The hypereosinophilic syndromes (HES) are a heterogeneous group of disorders defined as persistent and marked blood eosinophilia of unknown origin with systemic organ involvement. HES is a potentially severe multisystem disease associated with considerable morbidity. Skin involvement and cutaneous findings frequently can be seen in those patients. Skin symptoms consist of angioedema; unusual urticarial lesions; and eczematous, therapy-resistant, pruriginous papules and nodules. They may be the only obvious clinical symptoms. Cutaneous features can give an important hint to the diagnosis of this rare and often severe illness. Based on advances in molecular and genetic diagnostic techniques and on increasing experience with characteristic clinical features and prognostic markers, therapy has changed radically. Current therapies include corticosteroids, hydroxyurea, interferon-α, the tyrosine kinase inhibitor imatinib mesylate, and (in progress) the monoclonal anti-interleukin-5 antibodies. This article provides an overview of current concepts of disease classification, different skin findings, and therapy for HES.