Eruptive vellus hair cysts.

Umbilicated and nonumbilicated papules were present on the presternal area of a patient with eruptive vellus hair cysts. Histopathological examination of umbilicated papules showed penetration of vellus hairs through cyst walls, surrounding granulomatous foreign body reaction, and communication to the surface of the skin with released vellus hairs present in the pore. This suggests that resolution of some of the cysts in this disorder may occur by transepithelial elimination.

Combined adnexal tumor of the skin.

Two patients had distinctive adnexal skin tumors that showed cellular differentiation toward the formation of more than one adnexal structure. In one patient, the tumor showed differentiation toward pilar and sweat ductal structures. In the second patient, tumor differentiation toward the formation of sebaceous glands, pilar, and sweat ductal structures was found. We propose the term "combined adnexal tumor of the skin" for this neoplasm.

Erthropoietic protoporphyria: first report of cases in the American Nergo.

The first two cases of erthropoietic protoporphyria in the American Nergo are reported. The eruption was limited to the lips, subungual and periungual areas, which are relatively nonpigmented. Onset was at a later age and length of sun exposure required to produce symptoms was longer than that in most whites with the disease. The different presentation in Negroes is believed to be related to their increased pigmentation. Melanin appears to provide protection because of its free radical formation and absorption of 400 nm electromagnetic radiation.

Spitz's nevus in a black child.

A Spitz's nevus is described in an 11-year-old black girl. This is only the second such report in the medical literature. The difficulty in clinically and histologically differentiating this lesion from the rare malignant melanoma in blacks is illustrated by this case.

Genetic counseling in segmental neurofibromatosis.

We report two patients with segmental neurofibromatosis and review the literature with regard to possible hereditary transmission of this disorder. Patients that meet strict criteria for the diagnosis of segmental neurofibromatosis seem to have a low probability of transmitting the disease. We emphasize the importance of establishing and strictly adhering to a set of diagnostic criteria and of obtaining a comprehensive family history when reporting cases of segmental neurofibromatosis.

Autosomal dominant granulomatous arthritis, uveitis, skin rash, and synovial cysts.

In 1985, Blau reported a family with 11 members in four generations affected by granulomatous arthritis, iritis, skin rash, and periarticular synovial cysts. We report a second family with these abnormalities, thereby confirming this syndrome as a distinct familial entity with transmission compatible with autosomal dominant inheritance. Affected members in our family included a mother and two daughters. Disease onset was at 10 months to 8 years of age. Each had uveitis, symmetric polyarthritis, and synovial cysts overlying the ankle and wrist joints. In addition, both daughters had an intermittent generalized erythematous papular rash that on biopsy revealed noncaseating granulomatous infiltration. All three patients improved during alternate-day steroid therapy. Recognition of this disorder as distinct from other, more common causes of arthritis is important because of the apparent autosomal dominant transmission and because of the excellent responses to low-dose steroid therapy.