Lupus erythematosus-like lesions in a carrier of X-linked chronic granulomatous disease.
J Eur Acad Dermatol Venereol
; 14(5): 409-11, 2000 Sep.
Article
en En
| MEDLINE
| ID: mdl-11305387
Chronic granulomatous disease (CGD) is an inherited immunodeficiency disease. Carrier status of CGD has been reported in association with lupus erythematosus-type lesions. A 35-year-old woman, mother of a child with X-linked CGD presented an 8-year history of erythematous plaques with an arciform pattern on the upper trunk, back and arms. The nitroblue tetrazolium test revealed the carrier status of the patient. Haematological, biochemical and immunological tests (including ANA, DNA, SSA-Ro, SSB-La, RNP, SM and Jo1 antibodies) were normal or negative except for a polyclonal hypergammaglobulinaemia with high serum IgA. Histological examination showed a papillary and perifollicular lymphohistiocytic infiltrate. Direct immunofluorescence was negative. We report a female carrier of X-linked CGD who developed clinical subacute lupus erythematosus-like lesions. We review the literature and discuss the pathogenetic mechanisms involved in the condition.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Lupus Eritematoso Cutáneo
/
Enfermedad Granulomatosa Crónica
Límite:
Adult
/
Female
/
Humans
Idioma:
En
Revista:
J Eur Acad Dermatol Venereol
Asunto de la revista:
DERMATOLOGIA
/
DOENCAS SEXUALMENTE TRANSMISSIVEIS
Año:
2000
Tipo del documento:
Article
País de afiliación:
España
Pais de publicación:
Reino Unido