Mucha-Habermann disease: a diagnostic possibility for prolonged fever associated with systemic and skin symptoms

The severe form of Mucha-Habermann disease with systemic symptoms is a rarely diagnosed disease which should be considered for children with prolonged fever, impaired general condition, skin manifestations and elevated C-reactive protein concentration and/or erythrocyte sedimentation rate. Eleven cases have been described previously in children. We describe two acute episodes of this syndrome in a three-year-old child; the diagnosis was based on clinical, dermatological and histological findings. During both episodes, the fever lasted for more than one week, C-reactive protein concentration increased to more than 150 mg/l, and there was extensive lymph node enlargement. Skin eruption was initially maculopapulous, then vesiculous and finally pustulous. On skin biopsy, vasculitic changes were observed. We treated the second attack of our patient with high-dose gamma globulin; the first attack appeared to resolve itself spontaneously.