title: Histopathology of anti-laminin 5 mucous membrane pemphigoid.
abstract: BACKGROUND
  Anti-laminin 5 mucous membrane pemphigoid (MMP) is an autoimmune blistering disease characterized by autoantibodies against the major basement membrane component laminin 5 (laminin 332, epiligrin).
  
  
  OBJECTIVE
  We reviewed 17 biopsy specimens from 9 patients with anti-laminin 5 MMP in an attempt to define typical histopathologic features of the disease.
  
  
  RESULTS
  Fifteen specimens showed subepidermal blister formation, while two biopsy specimens revealed an epithelial ulcer. In 11 biopsies a sparse to moderate inflammatory infiltrate composed of lymphocytes and neutrophils with some eosinophils was observed. Four biopsies showed a dense infiltrate dominated by neutrophils in two cases and by eosinophils in one case. The remaining biopsy revealed a dense lymphoplasmacellular infiltrate without granulocytes. Scarring of the upper dermis was present only in 5 specimens. Immunohistochemical analysis localized type IV collagen to the dermal side of the blister, suggesting that split formation occurred within the lamina lucida of the cutaneous basement membrane.
  
  
  CONCLUSIONS
  The number of patients studied was relatively small.
  
  
  CONCLUSIONS
  Histopathology of anti-laminin 5 MMP is characterized by subepidermal blistering and a sparse to moderate superficial lymphohistiocytic infiltrate with neutrophils and/or eosinophils. Both infiltrate density and composition may vary, making anti-laminin 5 MMP indistinguishable from other autoimmune subepidermal blistering diseases by histopathology alone. Scarring is present only in a minority of cases and is not a sensitive clue to the diagnosis of anti-laminin 5 MMP.
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany.
authors.forename: Christian
authors.initials: C
authors.lastname: Rose
authors.forename: Enno
authors.initials: E
authors.lastname: Schmidt
authors.forename: Andreas
authors.initials: A
authors.lastname: Kerstan
authors.forename: Sybille
authors.initials: S
authors.lastname: Thoma-Uszynski
authors.forename: Ulrich
authors.initials: U
authors.lastname: Wesselmann
authors.forename: Ulrich
authors.initials: U
authors.lastname: Käsbohrer
authors.forename: Detlef
authors.initials: D
authors.lastname: Zillikens
authors.forename: Iakov
authors.initials: I
authors.lastname: Shimanovich
mesh_terms: D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000097:blood*; D001485:Basement Membrane / Q000276:immunology / Q000473:pathology; D001706:Biopsy; D015815:Cell Adhesion Molecules / Q000276:immunology*; D002648:Child; D005260:Female; D019085:Fluorescent Antibody Technique, Direct; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D007830:Larynx / Q000276:immunology / Q000473:pathology; D008297:Male; D009055:Mouth / Q000276:immunology / Q000473:pathology; D010390:Pemphigoid, Benign Mucous Membrane / Q000276:immunology* / Q000473:pathology*; D010614:Pharynx / Q000276:immunology / Q000473:pathology; D012867:Skin / Q000276:immunology / Q000473:pathology; D014844:Vulva / Q000276:immunology / Q000473:pathology; D000097794:Kalinin
chemical_list: D001323:Autoantibodies; D015815:Cell Adhesion Molecules; D007074:Immunoglobulin G; D000097794:Kalinin