This handout is for pemphigus (vulgaris & foliaceus). Your care team identified this based on: chronic painful non-healing oral (± conjunctival/genital/oesophageal) erosions, often the first and for months the only manifestation of pemphigus vulgaris (eadv/edf s2k pemphigus guideline, jeadv 2020; pmid 32830877).
Other reasons your team may use this plan: flaccid, easily ruptured bullae on non-erythematous skin leaving raw painful erosions, with a positive nikolsky sign — the intraepidermal acantholytic phenotype (eadv/edf s2k 2020; pmid 32830877); superficial scaly-crusted "corn-flake" erosions on the scalp/face/upper trunk with no mucosal involvement — pemphigus foliaceus (anti-dsg1 only) (murrell international consensus, jaad 2018; pmid 29438767); severe intractable haemorrhagic stomatitis + polymorphous lichenoid/em-like skin eruption (± conjunctival/nail) — suspect paraneoplastic pemphigus and search for an occult neoplasm (pan, j peking univ 2022; pmid 36241242).
Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.
| Medication | Starting dose | How | When | What it does |
|---|---|---|---|---|
| withdraw_offending_thiol_or_acei_drug | — | — | — | EADV/EDF S2k pemphigus 2020 (PMID 32830877) — thiol drugs (D-penicillamine, thiopronine) and ACE inhibitors / other non-thiol drugs can induce pemphigus; withdrawing the culprit can be disease-modifying and may obviate or reduce immunosuppression. |
| erosion_wound_oral_care_nutrition_and_infection_surveillance | — | — | — | EADV/EDF S2k 2020 (PMID 32830877) — non-adherent dressings + atraumatic wound care, oral antiseptic/anaesthetic care + analgesia + nutrition for painful oral/oesophageal erosions, and active surveillance for secondary infection (the leading cause of pemphigus death). |
| treat_underlying_neoplasm_and_route_oncology_if_paraneoplastic | — | — | — | Pan J Peking Univ 2022 (PMID 36241242) + Choi J Dermatol 2012 (PMID 22938021) — paraneoplastic pemphigus prognosis depends on the underlying neoplasm; treat/resect it (Castleman/NHL/CLL/thymoma) and route to oncology; bronchiolitis obliterans + fungal infection are independent mortality risks. |
Plan: Pemphigus (vulgaris & foliaceus) — rituximab-first severity-tiered ladder (EADV/EDF S2k 2020 / Joly Ritux 3 Lancet 2017)
Contact your care team if any of the following happen:
Call 911 or go to the nearest emergency room right away if you have:
Chronic relapsing-remitting maintenance: taper corticosteroid/immunosuppressant to the lowest effective dose guided by clinical remission + anti-Dsg ELISA trend, relapse-recognition education (new oral/skin erosions), planned/relapse-triggered repeat-rituximab strategy, periodic immunosuppressant + immunoglobulin + HBV surveillance, infection precautions, oral/dental and nutrition care, and — for drug-induced pemphigus — a permanent culprit-avoidance flag. For confirmed/treated paraneoplastic pemphigus, oncology-led neoplasm surveillance + pulmonary (bronchiolitis obliterans) monitoring. Dermatology continuity for any systemic agent; reassess the diagnosis (repeat biopsy/serology, rat-bladder IIF) if the course is atypical or treatment-refractory.
Guideline: EADV/EDF updated S2k guideline for the management of pemphigus vulgaris and foliaceus (Joly, Hertl, Schmidt et al, JEADV 2020; PMID 32830877, DOI 10.1111/jdv.16752) — rituximab is FIRST-LINE for moderate-to-severe pemphigus vulgaris (approved in Europe and the USA); systemic corticosteroid for rapid control; azathioprine/MMF as steroid-sparing adjuvants; IVIG / immunoadsorption / plasmapheresis / cyclophosphamide / repeat rituximab for refractory disease; diagnosis per the autoimmune-bullous canon (perilesional intercellular DIF + lesional acantholytic H&E + anti-Dsg1/Dsg3 ELISA + IIF, rat-bladder for paraneoplastic). Concordant international expert consensus (Murrell et al, JAAD 2018; PMID 29438767 — IV CD20 inhibitor first-line). Cornerstone RCTs: Joly Ritux 3 first-line rituximab (Lancet 2017; PMID 28342637); Beissert azathioprine-vs-MMF steroid-sparing (Arch Dermatol 2006; PMID 17116835); Amagai high-dose IVIG (JAAD 2009; PMID 19293008). Activity/serology: Hébert/Joly PDAI/ABSIS validation + anti-Dsg correlation (J Invest Dermatol 2018; PMID 30301637). Paraneoplastic-pemphigus prognosis: Pan (J Peking Univ 2022; PMID 36241242) + Choi (J Dermatol 2012; PMID 22938021).