Chronic bullous disease of childhood with IgG reactivity to p200 antigen

Patsatsi, Aikaterini; Meltzanidou, Parthena; Katafigiotis, Socrates; Sotiriadis, Dimitrios; Schmidt, Enno; Lambropoulos, Alexandros

doi:10.1111/ijd.13616

Cited by 7 publications

(8 citation statements)

References 14 publications

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“…DIF microscopy of perilesional skin was reported in 98 (88.1%) patients. Deposition of IgG and C3 was observed in 77 (78.6%), whereas the simultaneous deposition of IgG, IgA, and C3 was observed in 10 (10.2%) patients (3, 9, 12, 14, 20, 30). Deposition of IgG or C3 alone was observed in two (2.0%) (16, 31) and four (4.1%) patients (22, 32–34), respectively.…”

Section: Resultsmentioning

confidence: 98%

“…The mean age at onset was 65.5 years (range 5–94) (9, 10). The majority of the patients were males ( n = 85; 75.2%) and of Asian ancestry ( n = 57, 50.4%; Table 1).…”

Section: Resultsmentioning

confidence: 99%

“…The clinical presentation was described as resembling other subepidermal AIBD and inflammatory dermatoses by authors in 68 (60.2%) patients. The leading similar condition was BP ( n = 45; 66.2%), followed by linear IgA bullous dermatosis (LABD; n = 5; 7.4%) (9, 11–14), epidermolysis bullosa acquisita (EBA; n = 3; 4.4%) (15–17), dermatitis herpetiformis (DH; n = 3; 4.4%) (18–20), mucous membrane pemphigoid ( n = 3; 4.4%) (21–23), and others (Table 2). In the remaining 45 patients, a similarity to a distinct clinical entity was not mentioned.…”

Section: Resultsmentioning

confidence: 99%

“…In the remaining two patients, immunoblotting showed reactivity only to the recombinant C-terminus of laminin γ1 (38, 39). Of significant interest is that the sera of patients who were positive for antibodies when tested using immunoblot analysis with the recombinant C-terminus of laminin γ1 was positive in 17 of 24, with a 70.8% sensitivity (3, 9, 31, 38–43). Sera of 10 patients was tested by ELISA bound to a recombinant monomeric C-terminal fragment of human laminin γ1 (10, 44, 45) (Table 3).…”

Section: Resultsmentioning

confidence: 99%

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Anti-p200 Pemphigoid: A Systematic Review

Kridin

Ahmed

2019

Front. Immunol.

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The many clinical aspects of anti-p200 pemphigoid are not well-characterized. We aimed to analyze and correlate known existing data on the epidemiological, clinical, histological, and immunological features of anti-p200 pemphigoid. We performed a review using Medline, Embase, and Web of Science databases (1900–2018). Case reports and series of patients were included. A total of 68 eligible studies that comprised 113 anti-p200 pemphigoid patients were included in the qualitative analysis, where there was a mean age of onset of 65.5 years. All patients presented with bullae/vesicles, and 54.3% had urticarial plaques. A similarity to bullous pemphigoid was reported in 66.1% of cases, but palmoplantar (51.4%), cephalic (40.3%), and mucosal (38.5%) involvement, besides frequent development of scars/milia (15.7%), were reported. Autoantibodies against recombinant laminin γ1 were detected in the sera of 73.1% of patients. Psoriasis was present in 28.3% of anti-p200 pemphigoid patients, particularly among Japanese patients (56.4%). The incidence of pustular psoriasis in this subgroup, was significantly greater than in the normal population. In conclusion, the diagnosis of anti-p200 pemphigoid may be suspected when a subepidermal autoimmune blistering disease develops in a younger age group, along with significant acral and cephalic distribution and mucosal involvement.

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Section: Resultsmentioning

confidence: 98%

“…The mean age at onset was 65.5 years (range 5–94) (9, 10). The majority of the patients were males ( n = 85; 75.2%) and of Asian ancestry ( n = 57, 50.4%; Table 1).…”

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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Anti-p200 Pemphigoid: A Systematic Review

Kridin

Ahmed

2019

Front. Immunol.

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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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Autoimmune Bullous Diseases

Beek

Schmidt

2019

Harper's Textbook of Pediatric Dermatology

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Chronic bullous disease of childhood with IgG reactivity to p200 antigen

Cited by 7 publications

References 14 publications

Anti-p200 Pemphigoid: A Systematic Review

Anti-p200 Pemphigoid: A Systematic Review

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

Autoimmune Bullous Diseases

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