Cutaneous manifestations in patients with Wiskott-Aldrich syndrome submitted to haematopoietic stem cell transplantation.
BACKGROUND
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.
Division of Pediatric Dermatology, Federal University of Paraná, Hospital de Clínicas da Universidade Federal do Paraná, Curitiba, Paraná, Brazil.
Juliana Gomes
JG
Loyola Presa
Vania Oliveira
VO
de Carvalho
Laura Rogers
LR
Morrisey
Carmem Maria
CM
Bonfim
Kerstin Taniguchi
KT
Abagge
Angélica
A
Vasselai
Leide Parolin
LP
Marinoni
D002675:Child, Preschool; D004485:Eczema / Q000209:etiology*; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D013921:Thrombocytopenia / Q000209:etiology*; D014923:Wiskott-Aldrich Syndrome / Q000150:complications* / Q000628:therapy