Eduardo Weiss

Familial generalized perforating granuloma annulare

A 6-year-old boy and his 5-year-old sister had multiple skin-colored or lightly erythematous papules of firm consistency, measuring 0.5-3 mm in diameter. The lesions had a central umhilicated scale or crust and were located over the extensor surface of the hands, predominantly over the metacarpophalangeal and proximal interphalangeal joints (Figs. 1 and 2). Few lesions were also present over the elbows, knees, ankles, and popliteal regions, in a symmetric distribution! The girl had additional lesions located over the sternum, the nasal bridge, the cheeks, and both malar areas (Fig. 3). Historically, the lesions began to appear when both were approximately 1 year old and, besides being slightly pruritic, were for the most part asymptomatic. The patients had been treated with multiple topical medications, including imidazol creams, trichloroacetic acid, and cortlcosteroids of various potency, without success. When compressed, most umbilicated papules extruded a creamy material. Both children were the product of term gestation and uncomplicated delivery. There was a positive familial history of allergic rhinitis without history of diabetes mellitus. A 3-mm punch biopsy specimen was taken from one of the perforating papules of both children, located on the dorsal aspect of the hand. Both specimens showed almost identical histopathologic findings. The epidermis was atrophied with an area of parakeratosis over the central part where the epithelium was perforated. The perforating material was composed of collagen and cellular debris (Fig. 4). In the upper dermis, a well-circumscrlbed granulomatous infiltrate consisting of histiocytes, epithelioid cells, giant cells, and an intense focus of necrobiosis was observed next to the zone of perforation (Fig. 4). The necrobiotic areas contained a PAS-positive material, resistant to diastase digestion. The material stained positive with alcian blue at pH 2.5 and negative at pH 0.4. This, in conjunction with the positive colloidal iron and hyaluronidase labile stains, suggested the presence of hyaluronic acid in the necrobiotic zone. The orceine stain showed the absence of elastic fibers at the focus of the

American cutaneous Leishmaniasis : intermediate form

A man of Portuguese nationality presented with increased volume of the forehead and multiple pustules, which subsequently ulcerated. The pustules appeared progressively on the rest of the face and in the right scapular region during a period of 2 years.