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- Original article -
Bullous Systemic Lupus Erythematosus as an Initial Manifestation of SLE
Wataru Fujimoto, Toshihisa Hamada*, Jun Yamada*, Hironori Matsuura* and Keiji Iwatsuki*
Department of Dermatology, Kawasaki Medical School, Matsushima 577, Kurashiki-shi, Okayama 701-0192 Japan
*Department of Dermatology, Okayama University Graduate School of Medicine and Dentistry, 2-5-1, Shikata-cho, Okayama 700-8558 Japan
Bullous systemic lupus erythematosus (BSLE) is a rare subset of systemic lupus erythematosus that is often associated with autoimmunity to type VII collagen. We describe a 45-year-old woman with BSLE who presented with vesiculobullous lesions as an initial manifestation of SLE. The patient first noticed a widespread urticarial, erythematous eruption associated with tense blisters, erosions, and crusting. She was diagnosed with bullous pemphigoid and underwent a one-month course of treatment with betamethazone. Because of the appearance of marked proteinuria, a subsequent renal biopsy, and serological tests, the patient was diagnosed with rapidly progressive glomerulonephritis and systemic lupus erythematosus. The patient's IgG circulating antibodies labeled the dermal floor of salt-split skin and recognized type VII collagen in immunoblot studies. Although methylprednisolone pulse therapy for glomerulonephritis did not alleviate the vesicullobullous eruption, treatment with dapsone resulted in dramatic disappearance of the lesions. Cessation of dapsone therapy due to hemolysis with Heinz-body formation did not aggravate the bullous disease. Our case illustrates that a generalized vesiculobullous eruption can be the sole presenting manifestation of SLE. It also emphasizes the close temporal relationship between BSLE and lupus nephritis.
key words: bullous systemic lupus erythematosus; lupus nephritis; dapsone; type VII collagen; Heinz body
Received May 23, 2005; Accepted for publication June 27, 2005
Reprint requests to: Wataru Fujimoto, M.D., Department of Dermatology, Kawasaki Medical School, Matsushima 577, Kurashiki-shi, Okayama, 701-0192 Japan
J Dermatol 32 (12): 1021-1027, 2005
Japanese Dermatological Association
http://www.dermatol.or.jp
