title: Recalcitrant facial pemphigus vulgaris: correlation of skin lesions with the ratio of antidesmoglein antibodies 1 and 3.
abstract: Pemphigus vulgaris (PV) is an autoimmune bullous disease characterized by autoantibodies against desmogleins. We report a case of recalcitrant PV, which progressed from the mucosal to the mucocutaneous type, with a corresponding increase in anti-desmoglein (Dsg)1 and decrease in anti-Dsg3 antibody titres. Thus, the clinical features seemed to correlate with the ratio of anti-Dsg1 and 3. The patient also had anti-Dsg4 antibodies, which might be related to the nonscarring diffuse hair loss and marked facial involvement she also had. The patient did not respond to treatment with systemic steroid, ciclosporin, azathioprine, cyclophosphamide or double filtration plasmapheresis, and eventually died from fulminant thrombotic thrombocytopenic purpura of unknown cause.
authors.affiliation: Department of Dermatology, Asahikawa Medical College, Asahikawa, Japan. ma0dj@asahikawa-med.ac.jp
authors.forename: M
authors.initials: M
authors.lastname: Fujii
authors.forename: M
authors.initials: M
authors.lastname: Honma
authors.forename: S
authors.initials: S
authors.lastname: Iinuma
authors.forename: K
authors.initials: K
authors.lastname: Kaneta
authors.forename: S
authors.initials: S
authors.lastname: Komatsu
authors.forename: K
authors.initials: K
authors.lastname: Sato
authors.forename: H
authors.initials: H
authors.lastname: Takahashi
authors.forename: A
authors.initials: A
authors.lastname: Ishida-Yamamoto
authors.forename: H
authors.initials: H
authors.lastname: Iizuka
mesh_terms: D001323:Autoantibodies / Q000097:blood*; D051183:Desmoglein 1 / Q000276:immunology*; D051184:Desmoglein 3 / Q000276:immunology*; D005148:Facial Dermatoses / Q000276:immunology* / Q000473:pathology; D005260:Female; D006801:Humans; D008875:Middle Aged; D010392:Pemphigus / Q000276:immunology* / Q000473:pathology
chemical_list: D001323:Autoantibodies; D051183:Desmoglein 1; D051184:Desmoglein 3