Abstract
The concurrence of DH and SLE has previously been reported in 2 patients. Our patient is a 15 year old black girl with Marfan's Syndrome who developed a bultous eruption which was clinically, histologically, and immunopathologically characteristic of DH. Sulfapyridine was given. Two months later she developed fever, pleural effusion, renal insufficiency, nephrotic syndrome, hypertension, hypocomplementemia, positive ANIF and positive LE preparation. Immunofluorescence of uninvolved skin demonstrated IgG, IgM and C3 at dermal-epidermal junction and around papillary blood vessels. Kidney biopsy showed Type III (proliferation with deposits) lupus glomerulonephritis. Prednisone treatment resulted in improvement of clinical manifestations. She subsequently expired when cystic medial necrosis of the ascending aorta resulted in cardiac tamponade. Since both DH and SLE are diseases mediated by the deposition of immune complexes, our patient clearly exhibited a rare predisposition to formation of immune complexes. The presence of two immune-mediated diseases in the same patient suggests a common underlying immune defect.
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Aronson, A., Soltani, K., Ong, R. et al. SYSTEMIC LUPUS ERYTHEMATOSUS (SLE) AND DERMATITIS HERPETIFORMIS (OH) IN A GIRL WITH MARFAN'S SYNDROME. Pediatr Res 11, 484 (1977). https://doi.org/10.1203/00006450-197704000-00684
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DOI: https://doi.org/10.1203/00006450-197704000-00684