Abstract

BackgroundA close association between psoriasis and anti-p200 pemphigoid has been demonstrated by numerous studies. However, the clinical characteristics of patients suffering from these two entities have not yet been well-elucidated.ObjectiveThis study aimed to review the case reports and case series, summarizing clinical features and therapeutic strategies in patients suffering from anti-p200 pemphigoid and psoriasis.MethodsA systematic review was conducted by searching PubMed, EMBASE, and Web of Science databases for studies published in English involving patients with psoriasis and anti-p200 pemphigoid on 6 September 2021. All case reports and case series reporting patients diagnosed with anti-p200 pemphigoid and psoriasis were included in this systematic review.ResultsA total of 21 eligible studies comprising 26 anti-p200 pemphigoid patients with preceding psoriasis were included in the qualitative synthesis. The average age at blisters eruption was 62.5 years, and the mean duration between the two entities was 15.6 years. Twenty-four percent of patients developed bullous lesions during UV therapy. Clinical manifestation of bullae and/or vesicles was recorded in all patients, and the trunk (94.7%) was most frequently involved, with only 15.8% reporting mucosal involvement. Epitope spreading was detected by immunoblotting in 33.3% of patients. All the patients reached completed remission during the course of disease, with 36.8% experiencing at least one relapse. Monotherapy of prednisolone was the leading therapeutic approach (n=6, 31.6%) required for disease control, but 5 (83.3%) of them suffered from blister recurrence after tapering or ceasing corticosteroid.ConclusionMost of the clinical aspects of patients with anti-p200 pemphigoid and psoriasis were similar to what was demonstrated in previous articles on anti-p200 pemphigoid. Nevertheless, compared with other anti-p200 pemphigoid cases without psoriasis, a clinical manifestation pattern with more frequent involvement of the trunk and less mucosal involvement was illustrated in those with psoriasis. Generally, monotherapy is sufficient for a complete remission for such patients. However, one or more relapses have been recorded in a considerable portion of patients, especially those prescribed with prednisolone. It reminded us to be more cautious during a tapering of medication.

Highlights

  • Anti-p200 pemphigoid, a rare subset of autoimmune bullous disease (AIBD), was initially reported in 1996 by Japanese researchers [1, 2]

  • Results of DIF and salt-split skin (SSS)-indirect immunofluorescence (IIF) were demonstrated in 24 cases

  • On SSSIIF, an exclusively floor-binding picture was recorded in 18 cases, both roof- and floor-binding pattern in six patients [20, 25, 30, 32, 33, 36]

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Summary

Introduction

Anti-p200 pemphigoid, a rare subset of autoimmune bullous disease (AIBD), was initially reported in 1996 by Japanese researchers [1, 2]. Due to its rarity and unavailable detective technology in most countries, the prevalence of anti-lamg1/p200 pemphigoid has not been classified. According to the study conducted by Dainichi et al, a sizable portion of patients with anti-lamg pemphigoid could have been misdiagnosed as epidermolysis bullosa acquisita (EBA) [4]. In recently published studies, anti-lamg pemphigoid was considered to be the most common floor-binding subepidermal AIBD, of which detection by indirect immunofluorescence (IIF) microscopy on human salt-split skin (SSS) illustrated serum autoantibodies targeting at the dermal side of split [5–7]. The mean age of anti-lamg pemphigoid patients was 65.5 years (range 5–94) [9], younger than those with bullous pemphigoid (BP). The clinical characteristics of patients suffering from these two entities have not yet been well-elucidated

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