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Pemphigus vulgaris

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
    • Main name: Pemphigus vulgaris (PV) / Pemphigus vulgaris.
    • Other: Deep pemphigus, mucocutaneous pemphigus.
    • Variant form: pemphigus vegetans (Neumann, Hallopeau) as a subtype of PV. Buonavoglia 2019 [show_150ms_ease-in] » data-testid=»webpage-citation-pill»> PubMed
  • History
    • Clinical descriptions of compatible conditions from the 17th–18th centuries (Koenig, Wichmann, Macbride).
    • Classic review of the history and evolution of the concept: King & Holubar, 1983. King 1983
  • Epidemiology
    • Rare disease. Average global incidence ≈ 0.5–3 cases/million/year , with geographical variation (highest peaks in the Mediterranean, Middle East, India, Latin America). K ridin 2021 , Zhao 2023
    • It accounts for ~70–80% of all pemphigus cases. Pollmann 2018
    • Typical age of onset: 40–60 years , but it can occur outside this range. Kridin 2021
    • Ethnic preference:
      • Higher frequency among Ashkenazi Jews , populations of the Mediterranean, the Middle East, northern India, and Iran . Kridin 2021
    • Sex: slight female predominance or similar distribution depending on the series. Zhao 2023
    • Current mortality with specialized treatment: ~5–15% , mainly due to infections and complications of immunosuppression. Pollmann 2018
  • Codes
    • ICD-10: L10.0 – Pemphigus vulgaris.
    • ICD-11: EB40.0 – Pemphigus vulgaris (subcategories EB40.00, EB40.0Y, EB40.0Z).
  • Diagnostic checklist (quick)
    • Essential clinic
      • Painful oral erosions ± flaccid blisters/extensive skin erosions.
      • Nikolsky/Asboe-Hansen sign (if possible).
    • Histology
      • Biopsy of recent blister / edge → suprabasal blister + acantholysis + “row of tombstones”.
    • Immunopathology
      • Perilesional IFD: Intercellular IgG ± C3 in epidermal reticular pattern.
      • IFI and/or ELISA anti-Dsg3 ± Dsg1 positive.
    • Basic laboratory tests prior to immunosuppression
      • Blood count, liver/kidney function, glucose/lipids.
      • HIV, HBV, HCV serologies, latent TB test.
    • Biopsy – minimal technique
      • 1 punch for H&E (blister/edge).
      • 1 perilesional punch for IFD in Michel/physiological saline.

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