Histopathology of anti-laminin 5 mucous membrane pemphigoid.
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.
Rose
Christian
C
Department of Dermatology, University of Lübeck, Lübeck, Germany.
Schmidt
Enno
E
Kerstan
Andreas
A
Thoma-Uszynski
Sybille
S
Wesselmann
Ulrich
U
Käsbohrer
Ulrich
U
Zillikens
Detlef
D
Shimanovich
Iakov
I
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
D001323:Autoantibodies; D015815:Cell Adhesion Molecules; D007074:Immunoglobulin G; D000097794:Kalinin