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Dermatologic Manifestations of Wiskott-Aldrich Syndrome Medication

  • Author: Akimichi Morita, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
Updated: Sep 05, 2014

Medication Summary

Agents for Wiskott-Aldrich syndrome medical therapy are selected based on clinical presentation and response. When treating infections, if possible, identify the suspected pathogen before selecting antibiotics. Antibiotics are indicated to treat bacterial infections and for prophylaxis in patients who have had a splenectomy. Immunoglobulins and systemic corticosteroids are indicated to treat thrombocytopenia. Use topical steroids to treat eczema. Topical steroids are effective for the eczematous lesion.



Class Summary

These agents provide functional immunoglobulins in patients whose ability to respond to bacterial antigens is abnormal, and they may inhibit platelet sequestration by the reticuloendothelial system.

Immune globulin (Gamimune N, Gammagard S/D, Sandoglobulin)


Immune globulin is used to treat thrombocytopenia; it may be indicated if the serum IgG level is low or the patient cannot produce functional antibody responses (eg, to polysaccharide antigens). Little data support routine use for immune defects in WAS in the absence of low serum IgG levels.



Class Summary

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli.

Hydrocortisone topical (Dermacort, CortaGel, Cortaid, Westcort)


Topical hydrocortisone is an adrenocorticosteroid derivative suitable for application to skin or external mucous membranes. It has mineralocorticoid and glucocorticoid effects, resulting in anti-inflammatory activity. It is used to treat eczema.

Prednisone (Deltasone)


Prednisone is an immunosuppressant for the treatment of autoimmune disorders; it may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Prednisone stabilizes lysosomal membranes and suppresses lymphocyte and antibody production.

Contributor Information and Disclosures

Akimichi Morita, MD, PhD Professor, Chairman, Department of Dermatology, Nagoya City University Medical School, Japan

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Jacek C Szepietowski, MD, PhD Professor, Vice-Head, Department of Dermatology, Venereology and Allergology, Wroclaw Medical University; Director of the Institute of Immunology and Experimental Therapy, Polish Academy of Sciences, Poland

Disclosure: Received consulting fee from Orfagen for consulting; Received consulting fee from Maruho for consulting; Received consulting fee from Astellas for consulting; Received consulting fee from Abbott for consulting; Received consulting fee from Leo Pharma for consulting; Received consulting fee from Biogenoma for consulting; Received honoraria from Janssen for speaking and teaching; Received honoraria from Medac for speaking and teaching; Received consulting fee from Dignity Sciences for consulting; .

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Eczematous lesions in Wiskott-Aldrich syndrome. The lesion is essentially indistinguishable from that of atopic dermatitis except for the presence of purpura and petechiae.
A bloody crust can be seen on the red papules.
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