Anti-p200 pemphigoid

Holtsche, Maike M.; Goletz, Stephanie; Zillikens, Detlef

doi:10.1007/s00105-019-4376-x

Cited by 8 publications

(4 citation statements)

References 82 publications

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“…The LAD-like subtype is characterized by grouped blisters in an annular configuration. Palms and soles are frequently involved, whereas mucous membranes are affected in approximately 30%–40% of patients ( Figures 4D–F ), and an association with comorbid psoriasis, pronounced in Japanese patients, has been reported ( 43 – 46 ).…”

Section: Clinical Picturementioning

confidence: 97%

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State-of-the-art diagnosis of autoimmune blistering diseases

van Beek,

Holtsche,

Atefi

et al. 2024

Front. Immunol.

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Autoimmune blistering disorders (AIBDs) are a heterogeneous group of approximately a dozen entities comprising pemphigus and pemphigoid disorders and dermatitis herpetiformis. The exact diagnosis of AIBDs is critical for both prognosis and treatment and is based on the clinical appearance combined with the detection of tissue-bound and circulating autoantibodies. While blisters and erosions on the skin and/or inspectable mucosal surfaces are typical, lesions may be highly variable with erythematous, urticarial, prurigo-like, or eczematous manifestations. While direct immunofluorescence microscopy (IFM) of a perilesional biopsy is still the diagnostic gold standard, the molecular identification of the major target antigens opened novel therapeutic avenues. At present, most AIBDs can be diagnosed by the detection of autoantigen-specific serum antibodies by enzyme-linked immunosorbent assay (ELISA) or indirect IFM when the clinical picture is known. This is achieved by easily available and highly specific and sensitive assays employing recombinant immunodominant fragments of the major target antigens, i.e., desmoglein 1 (for pemphigus foliaceus), desmoglein 3 (for pemphigus vulgaris), envoplakin (for paraneoplastic pemphigus), BP180/type XVII collagen (for bullous pemphigoid, pemphigoid gestationis, and mucous membrane pemphigoid), laminin 332 (for mucous membrane pemphigoid), laminin β4 (for anti-p200 pemphigoid), type VII collagen (for epidermolysis bullosa acquisita and mucous membrane pemphigoid), and transglutaminase 3 (for dermatitis herpetiformis). Indirect IFM on tissue substrates and in-house ELISA and immunoblot tests are required to detect autoantibodies in some AIBD patients including those with linear IgA disease. Here, a straightforward modern approach to diagnosing AIBDs is presented including diagnostic criteria according to national and international guidelines supplemented by long-term in-house expertise.

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Section: Clinical Picturementioning

confidence: 97%

“…While by direct IFM and histopathology anti-p200 pemphigoid mainly resembles BP, its dermal reactivity on salt-split skin distinguishes it from BP ( 43 , 45 ). Direct IFM serration pattern is n-serrated, thus excluding EBA as a differential diagnosis ( 60 , 164 ).…”

Section: Diagnostic Criteriamentioning

confidence: 99%

State-of-the-art diagnosis of autoimmune blistering diseases

van Beek,

Holtsche,

Atefi

et al. 2024

Front. Immunol.

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“…• Epidermolysis bullosa acquisita (EBA) is a rare and clinically very heterogeneous PD, but due to the availability of pre-clinical model systems it is wellstudied (7,8). • Anti-p200/anti-laminin γ1 pemphigoid (p200) clinically mimics BP, but patients are younger and p200 usually responds well to treatment (9). • Lichen planus pemphigoides (LPP) is, like BP, caused by anti-BP180 antibodies, but in LPP these occur together with lichen planus.…”

mentioning

confidence: 99%

“…Anti-p200/anti-laminin γ1 pemphigoid (p200) clinically mimics BP, but patients are younger and p200 usually responds well to treatment ( 9 ).…”

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confidence: 99%

Drug Development in Pemphigoid Diseases

Bieber¹,

Ludwig²

2020

Acta Derm Venerol

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Despite detailed insights into the pathogenesis of pemphigoid diseases, their treatment still relies on unspecific immunosuppression. Since such treatment contributes significantly to the high patient morbidity and increased mortality, we propose pathways that may facilitate drug development for pemphigoid diseases. With this we aim to foster translational research to develop new treatment strategies for patients with pemphigoid diseases.Pemphigoid diseases are organ-specific autoimmune diseases of the skin and/or mucous membranes. They are caused by autoantibodies targeting adhesion molecules located at the dermal-epidermal junction. While the diagnostics of pemphigoid diseases and insights into their pathogenesis have improved significantly, the development of novel treatments that are effective and safe remains an unmet medical need. However, numerous pre-clinical studies and early clinical trials have recently been launched. This review summarizes some pathways leading to drug development in pemphigoid diseases, namely: (i) hypothesis-driven drug development; (ii) omics-based drug development; (iii) drug repurposing; (iv) screening-based drug development; and (v) drug development based on careful clinical observations. Ultimately, it is hoped that this will lead to personalized and curative treatments. Theme issue: Blistering skin disorders Advances in dermatology and venereology Acta Dermato-Venereologica 113 Drug development in pemphigoid diseases Theme issue: Blistering skin disordersof translational research in this field, which is expected to improve the treatment of patients with PD in the future. New drug approvals, more clinical trials, and personalized and curative treatments are expected.

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