Hautmanifestationen beim hypereosinophiliesyndrom (HES) - Klinik, therapie und pathophysiologie

The hypereosinophilic syndrome is a multisystem disease, characterized by blood and bone marrow infiltration as well as eosinophilic infiltration of various organ systems. Skin lesions occur frequently in HES. Thereby the release of toxic eosinophil products leads to organ damage. HES is an exclusion diagnosis, other eosinophil-associated illnesses have to be excluded. The disease is heterogenous and varies in organ damage, response to therapy and etiology. A short review of the literature concerning symptoms, therapy and pathophysiology is given and 9 patients with HES and polymorphous skin manifestations are presented. Patients' IgE levels and the eosinophilic cationic protein in serum as well as in skin lesions were increased. There was no correlation between blood eosinophilia and degree of organ involvement. Immunophenotyping of peripheral lymphocytes with FACS analysis revealed significant T-cell activation.