers surrounding individual chondrocytes

A, A yellowish hump-like mass with hairy surface on the anterior-inferior third border of the left sternocleidomastoid muscle (SCM)

B, Transverse T2-weighted, fat-suppressed magnetic resonance image (MRI) revealed a nodular bulge with 2 mild hyperintense foci (arrowheads) surrounded by hypointense tissue

C, Whole-slide hematoxylin-eosin stain showed normal-appearing epidermis, multiple pilosebaceous follicles, abundant adipose tissue, and 2 centrally circumscribed segments of cartilage (arrowheads) in the subcutis (original magnification ×10)


An 11-year-old girl with no significant medical history was referred to the pediatric otolaryngology department for a painless, left-sided neck mass that had been slowly enlarging for 8 months

On examination, there was an easily palpable 3-cm firm, fixed, well-circumscribed left supraclavicular mass without overlying skin changes or cutaneous involvement

She had no other masses, cervical lymphadenopathy, or systemic symptoms

In workup for this mass, she initially underwent ultrasound imaging, which demonstrated a hypoechoic mass

Findings from fine-needle aspiration and core-needle biopsy were inconclusive, demonstrating histiocytes

Magnetic resonance imaging demonstrated a well-circumscribed mass of mainly soft tissue density with contrast enhancement (Figure 1)

Magnetic resonance imaging of the left-sided supraclavicular mass

T2 sequencing with contrast demonstrates strong enhancement of the mass

Given persistence of the lesion, and inconclusive biopsies, the decision was made to pursue surgical excision

In the operating room, a well-defined 5.5-cm left level V mass was excised

The mass was found to attach deep to the middle scalene and was fed by several unnamed vessels

however, there was no involvement of the clavicle

Pathology confirmed complete excision of a well-circumscribed lesion with peripheral casing of bone, cyst-like spaces filled with serous fluid, walls of spindle-shaped fibroblasts, osteoclast-type giant cells, thin trabeculae of woven bone surfaced with osteoblasts, and scattered macrophages

There was local infiltration into surrounding skeletal muscle, but no atypical mitoses or pleomorphic cells were identified

The lesion was positive for USP6 locus rearrangement (17p13.2) on florescence in situ hybridization


A healthy, uncircumcised man in his 30s presented with a 14-year history of dyspareunia and recurrent, purulent discharge from his penis

He had undergone multiple evaluations by dermatologists and urologists and had not responded to systemic antibiotics and topical corticosteroids

Physical examination revealed an erythematous, pinpoint orifice on his glans penis, with peripheral telangiectasias and a linear bluish structure on dermoscopy (Figure, A and B)

Ultrasonography revealed a hypoechoic focal zone on the right side of the glans, with multiple hyperechoic bilaminar structures (Figure, C)

A, Clinical image of glans penis with a pinpoint orifice

B, Dermoscopy reveals an orifice surrounded by peripheral telangiectasias and an extending bluish linear structure

C, Ultrasonography of the glans penis demonstrates a hypoechoic focal zone in the parenchyma on the right containing multiple hyperechoic bilaminar tracts (arrowheads) (18 MHz

D, Hematoxlyin-eosin stain with original magnification ×100.


Cutaneous deposits of metastatic breast cancer

A 56-year-old woman presented at the clinic for follow-up of metastatic hormone receptor–positive, ERBB2-negative breast cancer

She had locally advanced disease that was invading the skin overlying her right breast and metastatic disease to the thoracic and lumbar spine, liver, and mediastinal, axillary, and retroperitoneal lymph nodes

Her disease had progressed after receiving first-line therapy with palbociclib and anastrozole

Subsequent treatments with fulvestrant and paclitaxel were also followed by progressive disease

She received treatment with capecitabine

after 6 weeks of treatment, she developed some redness on her hands and feet that resolved on its own

She then presented to the clinic with pruritic, erythematous, scaly macules and papules on her forearms, faces, chest, and upper back (Figure)

The rash had been present for 3 weeks and had recently worsened

The rash did not respond to treatment with topical clindamycin, oral solumedrol, or doxycycline

She had a history of substantial sun exposure but no history of skin cancer


A 12-year-old girl presented to the clinic with a 3-month history of intermittent stridor

Her symptoms were initially most prominent while playing sports and were suspected to be due to asthma or seasonal allergies

However, medical management with albuterol, intranasal fluticasone, and cetirizine failed to provide relief

More recently, the patient’s parents noted that she developed stridor while sleeping and while at rest, prompting the family to present for medical attention

Previous workup, including pulmonary function testing, was concerning for an upper airway obstructive process, for which she was referred to otolaryngology

In-office flexible videostroboscopy revealed a mass in the distal cervical trachea that appeared nearly obstructive

