Omenn syndrome: familial reticulo-endotheliosis with eosinophilia and combined immunodeficiency.

This article discusses omenn syndrome in a male patient aged one and half year.In the first months of life this baby showed a skin eruption of the scalp suggestive of a seborrhoeic dermatitis. From the age of i year a widespread pruritic skin disorder developed with fever, lymphadenopathy, anaemia and cosinophilia. Topical steroids were not effective. From the age of 14 months chronic diarrhoea was present. Biopsy from a nodule showed acanthosis, an oedematous papillary layer and in the lower part of the dermis a dense infiltrate consisting of histiocytes, lymphocytes, eosinophils and mast cells.