Langerhans-cell-histiozytose bei einem säugling unter dem bild einer varizelleninfektion

Background. Langerhans-cell-histiocytosis is a proliferative disease of Langerhans-cells of uncertain etiology. Its low incidence and a variable clinical presentation, imitating other diseases often lead to delay of diagnosis and treatment. Case report. We report on an ad admission 6 weeks old boy. He had had contact to a varicella incubated child and three weeks later he suffered from a varicella-like rash. The polymorphic rash consisted of papulae, vesicles, pustles and petechiae. In addition hepatomegaly, splenomegaly, dyspnoe, fever, thrompopenia and anaemia were present. Skin biopsy revealed infiltrates of proliferating Langerhans-cells expressing CD1a and S100. Typical Birbeck-granules were shown by electron microscopy. Thus diagnosis of disseminated Langerhans-cell-histiocytosis was established. Discussion. Langerhans-cell-histiocytosis should be considered in severely affected newborns and infants demonstrating polymorhic rash. Persistent unidentified skin lesions require a skin biopsy.