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pemphigus-vulgaris
Pemphigus Vulgaris
Pemphigus vulgaris (PV) is an IgG4-mediated autoimmune blistering disease targeting Dsg3 (mucous membranes) and Dsg1 (skin); suprabasal acantholysis. Rituximab (PEMPHIX: 90% vs 28% CR; FDA Jun 2018) and efgartigimod (ADHERE-SC; FDA Oct 2023) are approved therapies.
Entry Metadata
| Field | Value |
|---|---|
| ID | pemphigus-vulgaris |
| Name | Pemphigus Vulgaris |
| Status | draft |
| Last reviewed | 2026-06-07 |
| Atlas | 01-human |
| Scale | 07-system |
Cross-Atlas Connections
Sources
- Joly P, Maho-Vaillant M, Prost-Squarcioni C, et al. First-line rituximab combined with short-term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3): a prospective, multicentre, parallel-group, open-label randomised trial. Lancet. 2017;389(10083):2031-2040. · PubMed 28342637
- Murrell DF, Sprecher E, Maho-Vaillant M, et al. Efgartigimod alfa and hyaluronidase-qvfc in pemphigus vulgaris. N Engl J Med. 2024;390(5):419-430. · PubMed 38294978
- Amagai M, Tsunoda K, Zillikens D, Nagai T, Nishikawa T. The clinical phenotype of pemphigus is defined by the anti-desmoglein autoantibody profile. J Am Acad Dermatol. 1999;40(2 Pt 1):167-170. · PubMed 10025737