You are here

  • Home
  • Archive
  • Volume 98, Issue 4
  • Cutaneous manifestations in patients with Wiskott–Aldrich syndrome submitted to haematopoietic stem cell transplantation

Article Text

Article menu
Download PDFPDF
Original article
Cutaneous manifestations in patients with Wiskott–Aldrich syndrome submitted to haematopoietic stem cell transplantation
  1. Juliana Gomes Loyola Presa1,
  2. Vania Oliveira de Carvalho2,
  3. Laura Rogers Morrisey3,
  4. Carmem Maria Bonfim4,
  5. Kerstin Taniguchi Abagge2,
  6. Angélica Vasselai5,
  7. Leide Parolin Marinoni2
  1. 1Division of Pediatric Dermatology, Federal University of Paraná, Hospital de Clínicas da Universidade Federal do Paraná, Curitiba, Paraná, Brazil
  2. 2Division of Pediatric Dermatology, Department of Pediatrics, Federal University of Paraná, Hospital de Clínicas da Universidade Federal do Paraná, Curitiba, Paraná, Brazil
  3. 3Department of Dermatology, Michael G. DeGroote School of Medicine, McMaster University, Hamilton, Ontario, Canada 
  4. 4Division of Bone Marrow Transplantation, Federal University of Paraná, Hospital de Clínicas da Universidade Federal do Paraná, Curitiba, Paraná, Brazil
  5. 5Department of Pediatrics, Federal University of Paraná, Hospital de Clínicas da Universidade Federal do Paraná, Curitiba, Paraná, Brazil
  1. Correspondence to Vania Oliveira de Carvalho, Division of Pediatric Dermatology, Department of Pediatrics, Federal University of Paraná, Hospital de Clínicas da UFPR, Rua General Carneiro 181, 14 Andar, Rua Richard Strauss 62, Vista Alegre, Curitiba, Paraná CEP 80.820-110, Brazil; rcarvalho50{at}hotmail.com, jgloyola{at}hotmail.com

Abstract

Introduction Wiskott–Aldrich syndrome (WAS) is an X-linked primary immunodeficiency caused by a mutation of the WAS protein  gene. This protein actively participates in important cellular processes, and its presence is related to diverse clinical manifestations, including cutaneous alterations. The classical triad of WAS consists of recurrent infections, thrombocytopaenia with small platelets and atopic dermatitis (AD)-like  lesions.

Objective To evaluate the frequencies of cutaneous manifestations in patients with WAS prior to haematopoietic stem cell transplantation (HSCT).

Results Twenty-four boys diagnosed with WAS and treated with HSCT between 1992 and 2007 were included. The characteristic triad of WAS occurred in 46% of patients. Before HSCT, the most frequent cutaneous manifestations included eczema similar to AD (71%), followed by petechiae and/or ecchymosis (58%) and cutaneous infections (17%).

Conclusions Cutaneous manifestations in patients with WAS are frequent, especially those similar to the eczema found in AD.

  • Dermatology
  • Immunology

https://doi.org/10.1136/archdischild-2011-300812

Statistics from Altmetric.com

    Request Permissions

    If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

    View Full Text