Non-paraneoplastic autoimmune subepidermal bullous disease associated with fatal bronchiolitis obliterans

作者：Orime Mari; Tomiyama Katsuhiro; Hashidate Hideki; Yoshida Satoru; Hokari Satoshi; Tsuda Akiko; Yokoyama Hisashi; Narita Jun ichi; Uchida Youhei; Kanekura Takuro; Abe Riichiro; Ishii Norito; Hashimoto Takashi; Kawai Kazuhiro

来源：Journal of Dermatology, 2017, 44(4): 461-464.

DOI：10.1111/1346-8138.13706

• bronchiolitis obliterans
• collagen type XVII
• kalinin
• linear immunoglobulin A bullous dermatosis
• pemphigoid

Bronchiolitis obliterans is a small-airway obstructive lung disease for which immunologically mediated pathogenesis is supposed. Frequent association of bronchiolitis obliterans with paraneoplastic pemphigus is well known, but its association with other autoimmune bullous diseases has not been reported except for a case of anti-laminin-332-type mucous membrane pemphigoid in a patient with chronic graft-versus-host disease. We report a case of non-paraneoplastic autoimmune subepidermal bullous disease associated with fatal bronchiolitis obliterans in a patient without transplantation. Although the patient's serum contained immunoglobulin (Ig)A antibodies to the 180-kDa bullous pemphigoid antigen/type XVII collagen and IgG antibodies to laminin-332, diagnosis of either linear IgA bullous dermatosis or mucous membrane pemphigoid could not be made because of the failure to detect linear IgA deposition at the basement membrane zone by direct immunofluorescence and the lack of mucous membrane lesions. Physicians should be aware that autoimmune bullous diseases other than paraneoplastic pemphigus can also associate with this rare but potentially fatal lung disease.

• 出版日期2017-4