Necrolytic migratory erythema. Distinctive dermatosis of the glucagonoma syndrome.
Glucagon-secreting tumors of the pancreatic islets (glucagonomas) produce a distinctive syndrome in which weight loss, diabetes mellitus, anemia,and prominent mucocutaneous findings occur. The cutaneous component-necrolytic migratory erythema--may be polymorphous, but most commonly manifests as erosions and crusts of the groin, perineum, buttocks, distal part of the extremities, and central area of the face. Alternatively, scaly papules and plaques may predominate in these areas. The eruption may resemble such dermatoses as pemphigus foliaceus, acrodermatitis enteropathica, chronic mucocutaneous candidiasis, psoriasis, and severe seborrheic dermatitis. Two patients with chronic, previously undiagnosed dermatoses had necrolytic migratory erythemia, which led to the discovery of glucagonomas present in each. In one patient surgical resection of the tumor resulted in total clearing of the rash within 48 hours. Awareness of this distinctive entity may lead to early diagnosis and, possibly, cure.
D007516:Adenoma, Islet Cell / Q000150:complications / Q000378:metabolism* / Q000473:pathology; D000328:Adult; D003937:Diagnosis, Differential; D004890:Erythema / Q000175:diagnosis / Q000209:etiology*; D005260:Female; D005934:Glucagon / Q000378:metabolism*; D006801:Humans; D007515:Islets of Langerhans / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010190:Pancreatic Neoplasms / Q000150:complications / Q000378:metabolism* / Q000473:pathology; D012871:Skin Diseases / Q000175:diagnosis; D013577:Syndrome
D005934:Glucagon