Successful treatment of Brunsting-Perry pemphigoid with dupilumab

Blum, Franklin R.; Sigmon, Justin R.

doi:10.1016/j.jdcr.2021.02.010

Cited by 4 publications

(3 citation statements)

References 8 publications

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“…More rare subtypes of BP treated efficiently with dupilumab include pemphigoid gestationis [ 128 ], lichen planus pemphigoides [ 129 , 130 ], Brunsting–Perry cicatricial pemphigoid [ 131 , 132 ] and pemphigoid nodularis [ 133 ]. Additionally, a case of linear IgA-dermatosis was efficiently treated with dupilumab [ 134 ].…”

Section: Resultsmentioning

confidence: 99%

Dupilumab in Inflammatory Skin Diseases: A Systematic Review

et al. 2023

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Dupilumab was first approved for the treatment of atopic dermatitis (AD) and blocks the signaling of interleukin (IL)-4 and -13. Several other chronic skin conditions share mechanistic overlaps with AD in their pathophysiology, i.e., are linked to type 2 inflammation. Most recently, dupilumab was approved by the U.S. Food and Drug Administration for prurigo nodularis (PN). Given its relatively good safety profile, effective off-label use of dupilumab has been reported for a multitude of dermatologic diseases and several clinical trials for dermatologic skin conditions are currently ongoing. We conducted a systematic review of applications of dupilumab in dermatology other than AD and PN by searching the databases PubMed/Medline, Scopus, Web of Science and Cochrane Library as well as the clinical trial registry ClinicalTrials.gov. We found several reports for effective treatment of bullous autoimmune diseases, eczema, prurigo, alopecia areata, chronic spontaneous urticaria, Netherton syndrome and a variety of other chronic inflammatory skin diseases.

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Section: Resultsmentioning

confidence: 99%

Dupilumab in Inflammatory Skin Diseases: A Systematic Review

et al. 2023

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“… 8 In a multicenter case series of 13 patients with bullous pemphigoid, disease clearance or satisfactory response was achieved in 92.3% of the patients. 8 Dupilumab monotherapy has been reported in 2 cases to significantly improve Brunsting-Perry pemphigoid, 9 , 10 a variant of cicatricial pemphigoid characterized by involvement of the head, face, and neck with rare mucous membrane involvement.…”

Section: Discussionmentioning

confidence: 99%

Dupilumab monotherapy suppresses recalcitrant pemphigus vulgaris

Moore

Hurley

2023

JAAD Case Reports

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“…Included cases were sometimes described concisely, especially in terms of histologic description, treatment, and outcome. Many reports omit drug dosages; in reviewing steroid‐sparing treatments, an experimental treatment (dupilumab) 16 and few, very old, no more currently used drugs (arsenic, gold salts, and bismuth) were included. Tests aimed at identifying the culprit autoantigen have not been performed in the majority of BPP cases, since many were reported before the advent of ELISA or immunoblot analysis.…”

Section: Discussionmentioning

confidence: 99%

Brunsting‐Perry pemphigoid: a systematic review

et al. 2022

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Background Brunsting-Perry pemphigoid (BPP) is a rare, autoimmune bullous skin disorder classified within the spectrum of mucous membrane pemphigoid (MMP). Materials and Methods An a priori protocol was designed based on PRISMA guidelines.PubMed and Scopus databases were searched for English-language articles concerning BPP published between 1950 and July 2021.Results Thirty-six articles including 63 BPP patients were analyzed. The mean age at diagnosis was 62.9 years (range: 27-86). BPP was shown to be characterized by vesiculobullous lesions (46/63, 73.0%) on an erythematous base, erosions or ulcerations (27/63, 42.9%), atrophic scars (49/63, 77.8%), and milia (4/63, 6.3%). Exclusive oral mucosal involvement was documented in 22.2% of cases, usually manifesting after the cutaneous onset of the disease. Subepidermal blistering was a constant finding, often with an eosinophil-rich inflammatory infiltrate (21/58, 36.2%). Positive direct immunofluorescence was found in 92.0% of patients, almost always with linear IgG AE C3 deposits along the basement membrane (43/46, 93.5%). BP180 (12/15, 80.0%), BP230 (5/ 15, 33.3%), and laminin 332 (3/15, 20.0%) were the most frequently identified target antigens.Conclusions BPP nosologic position remains uncertain, given the overlap with other autoimmune bullous diseases, such as MMP, bullous pemphigoid, and epidermolysis bullosa acquisita, particularly in its BPP-like variant. Nonpredominant oral mucosal lesions may appear during the course of the disease, generally after cutaneous manifestations.Positivity of DIF and anti-BP180/230 autoantibodies detected on ELISA/immunoblotting in the absence of anticollagen VII antibodies may provide guidance in diagnosing BPP.

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Successful treatment of Brunsting-Perry pemphigoid with dupilumab

Cited by 4 publications

References 8 publications

Dupilumab in Inflammatory Skin Diseases: A Systematic Review

Dupilumab in Inflammatory Skin Diseases: A Systematic Review

Dupilumab monotherapy suppresses recalcitrant pemphigus vulgaris

Brunsting‐Perry pemphigoid: a systematic review

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