Multiple flat-topped scaly violaceous papules.

Epidermodysplasia verruciformis (EV) is an autosomal recessive genodermatosis characterized by susceptibility to beta-genus human papillomavirus (HPV) infection. Owing to TMC6/EVER1 and TMC8/EVER2 mutations that lead to abnormal transmembrane channels in the endoplasmic reticulum involved in immunological pathways, keratinocytes cannot combat infection from non-pathogenic HPV strains. Mutations involving RHOH, MST-1, CORO1A, and IL-7 have also been associated with EV in patients without TMC6 or TMC8 mutations. We highlight a 27-year-old man with multiple violaceous flat-topped papules with scale and irregular borders distributed on his chest, extremities, abdomen, and back. The striking physical examination and the subsequent biopsy findings of enlarged nests of cells in the granular and spinous layers with blue-gray cytoplasm and keratohyaline granules confirmed the diagnosis. We conclude with a brief discussion on the differential diagnosis, which includes confluent and reticulated papillomatosis, Darier disease, and disseminated superficial actinic porokeratosis.

Acute inflammatory edema as a variant of pseudocellulitis resolved after transcatheter aortic valve implantation.

Acute inflammatory edema is a noninfectious inflammatory condition of the skin that is commonly seen in critically ill patients. It is characterized by edematous, erythematous, and nontender plaques involving the abdomen and thighs, sparing areas of the skin subject to pressure. Risk factors include fluid overload, hypoalbuminemia, and obesity. Differentiating acute inflammatory edema from cellulitis can be challenging. Supportive care is the mainstay therapy for acute inflammatory edema, with interventions primarily focused on lowering the fluid burden. We report an unusual case of a nonobese patient in the outpatient setting with acute inflammatory edema.

Periorbital cutaneous neonatal lupus.

The authors report 3 infant girls with a similar periorbital eruption. A 5-month-old infant girl presented with a 3-month history of a photosensitive facial eruption that had failed to respond to topical 1% hydrocortisone cream. The otherwise healthy infant was the product of a term pregnancy. The 25-year-old mother was in good health except for occasional arthralgias. She had 2 other healthy children. Physical examination revealed a well-developed, well-nourished infant with annular, polycyclic, erythematous scaly plaques involving the cheeks and periorbital region (Figure 1). Potassium hydroxide scraping from the face was negative for dermatophyte and yeast. Laboratory studies revealed normal complete blood cell count, normal liver function tests, strongly positive anti-SSA antibody at 118 units (>80 units, strongly positive), and a negative anti-SSB antibody. Cardiac examination and electrocardiogram (ECG) were also normal. Laboratory evaluation of the mother revealed a positive anti-SSA at 158 units and mild anemia, but anti-SSB, anti-Smith, U1RNP, and anti-Scl-70 antibodies were all negative. Within 4 months the facial eruption cleared completely with the use of desonide cream 0.05% applied twice a day and sun protection. A 6-month-old girl was referred for dermatitis that began on the trunk and face at about 2 months of age. Although the truncal component resolved after 2 months, the facial dermatitis persisted. The infant was in good health and was the first-born child. The mother was known to have Sjögren syndrome. Physical examination revealed the characteristic erythematous, annular, scaling, polycyclic plaques along the forehead, periorbital cheeks, and eyelids (Figure 2). Laboratory evaluation of the infant revealed normal complete blood cell count, liver function tests, and chemistry profile. Anti-SSA antibody was positive at >6 units (reference, <1 unit) and anti-SSB antibody was positive at 2.84 units (reference, <1 unit). U1RNP antibody was negative. Cardiac examination and ECG were normal. The skin abnormalities cleared completely in 6 weeks with the topical application of tacrolimus 0.03% ointment and sun protection. A 5-month-old girl presented with a 2-month history of a persistent facial dermatitis. The infant was in good health and was the product of a healthy first pregnancy and delivery. The mother was in good health. Physical examination of the infant revealed erythematous, annular, polycyclic periorbital patches (Figure 3). Laboratory evaluation revealed positive SSA and SSB antibodies (units unavailable) and normal complete blood cell count, liver function tests, and chemistry profile. Cardiac examination and ECG were normal. The mother's laboratory results were also positive for anti-SSA and anti-SSB antibodies (units unavailable). The infant's facial eruption resolved without specific treatment.

Leuprolide acetate-induced dermatitis herpetiformis.

Dermatitis herpetiformis (DH) is a chronic, pruritic, papulovesicular dermatosis on extensor surfaces that is characterized by a neutrophilic infiltrate and granular immunoglobulin A deposition at the dermal papillae. Although the presence of immunoglobulin A in the skin and the severity of DH are known to be associated with gluten intake, few drugs have been implicated in the induction of DH. We report a case of DH triggered by intramuscular injections of leuprolide acetate, a gonadotropin-releasing hormone analog, in a patient with a history of prostate cancer.

A case of calciphylaxis and chronic myelomonocytic leukemia.

A 70-year-old woman presented for evaluation of symmetric necrotic ulcers of the lower extremities. Biopsy results revealed changes consistent with calciphylaxis. The predisposing factors in this patient included calcium supplementation, obesity, female gender, viscous blood, renal failure, and diabetes mellitus. To our knowledge, this is the first report of calciphylaxis occurring in the setting of chronic myelomonocytic leukemia. We discuss the history, clinical presentation, diagnosis, and treatment of calciphylaxis.

Pemphigus vulgaris and paraneoplastic pemphigus.

The history, epidemiology, pathophysiology, clinical presentation, diagnostic work-up, histopathology, and treatment of PV and PNP have been presented. These life-threatening, autoimmune, mucocutaneous bullous conditions may be encountered first by oral health providers and, therefore, deserve keen understanding and attention by the oral and maxillofacial surgeon. Great diagnostic and management strides have been made, but morbidity and life quality issues remain a reality for these chronically ill patients.

Cutaneous tumors as the initial presentation of non-T, non-B, nonmyeloid CD4+ CD56+ hematolymphoid malignancy in an adolescent boy.

We describe a 14-year-old Hispanic boy who presented with a 2-month history of enlarging plum-colored cutaneous tumors on his face, trunk, and proximal extremities. Histopathologic examination showed nodular infiltrates of malignant mononuclear cells extending from the superficial dermis to the deep subcutis. Immunohistochemical staining of the biopsy specimen and flow cytometry studies on a bone marrow aspirate revealed a CD4+, CD56+ hematolymphoid tumor that was negative for all other myeloid and lymphoid markers. Based on this information, the patient was diagnosed with the recently described, rare non-T, non-B, nonmyeloid CD4+ CD56+ hematolymphoid malignancy. To our knowledge, this is the youngest patient reported in the literature.

Adult onset of eruptive widespread Spitz's nevi.

Cases of multiple Spitz's nevi are rare, usually occurring in the agminate form or even less commonly as widespread eruptive Spitz's nevi. Previously reported cases of widespread eruptive Spitz's nevi arose in persons age 23 or younger. We describe a Hispanic male patient with eruptive Spitz's nevi that presented at the age of 35 years.