title: Bullous pemphigoid positive for anti-BP180 and anti-laminin 5 antibodies in a patient with graft-vs-host disease.
abstract: We report the case of a 55-year-old female with bullous pemphigoid (BP) who was positive for anti-BP180 and anti-laminin 5 antibodies after development of graft-vs-host disease (GVHD) caused by a bone marrow transplant. She had tense blisters on her trunk and extremities. Histologic examination showed a subepidermal blister and marked lymphocytic infiltration, especially eosinophils. Direct immunofluorescence revealed a linear deposition of IgG on the base membrane zone. Indirect immunofluorescence on 1M NaCl split skin revealed a linear IgG deposition to both sides of the epidermal and the dermal layers. Immunoblot assays using human epidermal extracts and BP180 NC16a domain recombinant protein confirmed the presence of IgG antibodies against BP180 and recombinant BP180 NC16a domain protein. Furthermore, immunoblotting using laminin 5 purified from human keratinocyte extract as the substrate demonstrated reactivity against the gamma2 and beta3 subunits but not the alpha3 subunit of laminin 5. We diagnosed BP and treated her with prednisolone (40 mg/day). Both skin and oral lesions resolved without leaving scars on the bulla. Immune disturbance as well as destruction of basal epidermal cells and base membrane by GVHD may result in the induction of autoimmune blistering diseases with unusual clinical and laboratory manifestations.
authors: Department of Dermatology, Kanazawa University Graduate School of Medical Science, Ishikawa, Japan.
authors: Rieko
authors: R
authors: Izumi
authors: Manabu
authors: M
authors: Fujimoto
authors: Norihito
authors: N
authors: Yazawa
authors: Hiroko
authors: H
authors: Nakashima
authors: Nobuko
authors: N
authors: Asashima
authors: Rei
authors: R
authors: Watanabe
authors: Yoshihoro
authors: Y
authors: Kuwano
authors: Mineo
authors: M
authors: Kurokawa
authors: Takashi
authors: T
authors: Hashimoto
authors: Kunihiko
authors: K
authors: Tamaki
mesh_terms: D001323:Autoantibodies / Q000097:blood*; D001324:Autoantigens / Q000276:immunology*; D016026:Bone Marrow Transplantation / Q000009:adverse effects; D015815:Cell Adhesion Molecules / Q000276:immunology*; D005260:Female; D019085:Fluorescent Antibody Technique, Direct; D019084:Fluorescent Antibody Technique, Indirect; D005938:Glucocorticoids / Q000627:therapeutic use; D006086:Graft vs Host Disease / Q000150:complications / Q000209:etiology; D006801:Humans; D007074:Immunoglobulin G / Q000378:metabolism; D007797:Laminin / Q000276:immunology*; D008875:Middle Aged; D024041:Non-Fibrillar Collagens / Q000276:immunology*; D010391:Pemphigoid, Bullous / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology* / Q000473:pathology; D011239:Prednisolone / Q000627:therapeutic use; D012867:Skin / Q000378:metabolism; D014018:Tissue Distribution; D000097794:Kalinin; D000097805:Collagen Type XVII
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D015815:Cell Adhesion Molecules; D005938:Glucocorticoids; D007074:Immunoglobulin G; D007797:Laminin; D024041:Non-Fibrillar Collagens; D011239:Prednisolone; D000097794:Kalinin; D000097805:Collagen Type XVII; C400567:LAMC2 protein, human