Systemic lupus erythematosus in a boy with chronic granulomatous disease: Case report and review of the literature

We describe a patient with X‐linked chronic granulomatous disease (CGD) who developed systemic lupus erythematosus, which was characterized by photosensitivity, malar rash, glomerulonephritis, leukopenia, hypocomplementemia, antinuclear antibodies, and anti–double‐stranded DNA antibodies, at age 3....

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Bibliographic Details
Published in:Arthritis and rheumatism 1991-01, Vol.34 (1), p.101-105
Main Authors: Manzi, Susan, Urbach, Andrew H., McCune, Ann B., Altman, Harold A., Kaplan, Sandra S., Medsger, Thomas A., Ramsey‐Goldman, Rosalind
Format: Article
Language:English
Subjects:
Biological and medical sciences
Child, Preschool
Granulomatous Disease, Chronic - complications
Granulomatous Disease, Chronic - genetics
Heterozygote
Humans
Immunodeficiencies
Immunodeficiencies. Immunoglobulinopathies
Immunopathology
Infant
Lupus Erythematosus, Systemic - complications
Male
Medical sciences
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Summary:We describe a patient with X‐linked chronic granulomatous disease (CGD) who developed systemic lupus erythematosus, which was characterized by photosensitivity, malar rash, glomerulonephritis, leukopenia, hypocomplementemia, antinuclear antibodies, and anti–double‐stranded DNA antibodies, at age 3. The patient's mother is an asymptomatic carrier of CGD, and her other son (the patient's half‐brother) also has CGD. Neither the mother nor the brother has clinical or serologic evidence of systemic lupus erythematosus. Previous cases of discoid lupus–like skin lesions have been reported both in carriers and in patients with CGD. Our patient represents the first reported case of an individual with convincing clinical, serologic, and pathologic evidence of systemic lupus erythematosus. The association between defective host defense mechanisms and autoimmune phenomena has been described previously in patients with Job's syndrome and in patients with B cell and T cell deficiency disorders, including the acquired immunodeficiency syndrome. The relationship between the known leukocyte defects in CGD and the pathogenesis of a lupus‐like illness is unclear.
ISSN:0004-3591
1529-0131
DOI:10.1002/art.1780340116