Ann Dermatol.  2017 Apr;29(2):219-222. 10.5021/ad.2017.29.2.219.

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

Affiliations
  • 1Department of Dermatology, Gangnam Severance Hospital, Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea. kimsc@yuhs.ac
  • 2Department of Dermatology, Severance Hospital, Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea.
  • 3Department of Dermatology, Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, Kurume, Japan.

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.

Keyword

Oral ulcer; Paraneoplastic syndromes; Pemphigus; Thymoma

MeSH Terms

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*
Autoantibodies
Desmoglein 1
Laminin
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