Chest radiography confirmed the presence of an approximately 1.5-cm, well-circumscribed soft tissue mass within the cervical trachea

Direct laryngoscopy and bronchoscopy were performed in the operating room under general anesthesia

This demonstrated an exophytic, pedunculated mass that emanated from the anterior wall of the cervical trachea (Figure 1A and B)

The patient was intubated via Seldinger technique to bypass the mass, with a 4.0-mm cuffed endotracheal tube loaded over a 0-degree Hopkins endoscope (Figure 1C)

With the airway secured, attention was turned to excision of the mass

Lidocaine, 1%, with epinephrine 1:100 000 was injected in a submucosal plane, and laryngeal scissors were used to excise the attachment of the mass from the tracheal wall

The mass was then resected en bloc and removed with laryngeal cupped forceps

The attachment site was then ablated with the Coblator (Figure 1D)

Intraoperative photographs demonstrating supraglottic view of intratracheal mass (A)

exophytic, pedunculated mass emanating from the anterior wall of the cervical trachea (B)

endotracheal intubation via Seldinger technique bypassing the mass (C)

and cervical trachea following ablation of attachment site (D)


A 25-month-old child with unilateral hearing loss was referred to a pediatric ear, nose, and throat surgeon in a secondary care hospital

Clinical findings suggested unilateral right middle ear effusion associated with ipsilateral conductive hearing loss

After an initial observation period, a right tympanostomy tube was inserted

During the procedure, a clear liquid pulsating from the middle ear was noted, and samples were sent for β-2-transferrine analysis

This confirmed the presence of a cerebrospinal fluid (CSF) leak

The patient was referred to our tertiary referral center hospital

The leak settled with conservative management, and imaging of the brain and skull base was performed

A high-resolution computed tomography scan of the temporal bone showed a lytic aspect of the right temporal bone extending to the petrous apex and the petrous bone, sparing the otic capsule (Figure 1A)

There was soft tissue opacification of the right mastoid air cells

Magnetic resonance imaging of the skull base with contrast was performed (Figure 1B)

It showed a hyperintense T2 signal in the petrous apex and mastoid air cells

There was a suspected communication into the soft tissues of the right neck in the posterior triangle

Magnetic resonance imaging also showed signs of Chiari malformation type 1. After a skull base multidisciplinary discussion, the patient underwent a bone biopsy through a transmastoid approach

A, Axial high-resolution computed tomography scan of the temporal bones at the level of the basal turn of the cochlea showed osteolysis involving the petrous part of the right temporal bone

There was associated soft tissue partial opacification of the right mastoid air cells

The destructive process did not involve the otic capsule

B, Coronal magnetic resonance imaging of the brain and skull base, T2-weighted images showed a hypersignal in the right mastoid and middle ear with suspected communication through the tegmen

There was also a hypersignal in the soft tissues of the right neck in keeping with extravasation of cerebrospinal fluid in cervical lymphatics


A woman in her 80s initially presented with numerous, primarily photodistributed, crusted, and ulcerated plaques of the trunk and extremities

She had a medical history of essential thrombocytosis (ET), which was treated with hydroxyurea for 14 years, with a total cumulative dose of approximately 2500 g

She was otherwise reasonably well and denied any other constitutional symptoms

The ulcerations had slowly increased in number and size over several years and were quite painful (Figure, A-C)

She did have a medical history of nonmelanoma skin cancer (NMSCa), which had previously been treated surgically

Several tangential shave biopsies were performed to help clarify the patient’s diagnosis

A-C, Clinical findings including widespread photodistributed ulcerated pink papules and plaques on the extremities

D, Histopathologic findings (hematoxylin-eosin stain)


A woman in her late 40s was admitted to the hospital with a 40-year history of multiple hard nodules on the scalp

At 5 years of age, the patient had developed multiple subcutaneous masses in the occipital and bilateral temporal regions

At age 10, she was diagnosed with neurofibromatosis and underwent surgical resection of the tumors at a local hospital

Tumors recurred 5 years after surgery, with their size and number gradually increasing with age

Physical examination revealed scalp masses from 4 to 6 cm in diameter, with hard textures and smooth surfaces (Figure, A), and subcutaneous nodules of 1 × 1 cm to 2 × 2 cm with hard textures on the hands (Figure, B), feet, trunk, and lower extremities

The patient had no tenderness, swelling, or other skin manifestations

The patient had consanguineous parents, but no other family member, including her children, had the disease

A, Multiple subcutaneous masses in the occipital and bilateral temporal regions

The diameter of the scalp masses varied from 4 to 6 cm, with hard textures and smooth surfaces

