Authors: Grace Monica P. Ibaviosa, MD; Johanna O. Flordelis, MD and Johannes F. Dayrit, MD, FPDS
Bullous systemic lupus erythematosus (BSLE) is an autoimmune-mediated, chronic, widespread, non-scarring, sub-epidermal blistering skin disease. It is typically caused by autoantibodies against type VII collagen. BSLE is an uncommon manifestation of SLE and is found in less than 5% of SLE cases.
We report a case of a 21-year-old woman with a two-year history of systemic lupus erythematosus who developed multiple pruritic vesicles on the neck six weeks prior to consultation. The lesions increased in number, size, and distribution, becoming bullae on the face, trunk, upper and lower extremities. Nikolsky and Asboe – Hansen signs were negative. Mucosal involvement and visceral organ impairment were not identified on physical examination. A 4-mm skin punch biopsy of a vesicle showed a subepidermal blister. Direct immunofluorescence of perilesional skin revealed linear deposits of IgG (+1), C3 (+1), IgM (+2) and granular fibrinogen (+2) at the basement membrane zone, consistent with bullous systemic lupus erythematosus. Oral prednisone 40 mg/day and hydroxychloroquine 200mg once a day were prescribed. The lesions became dry and flat after two weeks and no new eruption was noted after three months.
Patients with BSLE manifest with a widespread symmetrical distribution of vesicular skin lesions frequently favoring the upper part of the trunk, flexural and extensor aspects of the upper extremities, neck and face but may occur anywhere on the cutaneous surface. Treatment with corticosteroids and hydroxychloroquine was effective in our case. The prognosis of BSLE is good with variable duration characterized by spontaneous exacerbations and remissions influenced more by the systemic disease rather than by the eruption hence the need to follow closely the systemic disease activity.
Ibaviosa, GM, Flordelis, J & Dayrit, J. (2017). Bubbly lupus: A case of a Filipino woman with bullous systemic lupus erythematosus successfully treated with prednisone and hydroxychloroquine. Journal of the Philippine Dermatological Society, 26(1), 65-68.
bullous systemic lupus erythematosus, lupus, prednisone, hydroxychloroquine
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