Paraneoplastic Pemphigus Associated with Hepatocellular Carcinoma / 대한피부과학회지

ABSTRACT

PNP is a rare autoimmune mucocutaneous blistering disease associated with neoplasms, most frequently of the lymphoproliferative type. As PNP is clinically characterized by polymorphous mucosal lesions and cutaneous eruptions, it is important to differentiate it from erythema multiforme, Stevens-Johnson syndrome, lichen planus, and other bullous diseases. A diagnosis of PNP can be confirmed by immunologic studies such as direct and indirect immunofluorescence, immunoblotting, immunoprecipitation. Rare PNP cases related to nonhematological solid tumors have been reported. A 54-year-old male visited us with generalized pruritic scaly lichenoid lesions on the whole body from 5 weeks prior to his first visit. He also presented with extensive painful ulcers and erosions on the oral mucosa and lips for 2 months. Histopathologic findings showed lichenoid infiltration with vacuolar interface change, lichenoid interface dermatitis, keratinocyte apoptosis, and suprabasal acantholysis with cleft. Indirect immunofluorescence using normal human skin showed IgG deposition at the intercellular space. Immunoblotting using normal epidermal extracts in the serum of patient detected antibody to the 190 kDa (envoplakin), 210 kDa (periplakin) molecules polypeptides. He also had a hepatocellular carcinoma and chemoradiotherapy done before. The diagnosis of paraneoplastic pemphigus (PNP) was made. To our knowledge, there are only two reports of PNP associated with hepatocellular carcinoma worldwide, yet no report in Korean literature. Herein, we report the first case of PNP associated with hepatocellular carcinoma in Korea.