Brunsting‐Perry pemphigoid: a systematic review

Abstract

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.

Brunsting‐Perry type pemphigoid with IgG antibody against full‐length BP180 mimicking squamous cell carcinoma

“…In a previous study of 63 patients with BPP, the most common localization was the scalp, presenting with vesiculobullous lesions on an erythematous base or skin ulcerations. 1 BPP can present solitary and scaly lesions without vesiculobullous lesions, which is why it is sometimes mistaken for actinic keratosis or basal cell carcinoma. 4,5 To the best of our knowledge, cases similar to ours with SCC-like granulated tissue have not been previously reported.…”

“…Several potential triggering factors in BPP have been reported, including mechanical trauma, drugs (e.g., dipeptidyl peptidase-4 inhibitor, sartans), and sun exposure. [1][2][3] In our case, scratching the itchy scalp may have formed a skin ulcer, which was enlarged by subsequent scratching.…”

“…Dear Editor, Brunsting-Perry type pemphigoid (BPP) is a rare variant of autoimmune bullous dermatosis that presents with vesiculobullous lesions on an erythematous base, evolving in scars, and is limited to the scalp, face, and neck. [1][2][3] Here, we present a case of BPP resembling squamous cell carcinoma (SCC).…”

“…Autoantibodies of BPP such as BP180 NC16a, BP230, C-terminal portion of BP180, LAD-1, laminin 332, and/or type VII collagen have been reported. [1][2][3] In particular, BP180 was the most common BPP autoantibody and only a few cases of BPP showed positive reactivity other than against the BP180 NC16a domain. [1][2][3] In our case, IgG antibody reactivity against the BP180 NC16a domain was negative, whereas IgG antibody reactivity to full-length BP180 recombinant proteins was positive, indicating the immunological diversity of antibodies against basement membrane zone proteins.…”

Case Report: Bullous Pemphigoid Associated With Morphea and Lichen Sclerosus: Coincidental Diseases or Pathogenetic Association?

“…Classic clinical presentation of BP features erythema, urticarial plaques, blisters and erosions; non-bullous variants, including eczematous or prurigo-like forms, have been also described ( 8 ). Rare variants include Brusting-Perry pemphigoid ( 9 ) and laminin γ1 pemphigoid ( 10 ).…”