Bullous pemphigoid positive for anti-BP180 and anti-laminin 5 antibodies in a patient with graft-vs-host disease.
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.
Izumi
Rieko
R
Department of Dermatology, Kanazawa University Graduate School of Medical Science, Ishikawa, Japan.
Fujimoto
Manabu
M
Yazawa
Norihito
N
Nakashima
Hiroko
H
Asashima
Nobuko
N
Watanabe
Rei
R
Kuwano
Yoshihoro
Y
Kurokawa
Mineo
M
Hashimoto
Takashi
T
Tamaki
Kunihiko
K
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
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