Gianotti-Crosti syndrome associated with endogenous reactivation of Epstein-Barr virus.
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BACKGROUND: The spectrum of Dowling-Degos disease-reticulate acropigmentation of Kitamura (DDD-RAK) is a group of rare autosomal dominant disorders that have in common a unique histological picture of hyperpigmented digitate epidermal 'downgrowths'. Patients with the DDD-RAK spectrum may show hyperpigmented macules and papules, facial pits, breaks in dermatoglyphics and epidermoid cysts. OBSERVATIONS: We examined 5 unrelated patients, 3 females and 2 males (age range 22-35 years), who presented with clinical and histological features of the DDD-RAK spectrum. In addition, the patients presented with hypo- or depigmented macules and papules. Histopathology of the lesions revealed features that were identical to DDD-RAK; there were, however, diminution or absence of pigmentation. Family histories for pigmented lesions and leukoderma were positive in all patients and consistent with autosomal dominant modes of inheritance. CONCLUSION: These 5 cases, together with isolated reports in the literature of achromic lesions with histological features of DDD-RAK, point to the hypothesis that achromic macules and papules may be a feature of the DDD-RAK spectrum.
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A case of acro-angiodermatitis with congenital arteriovenous fistulae (Stewart-Bluefarb syndrome) at the distal right foot in a young man is reported. The lesion evolved slowly from a small dark-red spot existing from birth. Besides mechanical disturbances, the patient's only symptoms were a warmer and somewhat bigger right foot compared to the left nonaffected side. He presented a circumscribed spongy, livid-red coloured angiomatous lesion at the upper part of the distal right foot, also involving the proximal parts of the three middle toes. The diagnosis was based on the clinical aspect and the findings by Doppler ultrasonographic and oscillographic examination. The transcutaneous oxygen pressure showed a marked hypoxia at the edge of the acro-angiodermatitis. Finally, arteriovenous fistulae were demonstrated by arteriography.
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BACKGROUND AND OBJECTIVE: No complete review of the cutaneous manifestations of eating disorders exists. We therefore, set out to review and systematically describe the clinical and histopathologic features of dermatologic conditions associated with anorexia nervosa, bulimia nervosa, and obesity. Differential diagnosis, pathophysiology, laboratory studies, and treatment are also reviewed. METHODS: Index Medicus review (1966 to present) using Ovid-MEDLINE. Search terms included eating disorders, anorexia nervosa, bulimia nervosa, eating disorders not otherwise specified (ED-NOS), and obesity, as well as the terms dermatology skin and cutaneous manifestations, with cross-referencing sources. These were combined with our own clinical experience. All relevant publications, including case reports, case series, cohort studies, and histopathologic studies giving at least Level II-3 evidence (evidence from comparisons between times or places with or without the intervention, including dramatic results in uncontrolled experiments), were selected. CONCLUSION: Forty dermatological signs have been reported in eating disorder patients. Eating disorders have many cutaneous manifestations and cutaneous signs may lead to the diagnosis of an occult eating disorder. The resolution of skin eruptions in eating disorder patients often depends on treatment of the underlying disorder.
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The Gianotti-Crosti syndrome is an infrequently recognized disorder with distinctive characteristics. The eruption, which lasts for two to eight weeks, consists of large, flat-topped, nonpruritic papules on the face, buttocks, and limbs. Its onset may be preceded by fever and upper respiratory tract symptoms. Associated findings include generalized lymphadenopathy, anicteric hepatitis, and HBs antigenemia. Two children with the syndrome are described to bring this entity to the attention of pediatricians.