S2k guideline for the diagnosis of pemphigus vulgaris/foliaceus and bullous pemphigoid

Schmidt, Enno; Goebeler, Matthias; Hertl, Michael; Sárdy, Miklós; Sitaru, Cassian; Eming, Ruediger; Hofmann, Silke; Hunzelmann, Nicolas; Kern, Johannes S.; Kramer, Harald; Orzechowski, Hans‐Dieter; Pfeiffer, Christiane; Schuster, Volker; Sporbeck, Birte; Sticherling, Michael; Worm, Margitta; Zillikens, Detlef; Nast, Alexander

doi:10.1111/ddg.12612

Cited by 111 publications

(176 citation statements)

References 126 publications

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“…Differential diagnosis of erosive and crusted lesions on the scalp may be challenging, especially when the disease is restricted to the scalp. Diagnosis of autoimmune bullous diseases is based on patient's history, clinical picture, histopathology, direct immunofluorescence as well as indirect immunofluorescence and enzyme‐linked immunoassay . Trichoscopy is a recognized method for noninvasive diagnosis of disorders such as androgenetic alopecia, alopecia areata, lichen planus, frontal fibrosing alopecia, discoid lupus erythematosus, psoriasis, seborrheic dermatitis, tinea capitis, and hair shaft abnormalities …”

Section: Commentmentioning

confidence: 99%

“…Autoimmune bullous diseases, such as pemphigus, bullous pemphigoid (BP), and dermatitis herpetiformis (DH), can manifest as blisters and erosions on the scalp . The diagnosis of autoimmune bullous disease is based on direct immunofluorescence, histopathology, and immunoserological tests such as indirect immunofluorescence and enzyme‐linked immunoassay …”

Section: Introductionmentioning

confidence: 99%

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Trichoscopy – a useful tool in the preliminary differential diagnosis of autoimmune bullous diseases

2017

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Section: Commentmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Trichoscopy – a useful tool in the preliminary differential diagnosis of autoimmune bullous diseases

2017

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“…[27,28] Diagnosis of BP is based on a combination of linear deposits of IgG and/or C3 at the dermal-epidermal junction by direct immunofluorescence (IF) microscopy and serological studies, including indirect IF microscopy on monkey esophagus and salt-split human skin as well as ELISA for BP180 and BP230. [29,30] Lesional histopathology reveals subepidermal splitting and an eosinophil-rich inflammatory infiltrate in the upper dermis ( Figure 2). Furthermore, a link between eosinophils and coagulation has been identified via tissue factor.…”

Section: Introductionmentioning

confidence: 99%

IgE-mediated mechanisms in bullous pemphigoid and other autoimmune bullous diseases

Beek

Schulze

Zillikens

et al. 2015

Expert Review of Clinical Immunology

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Autoimmune bullous diseases (AIBDs) are characterized by autoantibodies against structural proteins of the dermal-epidermal junction (in pemphigoid diseases) and the epidermal/ epithelial desmosomes (in pemphigus diseases). By far, the most common AIBD is bullous pemphigoid, which is immunopathologically characterized by autoantibodies against BP180 (type XVII collagen) and BP230. IgG and, to a lesser extent, IgA autoantibodies are the major autoantibody isotypes in these disorders. IgE autoantibodies are increasingly reported in particular in bullous pemphigoid. The development of specific and sensitive anti-BP180 IgE ELISA systems, the report of two experimental murine models employing IgE autoantibodies against BP180, and the successful treatment of bullous pemphigoid with the anti-IgE antibody omalizumab have raised interest in the role of IgE autoantibodies and the modulation of their production in AIBDs. Here, the relevance of IgE autoantibodies in the diagnosis, pathophysiology, and treatment decisions of AIBDs, with a focus on bullous pemphigoid, is reviewed. ARTICLE HISTORY

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“…In a lesional biopsy, i.e. when a split formation can be seen microscopically, direct IF microscopy can be false negative due to the proteolytic degradation of Ig deposits at the BMZ or false positive due to unspecific binding if autoantibodies at the BMZ of the microscopic splitting [123].…”

Section: Direct If Microscopymentioning

confidence: 99%

“…Standard substrates for this technique are monkey or rabbit esophagus as well as normal human skin in which subepidermal splitting was induced by incubation with 1 M NaCl solution ( Figure 7) [3,123,131]. As in all pemphigoid diseases, antitype VII collagen antibodies bind along the BMZ on the monkey and rabbit esophagus.…”

Section: Indirect If Microscopymentioning

confidence: 99%

Clinical features and diagnosis of epidermolysis bullosa acquisita

Vorobyev

Ludwig

Schmidt

2016

Expert Review of Clinical Immunology

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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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S2k guideline for the diagnosis of pemphigus vulgaris/foliaceus and bullous pemphigoid

Cited by 111 publications

References 126 publications

Trichoscopy – a useful tool in the preliminary differential diagnosis of autoimmune bullous diseases

Trichoscopy – a useful tool in the preliminary differential diagnosis of autoimmune bullous diseases

IgE-mediated mechanisms in bullous pemphigoid and other autoimmune bullous diseases

Clinical features and diagnosis of epidermolysis bullosa acquisita

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