Paraneoplastic pemphigus associated with fatal bronchiolitis obliterans and appearance of anti‐ BP 180 antibodies in the late stage of the disease

• Published in: Journal of dermatology 2014-07, Vol.41 (7), p.628-630
• Main Authors: Kanaoka, Miwa, Matsukura, Setsuko, Ishikawa, Hideyuki, Matsuura, Midori, Ishii, Norito, Hashimoto, Takashi, Aihara, Michiko
• Format: Article
• Language: English
• ISSN: 0385-2407; 1346-8138
• DOI: 10.1111/1346-8138.12521

Paraneoplastic pemphigus ( PNP ) is an autoimmune bullous disease, which associates mainly with lymphoproliferative neoplasms. Bronchiolitis obliterans ( BO ) with progressive respiratory failure is a significant cause of death in PNP . We report a case of PNP associated with follicular lymphoma and BO , which showed findings suggesting coexistence of bullous pemphigoid ( BP ). The patient showed bullous and ulcerative lesions on the lips and oral cavity, and flaccid blisters on the trunk and thighs associated with anti‐desmoglein ( D sg)3 antibodies. At later disease stage after commencement of treatment, anti‐ BP 180 antibodies and tense blister formation were observed. It was proposed that persistent interface dermatitis is the first event in PNP , and subsequently induce the production of autoantibodies to D sg and components of the basement membrane zone, resulting in both intraepidermal and subepidermal blisters. We speculate that interface dermatitis caused by autoreactive T cells induced autoantibody production against D sg3, and subsequently against BP 180.