Histopathology of anti-laminin 5 mucous membrane pemphigoid - 07/08/11

Doi : 10.1016/j.jaad.2009.02.012

Christian Rose, MD ^a, Enno Schmidt, MD, PhD ^a, Andreas Kerstan, MD ^b, Sybille Thoma-Uszynski, MD ^c, Ulrich Wesselmann, MD ^d, Ulrich Käsbohrer, MD ^e, Detlef Zillikens, MD ^a, Iakov Shimanovich, MD ^a,^⁎
^a Department of Dermatology, University of Lübeck, Lübeck, Germany
^b Department of Dermatology, University of Würzburg, Würzburg, Germany
^c Department of Dermatology, University of Erlangen, Erlangen, Germany
^d Department of Dermatology, Helios Hospital Wuppertal, Wuppertal, Germany
^e Department of Dermatology, Oldenburg Hospital, Oldenburg, Germany

Reprint requests: Iakov Shimanovich, MD, Department of Dermatology, University of Lübeck, Ratzeburger Allee 160, 23538 Lübeck, Germany.

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 and Methods

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.

Limitations

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.

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Abbreviations used : BP, EBA, H&E, MMP

Plan

Introduction

Patients and methods

Patients

Histopathology, immunohistochemistry, and immunofluorescence microscopy

Immunoblot and immunoprecipitaton studies

Results

Discussion

	Funding sources: None.
	Conflicts of interest: None declared.
	This work was presented in part at the 28th Symposium of the International Society of Dermatopathology, November 14-17, 2007, Paris, France.