Facial bullous systemic lupus erythematosus

A 37-year-old woman presented with a 6-month history of malaise, fever, arthralgia, and mild butterfly facial erythema. A diagnosis of systemic lupus erythematosus (SLE) was established, based on clinical, laboratory, and histologic findings, and treatment with 6-methylprednisolone (80 mg daily) was administered, A week later, a blistering eruption appeared in and around the erythematous areas on the face. On physical examination, infiltrated erythematous plaques were present on the sun-exposed areas: the arms, dorsa of the hands, and upper chest. In and around the erythematous areas on the face, there were numerous tense vesicles (Fig. 1). A few ulcerations were observed on the lower lip and the hard palate. Laboratory findings revealed a reduceo white blood cell count (WBC) (2500/mm(3)), with normal values of hemoglobin, red blood cell count (RBC), hematocrit, platelets, creatinine, urea, total protein, blood glucose, and urinalysis. Antinuclear antibodies (ANA) were detected in titre of 1:320 an rat liver substrate. Anti-DNA, Ro, La-antibodies and LE-cells were positive. Anti BMZ-antibodies were not present on the indirect immunofluorescence (IIF) testing on normal human skin substrate. Histopathologic examination showed a subepidermal blister containing polymorphonuclears and eosinophils. There was a mixed inflammatory infiltrate in the upper dermis (Fig. 2). Direct immunofluorescence (DIF) of perilesional skin from the face showed homogenous linear deposits of IgG and IgA at the basement membrane zone (BMZ); DIF from non-sun-exposed normal skin demonstrated granular deposits of IgA and IgM at the BMZ. Besides the systemic steroid therapy with 6-methylprednisolone, a treatment with dapsone at an initial dosage of 50 mg, and later 100 mg, daily was applied. The condition was resistant to the steroid therapy, but after 4 weeks of treatment with dapsone the lesions completely resolved.