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Bullous pemphigoid

Published: September 18, 2026Updated: September 18, 2026

Important: Informational content intended for healthcare professionals. It does not replace clinical evaluation or establish a doctor-patient relationship.

Overview

  • Names
    • Bullous pemphigoid (PA)
    • Bullous pemphigoid
    • Bullous pemphigoid (BP) (English name, widely used in literature) Miyamoto 2019 , Bernard 2017 .
  • History
    • Subepidermal autoimmune blistering disease described as a distinct entity in the mid-twentieth century (Lever), currently a paradigm of autoimmune blistering disease in the elderly Bernard 2017 .
  • Epidemiology
    • Most common autoimmune blistering disease in older adults Miyamoto 2019 , Bernard 2017 .
    • Annual incidence in the general population: ~ 2.4–23 cases/million ; in >80 years it can reach 190–312/million Kridin 2018 .
    • Increasing incidence (≈2–4 times in recent decades) due to population aging, better recognition of non-bullous forms and more drug-induced cases Kridin 2018 , Miyamoto 2019 .
    • Typical age: >60–70 years ; cases in young people and children are rare Miyamoto 2019 .
    • Sex: slight female or male predominance depending on the series (approx. M:F ≈ 1:1–1:1.5) Kridin 2018 .
    • High burden of comorbidity: dementia, cerebrovascular disease, heart disease, diabetes and high thrombotic risk Kridin 2018 , Kridin 2019 , Miyamoto 2019 .
  • ICD Codes
    • ICD-10: L12.0 “Bullous pemphigoid”; widely used and validated in epidemiological cohorts Leisti 2023 .
    • ICD-11: EB41.0 “Bullous pemphigoid”
  • Diagnostic checklist (quick)
    • Essential clinic
      • Age >60 years.
      • Intense itching.
      • Tight blisters on erythematous skin or urticarial plaques or chronic recalcitrant eczema/prurigo in the elderly.
    • Key background information
      • High-risk drugs (gliptins, anti-PD-1/PD-L1, diuretics, neuroleptics).
      • Relevant neurological and cardiometabolic comorbidities.
    • Targeted exploration
      • Look for tight blisters, crusts, areas of eczema/plaque, mucosal involvement.
      • Rule out signs of mucous membrane pemphigoid (ocular/oral scarring).
    • Biopsy
      • 1 lesional punch (unabridged bullet) for H&E.
      • 1 perilesional punch for IFD (physiological saline, coordinate with the laboratory for immediate collection).
    • Specific immunology
      • DIF + IIF salt-split skin.
      • ELISA Anti-BP180 (± Anti-BP230 if available)
    • Basic laboratory
      • Blood count, liver and kidney function tests, blood glucose, lipids, serology tests if immunosuppression is planned.
    • Final diagnosis
      • Integrate: compatible clinical presentation + eosinophilic subepidermal bulla + linear IgG/C3 DIF in basement membrane ± BP180/BP230 autoantibodies.

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