Clinical and immunological features and outcome of anti-p200 pemphigoid

Commin, M.-H.; Schmidt, Enno; Duvert‐Lehembre, S.; Lasek, A.; Morice, C.; Estival, J.-L.; Debarbieux, S; Rigal, E.; Pauwels, C.; Quatrebarbes, Julie De; Roussel, A.; Goujon, E.; Stoebner, Pierre‐Emmanuel; Jouen, F.; Joly, P.

doi:10.1111/bjd.14629

Cited by 41 publications

(35 citation statements)

References 22 publications

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“…Fifty cases (44.2%) had been reported from Japan. The age of presentation in reported cases ranged between 5 and 94 years, with a mean of 65.5 years. The majority of patients were male ( n = 85; 75.2%) and of Asian ancestry ( n = 57, 50.4%; Table ).…”

Section: Resultsmentioning

confidence: 99%

Treatment and clinical outcomes in anti‐p200 pemphigoid: a systematic review

Britva

Amber

Cohen

et al. 2019

Acad Dermatol Venereol

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Background Current treatment paradigms in anti‐p200 pemphigoid rely on low levels of evidence, primarily originating from case reports and case series. Objective To systematically review the utilized treatment modalities for anti‐p200 pemphigoid and to synthesize the available clinical outcomes of treated patients. Methods We conducted a systematic review of the literature using Ovid‐Medline (1946–2018), Embase (1947–2018) and Web of Science (1900–2018) databases with a broad and inclusive search strategy along with a subsequent search of retrieved articles. All case reports and case series of patients with anti‐p200 pemphigoid were included. Results Sixty‐eight eligible studies comprising 113 anti‐p200 pemphigoid patients with a mean age of 65.5 years were included in the qualitative synthesis. The clinical outcome of patients following treatment was reported for 91 (80.5%) patients, of whom 83 (91.2%) had achieved complete remission at least once. Complete remission on‐therapy was observed in 51 (56.0%) and complete remission off‐therapy in 12 (13.2%) patients. Thirty‐six (39.6%) patients had experienced at least one flare during the duration of follow‐up. A combination of systemic corticosteroids and adjuvant immunomodulatory agents was the leading therapeutic approach (63.0%) required for disease control. Systemic and topical corticosteroids as monotherapy were sufficient to control the disease in 19.6% and 13.0% of cases, respectively. Dapsone was the most commonly used (41.3%) adjuvant agent. The highest rates of complete remission were achieved in patients managed by systemic corticosteroids as monotherapy (100%) and in those managed by systemic corticosteroids with adjuvant agents (90.7%). Conversely, 45.5% of patients treated only by topical corticosteroids experienced at least one relapse during follow‐up. Conclusion The vast majority of patients had reached a complete remission during the course of the disease, whereas a considerable proportion of patients experienced at least one relapse. A combination of systemic corticosteroids and adjuvant immunomodulatory agents was the most frequently utilized therapeutic approach.

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Section: Resultsmentioning

confidence: 99%

Treatment and clinical outcomes in anti‐p200 pemphigoid: a systematic review

Britva

Amber

Cohen

et al. 2019

Acad Dermatol Venereol

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“…This is a recently recognised disease with autoantibodies against BP 230, 180 and p200 which has probably been diagnosed over the years as BP or EBA. It shares clinical features with BP, with tense blisters, urticarial, eczematous and prurigo changes . However, it affects a younger population, with mucosal involvement in more than half and psoriasis in up to one third .…”

Section: Anti P200/anti‐laminin Gamma1 Pemphigoidmentioning

confidence: 97%

“…Immunoblotting reveals a 200 kDa antigen . Treatments include clobetasol propionate, systemic steroids, and steroid sparing agents like MTX, dapsone …”

Section: Anti P200/anti‐laminin Gamma1 Pemphigoidmentioning

confidence: 99%

Review of autoimmune blistering diseases: the Pemphigoid diseases

Daniel

Murrell

2019

Acad Dermatol Venereol

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Autoimmune Blistering Diseases of the Pemphigoid type is characterised by sub‐epidermal blisters (SEB) with circulating autoantibodies against components of the basement membrane zone (BMZ). The main disorders to date include bullous pemphigoid (BP), pemphigoid gestationis, mucous membrane pemphigoid (MMP), epidermolysis bullosa acquisita (EBA), linear IgA disease (LABD), dermatitis herpetiformis (DH), lichen planus pemphigoides and bullous lupus. This is in contrast to pemphigus and related disorders, which demonstrate intraepidermal acantholysis and a positive Nikolsky sign. The classification and management is based on clinical, histological and direct and indirect immunofluorescence findings. There are, however, overlapping clinical and histological features between the conditions and clinical heterogeneity within each disease.

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“…This disorder was initially considered as a more benign condition compared with bullous pemphigoid and epidermolysis bullosa acquisita, but in a recent series of patients, heterogeneous clinical presentations have been described, including cases with a more severe course than previously reported 2 . We describe a case with a highly active disease in a heart transplant patient who experienced a dramatic and complete remission after 1 cycle of rituximab.…”

Section: Introductionmentioning

confidence: 87%