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Lupus erythematosus‐like lesions in a carrier of X‐linked chronic granulomatous disease
Author(s) -
CórdobaGuijarro S,
Feal C,
Daudén E,
Fraga J,
GarcíaDíez A
Publication year - 2000
Publication title -
journal of the european academy of dermatology and venereology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.655
H-Index - 107
eISSN - 1468-3083
pISSN - 0926-9959
DOI - 10.1046/j.1468-3083.2000.00113.x
Subject(s) - medicine , chronic granulomatous disease , pathology , lupus erythematosus , immunology , antibody
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.