Journal article
Systemic lupus erythematosus in a boy with chronic granulomatous disease: Case report and review of the literature
Arthritis and rheumatism, v 34(1), pp 101-105
Jan 1991
PMID: 1984766
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Abstract
Abstract
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.
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Details
- Title
- Systemic lupus erythematosus in a boy with chronic granulomatous disease: Case report and review of the literature
- Creators
- Susan Manzi - University of PittsburghAndrew H. Urbach - University of PittsburghAnn B. McCune - University of PittsburghHarold A. Altman - University of PittsburghSandra S. Kaplan - University of PittsburghThomas A. Medsger - University of PittsburghRosalind Ramsey-Goldman - University of Pittsburgh
- Publication Details
- Arthritis and rheumatism, v 34(1), pp 101-105
- Publisher
- Wiley
- Number of pages
- 5
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- General Internal Medicine
- Web of Science ID
- WOS:A1991ER53700015
- Scopus ID
- 2-s2.0-0025959088
- Other Identifier
- 991021934003904721
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InCites Highlights
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- Web of Science research areas
- Rheumatology