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Paraneoplastic Pemphigus Associated with a Malignant Thymoma: A Case of Persistent and Refractory Oral Ulcerations Following Thymectomy

Authors
 Jung Min Lim  ;  Sang Eun Lee  ;  Jimyung Seo  ;  Do Young Kim  ;  Takashi Hashimoto  ;  Soo-Chan Kim 
Citation
 ANNALS OF DERMATOLOGY, Vol.29(2) : 219-222, 2017 
Journal Title
 ANNALS OF DERMATOLOGY 
ISSN
 1013-9087 
Issue Date
2017
Keywords
Oral ulce ; Paraneoplastic syndromes ; Pemphigus ; Thymoma
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.
Files in This Item:
T201702385.pdf Download
DOI
10.5021/ad.2017.29.2.219
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Dermatology (피부과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Do Young(김도영) ORCID logo https://orcid.org/0000-0002-0194-9854
Kim, Soo Chan(김수찬) ORCID logo https://orcid.org/0000-0002-2327-4755
Seo, Jimyung(서지명)
Lee, Sang Eun(이상은) ORCID logo https://orcid.org/0000-0003-4720-9955
Lim, Jung Min(임정민) ORCID logo https://orcid.org/0000-0002-0223-5933
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/160449
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