Response of cutaneous Rosai-Dorfman disease to topical and intralesional steroids.
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Biomedical subjects
Publications and source records attributed to J W Steger.
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Multiple endocrine neoplasia type 1 (MEN 1) predisposes affected persons to neoplasms of the parathyroid glands, the endocrine pancreas, the anterior pituitary, and the duodenum. We report the first case of adult-onset multiple angiofibromas of the central face associated with MEN 1. Seven siblings also developed adult-onset angiofibromas, none with evidence of tuberous sclerosis. Basic fibroblast growth factor (BFGF) is elevated in many patients with MEN 1 and may play a pathogenetic role.
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Zinc is an essential trace element whose malabsorption in early childhood may result in a skin disorder known as acrodermatitis enteropathica. Cutaneous lesions typical of acrodermatitis enteropathica have been described during total parenteral nutrition on zinc-deficient intravenous solutions in both adults and children. This condition has been named the "acute zinc depletion syndrome." A case is described in which a patient, despite a zinc intake of double the daily requirement, manifested the acute zinc depletion syndrome during therapy with combined liquid diet plus parenteral hyperalimentation. Predisposing factors in this individual included a short bowel syndrome and a large oral load of calcium lactate. Zinc metabolism is reviewed with attention to alterations in disease and during hyperalimentation. The clinical manifestations, predisposing factors, therapy and prevention of the acute zinc depletion syndrome are discussed.