Vesiculobullous Diseases
Pemphigus Vulgaris
Autoimmune intraepithelial blistering disease with IgG autoantibodies against desmoglein-3 (and desmoglein-1).
§ figuresFigures (1)
Fig. 1All about the skin تدعمه Google ترجمةترجمة Search DermNet NZ Search Home About Topics A–Z CME Donate Browse Contact Images Jobs Book Home » Topics A–Z » Drug-induced pemphigus Drug-induced pemphigus Author: Brian Wu, MD
“Pemphigus new photo due to antihypertensive drug” · Masryyy · CC BY-SA 4.0 · Wikimedia Commons
Verified open-access images only, reproduced for education under their stated licences · no AI-generated or illustrative artwork · Pemphigus Vulgaris figures pending faculty review.
§ overviewOverview
Autoimmune intraepithelial blistering disease with IgG autoantibodies against desmoglein-3 (and desmoglein-1).
§ etiologyEtiology
- 01Autoantibodies against desmoglein-3
- 02HLA-DR4 association
§ pathogenesisPathogenesis
IgG binds desmogleins → loss of keratinocyte adhesion (acantholysis) → intraepithelial suprabasal split.
§ clinicalClinical Features
- 01Oral lesions precede skin in 50–70%
- 02Flaccid bullae that rupture to painful erosions
- 03Positive Nikolsky sign
§ differentialDifferential Diagnosis
- 01Mucous membrane pemphigoid
- 02Erythema multiforme
- 03Paraneoplastic pemphigus
- 04Erosive lichen planus
§ histopathHistopathology
- 01Suprabasal intraepithelial split
- 02Tzanck cells (acantholytic keratinocytes)
- 03Basal cells attached to basement membrane ('row of tombstones')
§ investigationsInvestigations
- 01Direct immunofluorescence — intercellular IgG/C3 ('fish-net' pattern)
- 02Indirect IF — circulating antibodies
- 03ELISA — anti-desmoglein-3 titres
§ treatmentTreatment
- 01Systemic corticosteroids (prednisolone 1 mg/kg/day)
- 02Steroid-sparing: azathioprine, mycophenolate mofetil
- 03Rituximab (first-line in moderate-severe disease per current guidelines)
§ complicationsComplications
- 01Sepsis
- 02Fluid & electrolyte imbalance
- 03Steroid side effects
§ prognosisPrognosis
Untreated 80% mortality; with therapy 5–10%.
§ pearlsClinical Pearls
- 01Rituximab has revolutionised management — early referral is essential.
§ mnemonicsMnemonics
- 01Pemphigus = 'PV kills Deep' (Desmoglein-3, suprabasal)
§ evidenceReferences & Evidence
Current Classification
WHO Classification of Tumours Online — Head and Neck
IARC / WHO · 2022
Continuously updated online edition of the WHO head and neck tumour classification.
Evidence summary
Highest level of evidence linked to this page: WHO / Official Classification.
- L1 · Classification — 1
- L2 · Guideline — 1
- L6 · Textbook — 3
2 linked sources were published from 2024 onwards. Where guidance is limited or contested, rely on the higher-level sources above rather than this summary. Educational use only — not a substitute for clinical judgement.
Content last reviewed
September 2026
Evidence last reviewed
September 2026
Next scheduled review
September 2027
Review status
EVIDENCE REVIEWED
Search latest PubMed evidence
Last literature search: 7 September 2026
Current Classification
IARC / WHO. IARC · 2022
Continuously updated online edition of the WHO head and neck tumour classification.
Clinical Guidelines
Nanda A, Tedbirt B, Bodemer C et al.. Journal of the European Academy of Dermatology and Venereology : JEADV · 2026 · 40(7):1137-1161
European S2k guideline on the management of autoimmune blistering diseases.
Histopathology References
Barnes L. CRC Press · 2008
Detailed histopathological reference for head and neck diagnostic criteria.
Textbook References
Neville BW, Damm DD, Allen CM et al.. Elsevier · 2024
Standard reference textbook of oral and maxillofacial pathology used throughout this atlas.
Rajendran R, Sivapathasundharam B. Elsevier · 2020
Undergraduate and postgraduate oral pathology textbook widely used in BDS and FCPS curricula.
Draft — pending faculty review. Educational use only; verify against current guidelines and primary sources before clinical application.