Journal
ANNALS OF DERMATOLOGY
Volume 29, Issue 2, Pages 219-222Publisher
KOREAN DERMATOLOGICAL ASSOC
DOI: 10.5021/ad.2017.29.2.219
Keywords
Oral ulcer; Paraneoplastic syndromes; Pemphigus; Thymoma
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Paraneoplastic pemphigus is a rare, life-threatening auto immune mucocutaneous blistering disease associated with underlying neoplasia, commonly lymphoproliferative tumors. Herein we report a case of paraneoplastic pemphigus with a unique autoantibody profile associated with a malignant thymoma. A 56-year-old female patient presented with relapsing oral ulcerations accompanied by erythematous papules and patches on her extremities for 2 months. Skin and mucosal biopsies identified interface dermatitis with lichenoid lymphocytic infiltration in the upper dermis. Immunoblotting and enzyme-linked immunosorbent assays revealed that the patient had multiple autoantibodies against desmoglein 1, desmocol lin 1, 2, 3, laminin gamma-1, envoplakin, and periplakin. The skin lesions completely healed following thymectomy and systemic corticosteroid therapy, but the oral ulcerations persisted through a follow-up period of over 2 years.
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