Immunofluorescence serration pattern analysis as a diagnostic criterion in antilaminin‐332 mucous membrane pemphigoid: immunopathological findings and clinical experience in 10 Dutch patients

Terra, Jorrit B.; Pas, Hendrikus; Hertl, Michael; Dikkers, Frederik G.; Kamminga, Nicole Sillevis Smitt; Jonkman, Marcel F.

doi:10.1111/j.1365-2133.2011.10474.x

Cited by 55 publications

(64 citation statements)

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“…Deposition of IgG in an n-serrated pattern on DIF was reported to be characteristic of anti-laminin-332 MMP. [8] Our case also showed linear deposition of immune reactants at the BMZ. Because laminin-332 is present deep in the lamina lucida localized to anchoring filaments, circulating antibodies react with dermal side of salt split human skin.…”

Section: Discussionmentioning

confidence: 82%

“…[7] Recently, Terra et al, proposed a modified diagnostic criterion for anti-laminin-332 MMP, on clinical, histopathological and immunological basis. [8] On histopathology, MMP shows subepidermal bullae with lymphohistiocytic and eosinophilic infiltrate. Deposition of IgG in an n-serrated pattern on DIF was reported to be characteristic of anti-laminin-332 MMP.…”

Section: Discussionmentioning

confidence: 98%

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Mucous membrane pemphigoid with antibodies against β3 subunit of laminin-332: First report from India

Kanwar¹,

Vinay²,

Koga³

et al. 2012

Indian J Dermatol Venereol Leprol

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Mucous membrane pemphigoid (MMP) is a chronic, recurrent, progressive, subepidermal blistering disorder, mainly affecting the mucous membranes. Anti-laminin 332 MMP is a distinct subset of MMP with antibodies, mainly targeted against α3 or γ2 subunit. Antibodies exclusively against β3 subunit are rarely seen. An internal malignancy is frequently associated with anti-laminin 332 MMP. This disorder usually responds poorly to treatment, requiring multidisciplinary approach. Herein, we describe the first case of anti-laminin-332 MMP from India, which showed antibodies exclusively to β3 subunit.

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Section: Discussionmentioning

confidence: 82%

Section: Discussionmentioning

confidence: 98%

Mucous membrane pemphigoid with antibodies against β3 subunit of laminin-332: First report from India

Kanwar¹,

Vinay²,

Koga³

et al. 2012

Indian J Dermatol Venereol Leprol

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“…The n-serrated pattern is seen in several subepidermal AIBD, but the u-serrated pattern is unique to EBA (Figure 6). If this criterion is included for EBA diagnosis, the diagnostic sensitivity can be greatly increased [6, 190–192]. …”

Section: Diagnosis Of Ebamentioning

confidence: 99%

Clinical Presentation, Pathogenesis, Diagnosis, and Treatment of Epidermolysis Bullosa Acquisita

Ludwig

2013

ISRN Dermatology

124

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Epidermolysis bullosa acquisita (EBA) is a chronic mucocutaneous autoimmune skin blistering disease. The pathogenic relevance of autoantibodies targeting type VII collagen (COL7) has been well-documented. Therefore, EBA is a prototypical autoimmune disease with a well-characterized pathogenic relevance of autoantibody binding to the target antigen. EBA is a rare disease with an incidence of 0.2 new cases per million and per year. The current treatment of EBA relies on general immunosuppressive therapy, which does not lead to remission in all cases. Therefore, there is a high, so far unmet medical need for the development of novel therapeutic options. During the last 10 years, several novel in vitro and in vivo models of EBA have been established. These models demonstrated a critical role of the genetic background, T cells, and cytokines for mediating the loss of tolerance towards COL7. Neutrophils, complement activation, Fc gamma receptor engagement, cytokines, several molecules involved in cell signaling, release of reactive oxygen species, and matrix metalloproteinases are crucial for autoantibody-induced tissue injury in EBA. Based on this growing understanding of the diseases' pathogenesis, several potential novel therapeutic targets have emerged. In this review, the clinical presentation, pathogenesis, diagnosis, and current treatment options for EBA are discussed in detail.

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“…The u-serrated pattern is pathognomonic for skin-bound autoantibodies against type VII collagen as found in EBA and bullous systemic lupus erythematosus [26,124]. In all other pemphigoid diseases, a n-serrated pattern characterized by arches closed at the top giving a 'snake-like' appearance [110,124,125]. However, in mucosal biopsies and a number of skin samples, no u-or n-serrated pattern can be seen.…”

Section: Direct If Microscopymentioning

confidence: 99%

Clinical features and diagnosis of epidermolysis bullosa acquisita

Vorobyev

Ludwig

Schmidt

2016

Expert Review of Clinical Immunology

100

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Introduction: Epidermolysis bullosa acquisita (EBA) is a rare autoimmune blistering disease of skin and mucous membranes. EBA is caused by autoantibodies against type VII collagen, which is a major component of anchoring fibrils, attaching epidermis to dermis. Binding of autoantibodies to type VII collagen leads to skin fragility and, finally, blister formation. The clinical picture of EBA is polymorphic, with several distinct phenotypes being described. Despite recent progress in understanding the pathophysiology of EBA, its diagnosis is still challenging. Areas covered: This review provides an update on the clinical manifestations and diagnostic methods of EBA. We searched PubMed using the terms 'epidermolysis bullosa acquisita' covering articles in English between 1 January 2005 and 31 May 2016. Relevant older publications were retrieved form cited literature. Expert commentary: While the clinical picture is highly variable, diagnosis relies on direct immunofluorescence (IF) microscopy of a perilesional skin biopsy. Linear deposits of IgG, IgA and/or C3 along the dermal-epidermal junction with an u-serrated pattern are diagnostic for EBA alike the detection of serum autoantibodies against type VII collagen. Several test systems for the serological diagnosis of EBA have recently become widely available. In some patients, sophisticated diagnostic approaches only available in specialized centers are required. ARTICLE HISTORY

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Immunofluorescence serration pattern analysis as a diagnostic criterion in antilaminin‐332 mucous membrane pemphigoid: immunopathological findings and clinical experience in 10 Dutch patients

Cited by 55 publications

References 44 publications

Mucous membrane pemphigoid with antibodies against β3 subunit of laminin-332: First report from India

Mucous membrane pemphigoid with antibodies against β3 subunit of laminin-332: First report from India

Clinical Presentation, Pathogenesis, Diagnosis, and Treatment of Epidermolysis Bullosa Acquisita

Clinical features and diagnosis of epidermolysis bullosa acquisita

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