Maria Medenica

Birt–Hogg–Dubé Syndrome

Birt–Hogg–Dubé Syndrome is an autosomal dominant condition characterized by a triad of fibrofolliculomas, trichodiscomas, and acrochordons. Since the first description in 1977, many conditions have been described in association with its clinical triad. Recent epidemiological studies have shown a significant association between the occurrence of lesions in the fibrofolliculoma/trichodiscoma category with renal neoplasms and pneumothoracies. The BHD protein folliculin had recently been identified. The histological findings of the clinical lesions are distinctive. We report a patient with a history of melanoma who presented for routine surveillance. Facial lesions in the fibrofolliculoma/trichodiscoma category were identified. Diagnostic work‐up revealed concomitant multinodular goiter, pulmonary cyst, and renal mass. The patient later developed pneumothorax. Clinical manifestations, histological findings, associations, management, and a review of the Birt–Hogg–Dubé Syndrome are discussed.

Eccrine syringofibroadenoma (Mascaro). Report of two cases.

We report two cases of eccrine syringofibroadenoma. Both patients had a large, solitary hyperkeratotic nodular lesion over the extremities. Histologic sections showed spongelike masses of small cuboidal acrosyringeal cells. Fibrovascular connective tissue stroma resembling premalignant fibroepithelial tumor of Pinkus filled the spaces between the masses.

Reduced threshold to suction-induced blister formation in insulin-dependent diabetics

Diabetics may have an increased susceptibility to cutaneous blister formation clinically manifest as the bullous eruption of diabetes. We evaluated the mechanical force necessary to induce suction blisters in fifteen insulin-dependent diabetics and twenty age-matched normal controls. The mean suction blister threshold for the diabetics was 31.9 minutes versus 68.0 minutes for the controls, a highly significant difference (p less than 0.01). Histologic appearance of suction blisters was similar in diabetic and normal skin, under both light and electron microscopic examination, with a noninflammatory subepidermal separation in the lamina lucida between the cell membrane and the basal lamina. These results demonstrate that insulin-dependent diabetics have a marked reduction in suction blister threshold as compared to age-matched controls.

Interleukin-2 associated linear IgA bullous dermatosis

Linear IgA bullous dermatosis (LABD) in adults is characterized by subepidermal bullae associated with a linear deposition of IgA at the basement membrane zone. Its cause is unclear, although it appears to have an immune-mediated basis. The development of LABD in a cancer patient undergoing immunotherapy has been described in the French literature. We describe a similar case of LABD arising in a patient while undergoing interleukin-2 immunotherapy for renal cell carcinoma.

Multiple eccrine spiradenoma: case report and review

Benign eccrine spiradenoma is an adnexal tumor that occurs as either a solitary lesion or as multiple nodules. A case of multiple eccrine spiradenoma occurring on the scalp and mimicking multiple cylindroma is described and discussed. In addition, 9 previously reported cases of multiple eccrine spiradenoma are reviewed.

Isolation of Trichophyton soudanense in the United States

A case of tinea corporis, localized to the left side of the chin in a 68 year old Negro woman consistently yielded on culture the African dermatophyte Trichophyton soudanense. The patient is a native born U.S. citizen who has never been outside the country. This is the first recorded isolation of this organism in the United States.

Multiple adnexal tumors and a parotid basal cell adenoma

A 72-year-old woman had a preauricular mass and several scalp nodules. Her medical history included excisions of multiple cutaneous tumors, including cylindromas, trichoepitheliomas, basal cell epitheliomas, and a trichilemmoma. The parotid mass and a scalp nodule were excised. On light microscopy, the parotid mass and scalp nodule appeared similar. Ultrastructural features revealed by electron microscopic analysis of the tumors allowed diagnosis of a cylindroma of the scalp and a parotid basal cell adenoma. The association of trichilemmoma and membranous basal cell adenoma of the parotid has not been previously described. The pathogenesis of these multiple cutaneous tumors from a common cell of origin is considered.

Two Cases for Diagnosis: Asymmetric Childhood Scleredema or Stiff Skin Syndrome?

Abstract: Two children with chronic and asymmetric skin indurations are presented. The clinical and pathologic features are suggestive of asymmetric childhood scleredema and stiff skin syndrome. The key features of scleredema and stiff skin syndrome are discussed.

SOLITARY TRUNCAL POROKERATOTIC ECCRINE OSTIAL AND DERMAL DUCT NEVUS IN A SIXTY‐YEAR‐OLD MAN

A sixty‐year‐old white man presented with a 2 × 1.5 cm lesion on his right buttock. The lesion had been present for several years, but had not been present when the patient was a child. No other family members reported having similar lesions. His past medical history was unremarkable. By history, the lesion was slowly growing, but was neither painful nor pruritic. On examination, a well defined pink nodule was noted on his right buttock. The surface of the lesion had a crateriform appearance with verrucous edges. A shave biopsy was performed.

Pigmentary changes in Seckel’s syndrome

Seckels syndrome consists of multiple congenital anomalies, including bird-headed dwarfism, mental deficiency, and skeletal and ophthalmic defects. We report a patient with this syndrome who demonstrated pigmentary changes, including streaks of brown pigmentation on the neck, groin, and axillae. Histologic examination revealed pigment incontinence.