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Related Experiment Video

Updated: Oct 6, 2025

Technique of Conjunctival Biopsy and Direct Immunofluorescence for Diagnosing Mucous Membrane Pemphigoid
05:05

Technique of Conjunctival Biopsy and Direct Immunofluorescence for Diagnosing Mucous Membrane Pemphigoid

Published on: June 17, 2025

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Brunsting-Perry pemphigoid: a systematic review.

Italo Francesco Aromolo1,2, Carlo Alberto Maronese1,2, Chiara Moltrasio1

  • 1Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

International Journal of Dermatology
|January 20, 2022
PubMed
Summary

Brunsting-Perry pemphigoid (BPP) is a rare autoimmune skin disorder. Diagnosis can be guided by direct immunofluorescence and specific autoantibodies, despite its uncertain nosologic position.

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Distinctive Reactivity to the C-terminal Epitope of BP180 Characterizes Immune Checkpoint Inhibitor-associated Bullous Pemphigoid, and an ELISA Based on the BP180 Ectodomain Enables Prompt Diagnosis in a Subset of Patients.

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Area of Science:

  • Dermatology
  • Autoimmune Diseases
  • Immunology

Background:

  • Brunsting-Perry pemphigoid (BPP) is a rare autoimmune bullous skin disorder.
  • It is classified within the spectrum of mucous membrane pemphigoid (MMP).

Purpose of the Study:

  • To review and characterize Brunsting-Perry pemphigoid (BPP).
  • To clarify the diagnostic criteria and nosologic position of BPP.

Main Methods:

  • A systematic literature review was conducted using PRISMA guidelines.
  • PubMed and Scopus databases were searched for English-language articles on BPP published between 1950 and July 2021.

Main Results:

  • Analysis of 36 articles involving 63 BPP patients revealed characteristic skin lesions including blisters, erosions, scars, and milia.

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  • Subepidermal blistering with eosinophilic infiltrate was constant; direct immunofluorescence was positive in 92.0% of cases, typically showing linear IgG deposits.
  • Target antigens frequently identified included BP180 (80.0%), BP230 (33.3%), and laminin 332 (20.0%).

    • Conclusions:

    • The nosologic position of BPP remains uncertain due to overlap with other autoimmune bullous diseases.
    • Oral mucosal lesions can occur, usually after cutaneous manifestations.
    • Direct immunofluorescence positivity and anti-BP180/230 autoantibodies, in the absence of anti-collagen VII antibodies, may aid in BPP diagnosis.