Autoimmune bullous diseases: pemphigoid and pemphigus
Bullous pemphigoid and pemphigus vulgaris compared; tense versus flaccid blisters, the two biopsies and direct immunofluorescence patterns, gliptin and checkpoint inhibitor triggers, superpotent topical steroid and doxycycline (BLISTER) for pemphigoid, rituximab first line for pemphigus, and pre-immunosuppression screening.
Tier 2Condition
Written with AI assistance and fact-checked against the sources below. For exam study, not clinical decisions. How notes are made
At a glance
- Bullous pemphigoid (BP) is the commonest autoimmune blistering disease. It affects people over 70, causes tense blisters on an itchy, urticarial base and rarely involves the mouth.
- Pemphigus vulgaris (PV) is rarer, affects younger adults (40 to 60), starts with painful oral erosions and causes flaccid blisters that break easily.
- Both need two biopsies: histology and direct immunofluorescence (DIF). Serology confirms the target antigen.
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Sources
- Therapeutic Guidelines: Dermatology (blistering disorders)AU/NZTherapeutic Guidelines Limited (eTG complete), current eTG edition (topic version not verified)
- Rituximab biosimilar medicine fact sheet: unrestricted PBS (S100 Highly Specialised Drugs) listing from 1 September 2022AU/NZAustralian Government Department of Health and Aged Care, 2022
- Borradori L et al. Updated S2K guidelines for the management of bullous pemphigoid initiated by the European Academy of Dermatology and Venereology (EADV). J Eur Acad Dermatol Venereol 2022;36:1689-704European Academy of Dermatology and Venereology, 2022 (no later EADV update found, November 2026)
- Joly P et al. Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the European Academy of Dermatology and Venereology (EADV). J Eur Acad Dermatol Venereol 2020;34:1900-13European Academy of Dermatology and Venereology, 2020
- Venning VA et al. British Association of Dermatologists' guidelines for the management of bullous pemphigoid 2012. Br J Dermatol 2012;167:1200-14British Association of Dermatologists, 2012 (no newer BAD edition found, November 2026)
- Harman KE et al. British Association of Dermatologists' guidelines for the management of pemphigus vulgaris 2017. Br J Dermatol 2017;177:1170-201British Association of Dermatologists, 2017
- Williams HC et al. Doxycycline versus prednisolone as an initial treatment strategy for bullous pemphigoid (BLISTER). Lancet 2017;389:1630-8The Lancet, 2017
- Joly P et al. A comparison of oral and topical corticosteroids in patients with bullous pemphigoid. N Engl J Med 2002;346:321-7New England Journal of Medicine, 2002
- Joly P et al. First-line rituximab combined with short-term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3). Lancet 2017;389:2031-40The Lancet, 2017
- Werth VP et al. Rituximab versus mycophenolate mofetil in patients with pemphigus vulgaris (PEMPHIX). N Engl J Med 2021;384:2295-305New England Journal of Medicine, 2021
- Murrell DF et al. Study design of a phase 2/3 randomized controlled trial of dupilumab in adults with bullous pemphigoid (LIBERTY-BP ADEPT). Adv Ther 2024;41:2991-3002Advances in Therapy, 2024 (trial protocol)
- Dupixent (dupilumab) US Prescribing Information, section 14 (bullous pemphigoid, ADEPT: sustained remission at week 36 18.3% vs 6.1%)US Food and Drug Administration (DailyMed), Revised April 2026
- Last updated
- 14 November 2026
What changed
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