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Dermatologic Manifestations of Wiskott-Aldrich Syndrome Treatment & Management

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

Medical Care

Transfusions of platelets and plasma decrease the risk of fatal hemorrhage in Wiskott-Aldrich syndrome patients.

Appropriate antibiotics should be used to treat bacterial infections. The antibiotics should be chosen based on the bacteria obtained and detected from the infected site.

Intravenous infusions of immune globulin decrease the risks of infection.

Infusion of transfer factor results in decreased frequency of infection and improvement of eczema.[9]

Bone marrow transplantation with HLA-identical marrow should be considered if patients have recurrent problems. Full engraftment results in normal platelet numbers and functions, immunologic status, and clearance of the eczema.[10]

Topical steroids may improve the eczema.

Gene therapy is promising for Wiskott-Aldrich syndrome.[11, 12, 13]

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Surgical Care

Splenectomy should be considered for Wiskott-Aldrich syndrome patients with thrombocytopenia who have no HLA-matched bone marrow transplantation donor.[10, 14, 15]

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Consultations

Possible consultations include a pediatrician and/or oncologist.

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Diet

In patients with food allergies, diet control must be considered.

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Contributor Information and Disclosures
Author

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; .

References
  1. Derry JM, Ochs HD, Francke U. Isolation of a novel gene mutated in Wiskott-Aldrich syndrome. Cell. 1994 Aug 26. 78(4):635-44. [Medline].

  2. Guillén-Rocha N, López-Rocha E, Danielian S, Segura-Méndez N, López-González L, Lugo-Reyes SO. [Wiskott-Aldrich syndrome. A report of a new mutation]. Rev Alerg Mex. 2014 Jul-Sep. 61(3):219-23. [Medline].

  3. Perry GS 3rd, Spector BD, Schuman LM, et al. The Wiskott-Aldrich syndrome in the United States and Canada (1892-1979). J Pediatr. 1980 Jul. 97(1):72-8. [Medline].

  4. Conley ME, Wang WC, Parolini O, Shapiro DN, Campana D, Siminovitch KA. Atypical Wiskott-Aldrich syndrome in a girl. Blood. 1992 Sep 1. 80(5):1264-9. [Medline].

  5. Silvin C, Belisle B, Abo A. A role for Wiskott-Aldrich syndrome protein in T-cell receptor-mediated transcriptional activation independent of actin polymerization. J Biol Chem. 2001 Jun 15. 276(24):21450-7. [Medline].

  6. Pulecio J, Tagliani E, Scholer A, et al. Expression of Wiskott-Aldrich syndrome protein in dendritic cells regulates synapse formation and activation of naive CD8+ T cells. J Immunol. 2008 Jul 15. 181(2):1135-42. [Medline].

  7. Jia D, Gomez TS, Metlagel Z, Umetani J, Otwinowski Z, Rosen MK, et al. WASH and WAVE actin regulators of the Wiskott-Aldrich syndrome protein (WASP) family are controlled by analogous structurally related complexes. Proc Natl Acad Sci U S A. 2010 Jun 8. 107(23):10442-7. [Medline]. [Full Text].

  8. Yamada M, Ariga T, Kawamura N, et al. Determination of carrier status for the Wiskott-Aldrich syndrome by flow cytometric analysis of Wiskott-Aldrich syndrome protein expression in peripheral blood mononuclear cells. J Immunol. 2000 Jul 15. 165(2):1119-22. [Medline].

  9. Sellars WA, South MA. Wiskott-Aldrich syndrome with 18-year survival. Treatment with transfer factor. Am J Dis Child. 1975 May. 129(5):622-7. [Medline].

  10. Mullen CA, Anderson KD, Blaese RM. Splenectomy and/or bone marrow transplantation in the management of the Wiskott-Aldrich syndrome: long-term follow-up of 62 cases. Blood. 1993 Nov 15. 82(10):2961-6. [Medline].

  11. Dupre L, Marangoni F, Scaramuzza S, et al. Efficacy of gene therapy for Wiskott-Aldrich syndrome using a WAS promoter/cDNA-containing lentiviral vector and nonlethal irradiation. Hum Gene Ther. 2006 Mar. 17(3):303-13. [Medline].

  12. Boztug K, Schmidt M, Schwarzer A, Banerjee PP, Díez IA, Dewey RA, et al. Stem-cell gene therapy for the Wiskott-Aldrich syndrome. N Engl J Med. 2010 Nov 11. 363(20):1918-27. [Medline]. [Full Text].

  13. Williams DA. Curing genetic disease with gene therapy. Trans Am Clin Climatol Assoc. 2014. 125:122-9. [Medline]. [Full Text].

  14. Corash L, Shafer B, Blaese RM. Platelet-associated immunoglobulin, platelet size, and the effect of splenectomy in the Wiskott-Aldrich syndrome. Blood. 1985 Jun. 65(6):1439-43. [Medline].

  15. Syrigos KN, Makrilia N, Neidhart J, Moutsos M, Tsimpoukis S, Kiagia M, et al. Prolonged survival after splenectomy in Wiskott-Aldrich syndrome: a case report. Ital J Pediatr. 2011 Sep 10. 37:42. [Medline]. [Full Text].

  16. Cotelingam JD, Witebsky FG, Hsu SM, Blaese RM, Jaffe ES. Malignant lymphoma in patients with the Wiskott-Aldrich syndrome. Cancer Invest. 1985. 3(6):515-22. [Medline].

  17. Notarangelo LD, Mori L. Wiskott-Aldrich syndrome: another piece in the puzzle. Clin Exp Immunol. 2005 Feb. 139(2):173-5. [Medline].

  18. Tsuji Y, Imai K, Kajiwara M, et al. Hematopoietic stem cell transplantation for 30 patients with primary immunodeficiency diseases: 20 years experience of a single team. Bone Marrow Transplant. 2006 Mar. 37(5):469-77. [Medline].

 
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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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