Pemphigus Erythematosus specially its localized form is a rare autoimmune blistering disease combining features of Pemphigus Foliaceus and Lupus Erythematosus. We reported a 60 years old woman with skin lesion which presented Erythemato squamo plaque with oozing in the right cheek. The chief compliant was burning and pruritus. Skin biopsy was obtained and discoid Lupus Erythemato was reported. She was prescribed with topical steroid but did not improve. All of the lab tests such as Anti DNA, ESR, RF, CRP, CH50, C3, C4, ANA were normal. The biopsy repeated and Pemphigus Erythematosus was reported. Direct immunoflurescent test confirmed the diagnosis. We treated the patient with systemic steroid (80mg/day). The patient responed well and showed complete remission during 3 months.

Keywords: Localized, pemphigus, negative serology

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