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

• Lim JM
• Lee SE
• Seo J
• Kim DY
• Hashimoto
• Kim SC

• KMID: 2394848
• DOI: http://doi.org/10.5021/ad.2017.29.2.219

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

Figure

• 

  Fig. 1 Clinical findings. The patient initially presented with oral ulcerations (A, B) and erythematous papules on the extremities (D). Oral lesions persist even 3 months following thymectomy (C). A mediastinal tumor was found on chest computed tomography (E, arrowheads), which was later confirmed to be a malignant thymoma.

• 

  Fig. 2 Histopathologic findings. Skin biopsies of (A) the oral mucosa lesion and (B) the foot lesion. Both of them demonstrate interface dermatitis with diffuse lymphocytic infiltration in the upper dermis. (B) Several necrotic keratinocytes are scattered in the epidermis (H&E; A, B: ×200 magnification).

• 

  Fig. 3 Immunologic findings. (A) Indirect immunofluorescence demonstrates IgG deposition at the intercellular space of human skin at 1:40 dilution and (B) IgG deposition at the epithelia of rat bladder at 1:10 dilution. (C) Immunoblotting of normal human epidermal extracts is positive for envoplakin (210 kDa) and periplakin (190 kDa; arrows). (D) Immunoblotting of dermal extracts is positive for laminin gamma-1 (200 kDa). BP: bullous pemphigoid, PNP: paraneoplastic pemphigus, PV: pemphigus vulgaris, EBA: epidermolysis bullosa acquisita.

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