Current Allergy and Asthma Reports

, Volume 12, Issue 2, pp 85–98

Clinical Overview of Cutaneous Features in Hypereosinophilic Syndrome

  • Sabine Gisela Plötz
  • Bettina Hüttig
  • Birgit Aigner
  • Christian Merkel
  • Knut Brockow
  • Cezmi Akdis
  • Ulf Darsow
  • Johannes Ring
Invited Commentary

DOI: 10.1007/s11882-012-0241-z

Cite this article as:
Plötz, S.G., Hüttig, B., Aigner, B. et al. Curr Allergy Asthma Rep (2012) 12: 85. doi:10.1007/s11882-012-0241-z

Abstract

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.

Keywords

Hypereosinophilic syndrome (HES)CutaneousUrticariaAngioedemaEczemaImmunologic diseasesEosinophilia

Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  • Sabine Gisela Plötz
    • 1
    • 2
  • Bettina Hüttig
    • 1
  • Birgit Aigner
    • 2
  • Christian Merkel
    • 1
  • Knut Brockow
    • 1
  • Cezmi Akdis
    • 3
  • Ulf Darsow
    • 1
  • Johannes Ring
    • 1
    • 4
  1. 1.Department of Dermatology and Allergology am BiedersteinTechnical University Munich (TUM)MunichGermany
  2. 2.Dermatology Munich-HarlachingMunichGermany
  3. 3.Swiss Institute for Allergy and Asthma Research (SIAF)DavosSwitzerland
  4. 4.Christine Kühne Center for Allergy Research, Education (CK-CARE)Technical University MunichMunichGermany