Paraneoplastic Pemphigus Associated with a Malignant Thymoma: A Case of Persistent and Refractory Oral Ulcerations Following Thymectomy.
- Author:
Jung Min LIM
1
;
Sang Eun LEE
;
Jimyung SEO
;
Do Young KIM
;
Takashi HASHIMOTO
;
Soo Chan KIM
Author Information
- Publication Type:Case Report
- Keywords: Oral ulcer; Paraneoplastic syndromes; Pemphigus; Thymoma
- MeSH: Autoantibodies; Biopsy; Blister; Dermatitis; Dermis; Desmoglein 1; Enzyme-Linked Immunosorbent Assay; Extremities; Female; Follow-Up Studies; Humans; Immunoblotting; Laminin; Middle Aged; Oral Ulcer*; Paraneoplastic Syndromes; Pemphigus*; Skin; Thymectomy*; Thymoma*
- From:Annals of Dermatology 2017;29(2):219-222
- CountryRepublic of Korea
- Language:English
- Abstract: Paraneoplastic pemphigus is a rare, life-threatening autoimmune 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, desmocollin 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.