B, Subcutaneous hard nodules on the hands, ranging from 1 × 1 cm to 2 × 2 cm

C and D, Large amounts of homogeneous eosinophilic hyaline matrix with scattered fibroblasts


A man in his 50s presented with bleeding wounds and nodules on the bilateral hips and forearms

He previously received failed systemic and radiation therapy for tumor stage mycosis fungoides (MF)

He had received 11 courses of extended beam radiation therapy, including 3 courses of total skin irradiation of 24 to 30 Gy, and focal irradiation to the left posterior thigh and hip/buttock with 2.5/3 Gy to 12.5/24 Gy, respectively, and the right posterior thigh and hip with 2 Gy to 20 and 26 Gy, respectively

Seven years before this visit, he underwent a reduced-intensity, matched, unrelated donor, allogeneic hematopoietic stem cell transplant (HSCT)

His post-transplant course was complicated by severe chronic graft vs host disease of the skin, eyes, and gut, for which he received systemic corticosteroid therapy, tacrolimus, methotrexate, and long-term extracorporeal photopheresis

Three years after the HSCT, he developed chronic ulcerations on the hips, back, and arms at the site of previously irradiated tumors, as well as multiple friable, hemorrhagic, and bleeding nodules on bilateral hips and his forearms while receiving treatment with corticosteroids and extracorporeal photopheresis

He underwent several debulking procedures and excision biopsies of the nodules, which revealed granulation tissue on histology

In this latest visit, physical examination revealed recurrent hemorrhagic and friable nodules on his left lateral hip and left lateral buttock (Figure 1)

Similar nodules were noted on his right hip and forearm

The patient underwent repeated debulking and biopsy

He was also treated with micafungin, amphotericin B, levofloxacin, dapsone, acyclovir, pentamidine, penicillin VK, and posaconazole

Positron emission tomography (PET) computed tomography (CT) results did not show evidence of visceral or extracutaneous disease

Recurrent hemorrhagic and friable nodules on the left lateral hip


Invasive fungal sinusitis with orbital extension

A 48-year-old woman presented with 3 days of worsening right orbital swelling, eye pain, blurry vision, and 3 months of bilateral photophobia

She also reported bilateral rhinorrhea, congestion, and right frontal and temporal headaches

Active medical issues included hypertension, very poorly controlled type 2 diabetes, hyperlipidemia, tobacco use, and asthma

Her brother and father both had a history of chronic orbital swelling

Vital signs were unremarkable, and examination of the right eye showed moderate chemosis, injected conjunctiva, and periorbital edema

Both eyes were proptotic, right worse than left

She had normal bilateral extraocular movements, pupillary light reflexes, and gross vision

Computed tomography with contrast revealed bilateral ethmoid and frontal sinus opacification and right worse than left orbital fat stranding (Figure 1A)

Magnetic resonance imaging with contrast demonstrated both enlargement and enhancement of bilateral extraocular muscles, especially the right superior rectus (Figure 1B)

Initially she was started on antibiotic therapy

Right nasal biopsy, right endoscopic sinus surgery, and right anterior superior orbital tissue biopsy were performed

Intraoperatively, the sinus mucosa demonstrated only edema and mild secretions, but there was no necrosis or purulence

Both orbital and nasal cultures grew methicillin-resistant Staphylococcus aureus

Orbital biopsy showed nodular lymphoplasmacytic infiltrates without evidence of fungal infection or necrosis (Figure 1C)

Further workup revealed elevated erythrocyte sedimentation rate, C-reactive protein, total IgG, and IgG4 levels and normal white blood cell count, thyroid-stimulating hormone, angiotensin-converting enzyme, lysozyme, antinuclear antibody, and antineutrophil cytoplasmic antibody (ANCA) levels

A, Computed tomography with contrast revealed bilateral ethmoid and frontal sinus opacification and right worse than left orbital fat stranding

B, Magnetic resonance imaging with contrast demonstrated both enlargement and enhancement of bilateral extra ocular muscles, with the right superior rectus muscle being the most severely affected

C, Orbital biopsy showed nodular lymphoplasmacytic infiltrates separated by thick collagen bundles, without evidence of fungal infection or necrosis (hematoxylin-eosin)


A 69-year-old woman with a newly diagnosed T3N2cM0 poorly differentiated squamous cell carcinoma unrelated to human papillomavirus (HPV) of the lower lip mucosa presented to the medical oncology clinic 3 days after initiating neoadjuvant immune checkpoint blockade immunotherapy with redness and swelling of the tumor site

Four days after treatment, a white head formed overlying the tumor that erupted through the skin surface (Figure 1)

Thin, cloudy discharge that was not foul-smelling drained from the tumor from posttreatment days 5 to 10, at which point the drainage stopped spontaneously

