title: Non-paraneoplastic autoimmune subepidermal bullous disease associated with fatal bronchiolitis obliterans.
abstract: 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.
authors.affiliation: Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
authors.forename: Mari
authors.initials: M
authors.lastname: Orime
authors.affiliation: Department of Dermatology, Niigata City General Hospital, Niigata, Japan.
authors.forename: Katsuhiro
authors.initials: K
authors.lastname: Tomiyama
authors.affiliation: Department of Pathology, Niigata City General Hospital, Niigata, Japan.
authors.forename: Hideki
authors.initials: H
authors.lastname: Hashidate
authors.affiliation: Department of Emergency and Critical Care Medicine Center, Niigata City General Hospital, Niigata, Japan.
authors.forename: Satoru
authors.initials: S
authors.lastname: Yoshida
authors.affiliation: Department of Respiratory Medicine and Infectious Diseases, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
authors.forename: Satoshi
authors.initials: S
authors.lastname: Hokari
authors.affiliation: Department of Endocrinology and Metabolism, Kido Hospital, Niigata, Japan.
authors.forename: Akiko
authors.initials: A
authors.lastname: Tsuda
authors.affiliation: Department of Gastroenterology and Hepatology, Kido Hospital, Niigata, Japan.
authors.forename: Hisashi
authors.initials: H
authors.lastname: Yokoyama
authors.affiliation: Department of Respiratory Medicine, Kido Hospital, Niigata, Japan.
authors.forename: Jun-Ichi
authors.initials: JI
authors.lastname: Narita
authors.affiliation: Department of Dermatology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.
authors.forename: Youhei
authors.initials: Y
authors.lastname: Uchida
authors.affiliation: Department of Dermatology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.
authors.forename: Takuro
authors.initials: T
authors.lastname: Kanekura
authors.affiliation: Division of Dermatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan.
authors.forename: Riichiro
authors.initials: R
authors.lastname: Abe
authors.affiliation: Department of Dermatology, Kurume University School of Medicine and Kurume University Institute of Cutaneous Cell Biology, Kurume, Japan.
authors.forename: Norito
authors.initials: N
authors.lastname: Ishii
authors.affiliation: Department of Dermatology, Kurume University School of Medicine and Kurume University Institute of Cutaneous Cell Biology, Kurume, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Hashimoto
authors.affiliation: Department of Dermatology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.|Department of Dermatology, Kido Hospital, Niigata, Japan.
authors.forename: Kazuhiro
authors.identifier: http://orcid.org/0000-0001-9375-0713
authors.initials: K
authors.lastname: Kawai
mesh_terms: D000368:Aged; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D001323:Autoantibodies / Q000097:blood; D001324:Autoantigens / Q000276:immunology*; D001485:Basement Membrane / Q000378:metabolism; D001706:Biopsy; D001989:Bronchiolitis Obliterans / Q000097:blood / Q000150:complications*; D015815:Cell Adhesion Molecules / Q000276:immunology*; D017809:Fatal Outcome; D019085:Fluorescent Antibody Technique, Direct; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007070:Immunoglobulin A / Q000097:blood / Q000378:metabolism; D007074:Immunoglobulin G / Q000097:blood; D008297:Male; D008911:Minocycline / Q000627:therapeutic use; D009536:Niacinamide / Q000627:therapeutic use; D024041:Non-Fibrillar Collagens / Q000276:immunology*; D010257:Paraneoplastic Syndromes / Q000150:complications; D010391:Pemphigoid, Bullous / Q000150:complications* / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D011239:Prednisolone / Q000627:therapeutic use; D012131:Respiratory Insufficiency / Q000209:etiology*; D014803:Vitamin B Complex / Q000627:therapeutic use; D000097794:Kalinin; D000097805:Collagen Type XVII
chemical_list: D000900:Anti-Bacterial Agents; D001323:Autoantibodies; D001324:Autoantigens; D015815:Cell Adhesion Molecules; D005938:Glucocorticoids; D007070:Immunoglobulin A; D007074:Immunoglobulin G; D008911:Minocycline; D009536:Niacinamide; D024041:Non-Fibrillar Collagens; D011239:Prednisolone; D014803:Vitamin B Complex; D000097794:Kalinin; D000097805:Collagen Type XVII
keywords: bronchiolitis obliterans; collagen type XVII; kalinin; linear immunoglobulin A bullous dermatosis; pemphigoid