Anti-P 200 pemphigoid – The most common floor binding subepidermal autoimmune bullous disease in a tertiary care center in south India

Rai, Reena; Anand, Jawahar; Shanmugasekar, C; Arunprasath, P; Chaitra, V; Zillikens, Detlef; Schimdt, Enno

doi:10.25259/ijdvl_79_20

Cited by 5 publications

(3 citation statements)

References 41 publications

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“…In anti-p200 pemphigoid, direct immunofluorescence (DIF) using patient perilesional skin/mucosa usually shows linear deposits of IgG and C3 to the BMZ ( 9 – 13 ). Indirect immunofluorescence (IIF) using normal human skin detects circulating IgG antibodies to the BMZ ( 14 – 17 ), which reacts with dermal side of 1M NaCl-split normal human skin (ssIIF) ( 14 , 18 – 21 ). Immunoblotting (IB) using normal human dermal extract detects IgG reactivity with p200 ( 14 , 22 – 25 ).…”

Section: Introductionmentioning

confidence: 99%

Potential contribution of anti-p200 autoantibodies to mucosal lesions in anti-p200 pemphigoid

et al. 2023

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Anti-p200 pemphigoid is a relatively rare subepidermal autoimmune bullous disease (AIBD), which was firstly reported by Detlef Zillikens, Takashi Hashimoto and others in 1996. Skin lesions are considered as the major clinical features of this disease, with occasional involvement of mucosal lesions. The mechanism of mucosal lesions involved in anti-p200 pemphigoid is still unclear. In the present study, we aimed to analyze published data on cases and case series of anti-p200 pemphigoid with mucosal lesions and explored the potential contribution of anti-p200 autoantibodies to mucosal lesions. A total of 32 papers that comprised 52 anti-p200 pemphigoid patients with various mucosal lesions were included in this review. Oral lesions were involved in 75.0% patients, followed by genital lesions (26.9%) and ocular lesions (11.54%). Only one patient had psoriasis, 26.9% patients had multiple mucosal lesions, and 30.8% cases had comorbidity of other AIBDs, particularly anti-laminin (LM) 332-type mucous membrane pemphigoid (MMP). In comparison with anti-LM332-type MMP, anti-BP180-type MMP and epidermolysis bullosa acquisita, higher frequency of genital lesions was identified as a unique character of anti-p200 pemphigoid with mucosal lesions. These results indicated that anti-p200 autoantibodies might contribute to mucosal lesions in a pattern different from other MMP-related autoantibodies, although its pathogenetic mechanisms are still unclear.

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Section: Introductionmentioning

confidence: 99%

Potential contribution of anti-p200 autoantibodies to mucosal lesions in anti-p200 pemphigoid

et al. 2023

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“…Additional testing using immunoblotting is essential to categorize these lesions 14 . Immunoblotting with recombinant C‐terminus of laminin γ1 and dermal extract showed IgG4 reactivity against both, a 200‐kDa protein in five (71.4%) of these cases, diagnosing them as anti‐p200 pemphigoid 18 . Lau and Goletz in their study to determine the frequency of pemphigoid diseases with dermal binding antibodies on SSS reported anti‐p200 pemphigoid as the most frequent (78.4%) pemphigoid disease 19,20 .…”

Section: Discussionmentioning

confidence: 99%

Utility of the pattern‐based approach using BIOCHIP‐indirect immunofluorescence in the evaluation of subepidermal bullous disorders

et al. 2022

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Background Subepidermal bullous disorders (SEBD) are a heterogeneous group of vesiculobullous diseases because of antibody‐mediated destruction of proteins of the dermo‐epidermal junction. Direct immunofluorescence (DIF) is the gold standard for diagnosis. BIOCHIP‐indirect immunofluorescence (IIF) is a novel serological test that combines multiple target antigens in a single field. The present study aimed to evaluate the utility of the pattern‐based approach in BIOCHIP‐IIF for the diagnosis of SEBD. Methods Seventy cases of BIOCHIP‐IIF that showed clinical, histopathological, and/or DIF features favoring SEBD were included in the study. The interpretation in the BIOCHIP was categorized into one of the following patterns. Pattern I: basement membrane zone (BMZ) staining in monkey esophagus (ME), primate salt‐split skin (SSS)‐roof staining, BP180+ and/or BP230+; Pattern II: roof staining in SSS, BP180– and BP230– with or without BMZ staining in ME; Pattern III: floor staining in SSS, BP180– and BP230–; and pattern IV: negative in SSS and other substrates. The findings were correlated with histopathology and/or DIF. Results Fifty (71.5%) cases showed pattern I or the typical bullous pemphigoid (BP) pattern. Eight (11.4%) cases showed pattern II. Patterns III and IV were observed in seven (10%) and five (7.1%) cases, respectively. BP was the most common diagnosis in patterns I and II, while anti‐p200 pemphigoid was most common in pattern III, as confirmed by immunoblotting. The sensitivity of pattern I in the diagnosis of BP was 96%. Conclusion BIOCHIP‐IIF showed a good correlation with DIF and histopathology in the diagnosis of SEBD. This can be used as a first‐line investigation in case of bullous disorders.

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“…According to the study conducted by Dainichi et al, a sizable portion of patients with anti-lamg1 pemphigoid could have been misdiagnosed as epidermolysis bullosa acquisita (EBA) (4). Additionally, in recently published studies, anti-lamg1 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)(6)(7). Moreover, a clear male predominance of approximately 75% of 113 patients with anti-lamg1 pemphigoid was summarized by Kridin et al (8,9).…”

Section: Introductionmentioning

confidence: 99%

Coexistence of Anti-p200 Pemphigoid and Psoriasis: A Systematic Review

Xie

Wang

et al. 2022

Front. Immunol.

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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.

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Anti-P 200 pemphigoid – The most common floor binding subepidermal autoimmune bullous disease in a tertiary care center in south India

Cited by 5 publications

References 41 publications

Potential contribution of anti-p200 autoantibodies to mucosal lesions in anti-p200 pemphigoid

Potential contribution of anti-p200 autoantibodies to mucosal lesions in anti-p200 pemphigoid

Utility of the pattern‐based approach using BIOCHIP‐indirect immunofluorescence in the evaluation of subepidermal bullous disorders

Coexistence of Anti-p200 Pemphigoid and Psoriasis: A Systematic Review

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