NECROLYTIC MIGRATORY ERYTHEMA WITHOUT GLU CAGONOMA

Introduction. The glucagonoma syndrome is an uncommon but well-known e ntity associating erythema necroticans migrans (ENM) with glucagonoma. Case report. A 43-year-old man with a past history of alcoholic cirrh osis and ascitis was hospitalized for skin disorders which had develop ed over the past 4 months. Centrifugal erythematous skin lesions were observed, some with non-turgid bullae and marginal desquamation, other s with an erosive center. Lesions first appeared on the hands then dif fused widely without involving the periorifical areas, folds and lower limbs. Laboratory results revealed an anemia, hypovitaminosis K, chol estatic liver failure, a beta-gamma block and low zinc levels. Histolo gy study of the skin biopsy demonstrated a << Neapolitan trench >> ima ge suggestive of ENM. A paraneoplasic syndrome and pancreas tumor were not found. Despite supplementation with zinc, amino acids and vitamin s, the patient died from his liver disease. Discussion. There have bee n 4 cases of ENM reported in the literature, all in cirrhosis patients , two of which had low zinc levels. Our case is thus the third with ci rrhosis and low zinc. Bazer's syndrome, acrodermatitis enteropathica, annular chronic lupus erythematosis and annular superficial pemphigus were eliminated as possible diagnoses. The failure of zinc and amino a cid supplementation would favor the secondary nature of the zinc defic iency and the predominant role of cirrhosis in this skin disease. The possible role of essential fatty acids in ENM is raised.