Throughout, the patient reported no pain and remained afebrile

Laboratory results on days 5, 9, and 14 demonstrated the absence of leukocytosis, and serial wound cultures failed to demonstrate pathogenic growth

The patient completed the neoadjuvant immunotherapy and underwent a margin-negative tumor resection without intraoperative or postoperative complications

This photo documents clinical tumor behavior after neoadjuvant immunotherapy treatment


Pigmented dermatofibrosarcoma protuberans (Bednar tumor)

An otherwise healthy woman in her 30s presented with a painful bruise on her hand that initially appeared a few days after she sustained a twisting injury to the affected hand while participating in acroyoga 2 years before

Since the initial onset, the bruise and pain had persisted despite icing and resting the area

She denied any personal or family history of bleeding disorders

On examination, an ill-defined indurated blue-green plaque was noted on the dorsum of the left hand (Figure 1)

Radiography results showed soft tissue swelling but were otherwise unremarkable

Subsequent magnetic resonance imaging showed a heterogeneously fluid hyperintense and T1 heterogeneously hyperintense lesion measuring approximately 1.6 × 0.5 × 2.9 cm that was centered within the dorsal subcutaneous soft tissue of the hand


Eosinophilic, polymorphic, and pruritic eruption associated with radiotherapy (EPPER)

A woman in her 60s presented for evaluation of a new pruritic rash

The rash initially developed on the patient’s legs 13 days before presentation and subsequently progressed to her feet, arms, hands, and trunk

Associated symptoms included a burning sensation and occasional pain

Her medical history was notable for endometrial adenocarcinoma status post hysterectomy and recent postoperative radiotherapy (total of 50.4 Gy in 28 fractioned doses), which was completed 4 days after the onset of the rash

She denied any new medications or exposure to chemotherapy, imaging with intravenous contrast, or recent travel

The remainder of her review of systems was unremarkable

On physical examination, there were numerous erythematous edematous coalescing papules and plaques on the upper and lower extremities and scattered erythematous papules and plaques on the back and buttocks (Figure, A)

Some plaques were annular and polycyclic in configuration, with central hyperpigmentation (Figure, B)

Her chest, abdomen, face, and oral mucosa were clear

A complete blood cell count was notable for an eosinophilia of 11%, with an absolute cell count of 0.80 ×103/uL

Results from a comprehensive metabolic panel were normal

A biopsy was performed on her right thigh for histopathological examination

A, Clinical photographs show numerous erythematous edematous coalescing papules and plaques on the bilateral upper and lower extremities

B, Some plaques were annular and polycyclic in configuration with central hyperpigmentation

C and D, Punch biopsy results demonstrated a moderately dense perivascular and interstitial infiltrate of mononuclear cells with innumerable eosinophils (hematoxylin-eosin stain)


Synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome

A 51-year-old previously healthy man was admitted with severe neck pain for 6 days

In the past month, he felt neck stiffness, accompanied by swelling and persistent dull pain of the upper anterior chest wall, which could be transiently relieved by taking oral ibuprofen

Six days before admission, the patient’s neck stiffness transited to an endurable dull ache

When turning his head, there was severe burning pain in the posterior neck and occiput, followed immediately by an electric shocklike numbness on ipsilateral side tongue, which led to a forced head position

Magnetic resonance imaging of the cervical spine with gadolinium on admission revealed signal abnormality in C1/C2 vertebrae (Figure 1A)

In the past 3 days, with the intensification of neck pain, the patient found pustules on both hands, which gradually increased

He had no history of tobacco, alcohol, or drug misuse

There was tenderness in the right sternoclavicular articulation, upper neck, post aurem, and occiput

Multiple pustules were seen in the palm and back of both hands (Figure 1B)

Complete blood cell count and liver and kidney function yielded normal results, except for elevated erythrocyte sedimentation rate (ESR) of 93 mm/h and C-reactive protein (CRP) level of 5.5 mg/dL (to convert to milligrams per liter, multiply by 10)

Results of workup for rheumatic and infectious disease, including antinuclear, antineutrophil cytoplasmic and anticardiolipin antibodies, human leukocyte antigen B27, repeated blood cultures, rapid plasma reagin test, Aspergillus galactomannan antigen, brucella, Mycobacterium tuberculosis, and rickettsial antibodies, were all negative

Cell count, protein, and glucose in cerebrospinal fluid were normal

Cultures of cerebrospinal fluid revealed no organisms

Whole-body fluorodeoxyglucose–positron emission tomography/computed tomography revealed no malignancy

Abnormal enhancement of C1/C2 vertebrae with prevertebral and retrodental tissue as well as of the anterior vertebrae at C4/C5 with hyperostotic anterior osteophytes on magnetic resonance imaging (MRI) (A) and multiple pustules on the palm (B)