Dermatologic Manifestations of Wiskott-Aldrich Syndrome Medication

Updated: Aug 13, 2019
  • Author: Akimichi Morita, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
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Medication

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.

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Immunoglobulins

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.

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Corticosteroids

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.

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