Serration pattern analysis as a practical adjunct tool for categorization of subepidermal autoimmune blistering diseases

Arora, Sukriti; Shetty, Varsha M.; Rao, Chythra R; Pai, Satish; Rao, Raghavendra

doi:10.25259/ijdvl_1232_20

Cited by 4 publications

(4 citation statements)

References 25 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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“…Secondly, the DIF assays were not all performed in our institute, Department of Dermatology, Peking University First Hospital, Beijing, China. In addition, the patient lacks data on serration pattern analysis, a technique first reported by Vodegel et al for differentiation between AIBDs with anti-COL7 antibodies and other pemphigoid diseases ( 27 ). However, in order to follow precisely and completely the steps of the clinical and diagnostic criteria for EBA developed by the International Bullous Diseases Group ( 11 ), we performed IIF, ss-IIF, immunoblotting and ELISA, whose results were all consistent with the diagnosis of EBA.…”

Section: Discussionmentioning

confidence: 99%

Clinical Features of Paediatric Inflammatory Epidermolysis Bullosa Acquisita: A Case Series Study

Zhang,

Dang,

et al. 2024

Acta Derm Venereol

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Epidermolysis bullosa acquisita (EBA) rarely develops in childhood. This study retrospectively recruited paediatric patients with EBA (age ≤ 16 years), diagnosed by clinical and histopathological features and results of immunofluorescence, immunoblotting and enzyme-linked immunosorbent assay (ELISA), and reviews their clinical manifestations, histopathology, immunological features, and responses to various treatments. All 7 included patients presented with inflammatory EBA. Among them, 3 had a bullous pemphigoid-like phenotype. Pathologically, in addition to dermal–epidermal blistering, in all patients, the distribution of neutrophils was superficial perivascular or interstitial, or in the dermal papilla. Mixed neutrophils and eosinophils were detected in 2 of the 3 patients with bullous pemphigoid-like phenotypes. In addition to treatment with glucocorticoids, dapsone was administered in 4 patients, while thalidomide and sulfasalazine were administered in 1 patient. All patients responded to the these therapies. Relapse was mainly due to reduction and cessation of glucocorticoids. In conclusion, EBA in childhood may be unique, and thus distinct from its adult counterpart. Specific treatment and follow-up protocols are required for therapy of this rare autoimmune skin disease in children.

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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%

“…This study may provide a new IB mode for detecting anti-lamγ1 pemphigoid, and facilitate the diagnosis of this disease worldwide to a certain degree. Apart from this, several researchers attempted to apply the serration pattern analysis to the classification of AIBDs ( 7 , 14 , 15 ), which remains to be verified in larger populations.…”

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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Serration pattern analysis as a practical adjunct tool for categorization of subepidermal autoimmune blistering diseases

Cited by 4 publications

References 25 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

Clinical Features of Paediatric Inflammatory Epidermolysis Bullosa Acquisita: A Case Series Study

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

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