Paraneoplastic Pemphigus Associated with a Malignant Thymoma: A Case of Persistent and Refractory Oral Ulcerations Following Thymectomy
Annals of Dermatology
; : 219-222, 2017.
Article
in En
| WPRIM
| ID: wpr-25581
Responsible library:
WPRO
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.
Key words
Full text:
1
Index:
WPRIM
Main subject:
Paraneoplastic Syndromes
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Skin
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Autoantibodies
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Thymectomy
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Thymoma
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Biopsy
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Enzyme-Linked Immunosorbent Assay
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Immunoblotting
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Follow-Up Studies
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Blister
Type of study:
Observational_studies
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Prognostic_studies
Limits:
Female
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Humans
Language:
En
Journal:
Annals of Dermatology
Year:
2017
Type:
Article