AIDS and the dermatologist. A perspective.
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Biomedical subjects
Publications and source records attributed to N S Prose.
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Five children, aged 3 to 11 years, developed a distinctive perioral, perinasal, and periorbital rash, consisting of tiny, closely spaced, flesh-colored "micronodules." Histopathologic examination in all five cases revealed upper dermal and perifollicular granulomas admixed with lymphocytes. There were no associated systemic abnormalities. The lesions resolved after months to years, leaving no scars. We propose that this condition is a form of perioral dermatitis with granulomatous histologic features, which can be distinguished from sarcoidosis and other facial eruptions in childhood both on clinical and histologic grounds.
The diagnosis and treatment of disorders of the skin and subcutaneous tissue represents a significant aspect of general pediatric practice. A study was carried out to assess the status of training in dermatology among residents in pediatrics. Sixty five pediatric residents in seven different training programs were asked to identify color transparencies of 20 common dermatoses. Their mean score of 53.2 percent was considerably lower than that of an equivalent group of residents in dermatology (86.4%). A survey of pediatric residencies throughout the United States indicated that 67 percent of those responding offer an elective in pediatric dermatology.
We examined a patient with systemic lupus erythematosus and sepsis due to Pseudomonas aeruginosa. Early in the infection, she developed skin lesions that consisted of indurated tender nodules and hemorrhagic and nonhemorrhagic bullae. Blister fluid contained gram-negative rods, which were identified as P. aeruginosa on culture. Bullae and nodules, as well as ecthyma gangrenosum, can be early cutaneous signs of pseudomonal sepsis.
Acquired immunodeficiency syndrome was first observed in children in 1982. Human immunodeficiency virus (HIV) is now known to be the etiologic agent of this disease complex. Children acquire the viral infection in utero or perinatally, or by receiving contaminated blood products. The cutaneous manifestations include persistent oral thrush, herpes simplex, herpes zoster, molluscum contagiosum, and a variety of fungal and bacterial skin infections. Vasculitis, unusual drug eruptions, and cutaneous manifestations of nutritional deficiencies are also seen.
A 6-week-old girl with congenital melanoma of the skin is reported and the literature is reviewed. We believe this to be the first case of congenital melanoma in a black infant who had no underlying giant melanocytic nevus. Because of the significant relationship between the early treatment and prognosis of malignant melanoma, we wish to alert pediatric clinicians to the existence of this rare congenital neoplasm.
We examined two patients with facial lesions of fixed cutaneous sporotrichosis. That condition should be considered in the differential diagnosis of persistent nodules or plaques in this anatomic location.
Multiple benign juvenile melanoma is a rare entity, seen primarily in children. Two 3-year-old patients who developed multiple juvenile melanomas in areas of congenital hyperpigmentation are presented. The distinct clinical presentations of this lesion are discussed, and its ultrastructure is described.
The clinical findings in eight young homosexual men in New York with Kaposi's sarcoma showed some unusual features. Unlike the form usually seen in North America and Europe, it affected younger men (4th decade rather than 7th decade); the skin lesions wee generalised rather than being predominantly in the lower limbs, and the disease was more aggressive (survival of less than 20 months rather 8-13 years). All eight had had a variety of sexually transmitted diseases. All those tested for cytomegalovirus antibodies and hepatitis B surface antigen of anti-hepatitis B antibody gave positive results. This unusual occurrence of Kaposi's sarcoma in a population much exposed to sexually transmissible diseases suggests that such exposure may play a role in its pathogenesis.
A 51-year-old man with Hailey-Hailey disease was treated with a split thickness skin graft of the left axilla. The dramatic improvement in the grafted area is contrasted with the continued disease activity in the untreated axilla and other intertriginous areas. Local excision and grafting, with a resultant decrease in sweating and maceration, is an effective form of therapy for Hailey-Hailey disease.
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Two infants, one with a T-cell-signaling defect resulting in a primary immunodeficiency syndrome and the other with severe combined immunodeficiency (SCID), are described. Both infants developed cutaneous infections secondary to their bacillus Calmette-Guérin (BCG) vaccinations. Both patients were from countries where BCG is routinely administered in infancy. The infant with the T-cell-signaling defect developed a disseminated infection involving the skin, while the infant with SCID developed a localized cutaneous infection at the site of his BCG immunization. These two cases resemble other reported cases of cutaneous BCG infection following routine vaccination in immunocompromised patients. Mycobacterium bovis infection should be considered in patients with cutaneous eruptions who have received BCG vaccination, especially those who are immunocompromised.
Waxy keratoses of childhood is a rare genodermatosis previously noted in both familial and isolated presentations. Three previous cases have been described in which the lesions appeared over the trunk and extremities. We report a case in which the waxy keratoses of childhood lesions appeared in a segmental distribution along a single lower extremity, differing from the more extensive patterns reported previously. We suggest a possible postzygotic mutation hypothesis for this unique segmental distribution, addressing both the possibility of a genomic mosaicism and loss of heterozygosity.
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