Brunsting-Perry pemphigoid: a systematic review

Int J Dermatol. 2022 Nov;61(11):1353-1358. doi: 10.1111/ijd.16045. Epub 2022 Jan 20.


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 ± 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.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Autoantibodies
  • Autoimmune Diseases*
  • Epidermolysis Bullosa Acquisita* / diagnosis
  • Epidermolysis Bullosa Acquisita* / pathology
  • Humans
  • Immunoglobulin G
  • Middle Aged
  • Pemphigoid, Bullous*
  • Skin Diseases, Vesiculobullous*


  • Autoantibodies
  • Immunoglobulin G