What are the PDAI remission criteria?

Complete remission off therapy: PDAI = 0 with no treatment for ≥2 months; complete remission on minimal therapy: PDAI = 0 on prednisolone ≤10 mg/day ± azathioprine for ≥2 months; partial remission: PDAI 0 on any therapy.

What anti-desmoglein profile correlates with pemphigus type?

Anti-Dsg3 (mucosal type): pemphigus vulgaris mucosal disease; Anti-Dsg1 + Dsg3: PV with skin + mucosa; Anti-Dsg1 only: pemphigus foliaceus (superficial skin only); anti-Dsg titers correlate with disease activity and fall with treatment.

When is rituximab first-line for pemphigus?

RITUX3 trial: rituximab (1g IV ×2) + short-course prednisolone superior to conventional prednisolone alone; complete remission at 24 months: 90% vs 34%; rituximab now first-line for moderate-severe pemphigus in EADV guidelines 2020.

What steroid dose is used for pemphigus induction?

Prednisolone 1-1.5 mg/kg/day until no new lesions for 2 weeks; taper 10 mg/2 weeks until 40 mg, then slower; azathioprine/MMF added as steroid-sparing; dexamethasone pulse (300 mg IV) for rapidly progressing disease.

How is bullous pemphigoid distinguished from pemphigus?

BP: tense blisters (subepidermal), skin >mucosa affected, older patients, anti-BP180 and BP230 antibodies, linear IIF; PV: flaccid blisters (intraepidermal), mucosa > skin, Nikolsky positive, anti-desmoglein IgG, lacy IIF pattern.

Pemphigus Severity Index and Rituximab Response Assessment

Calculates PDAI (Pemphigus Disease Area Index) for skin and mucous membranes, anti-desmoglein 1 and 3 titers for disease monitoring, and guides rituximab vs azathioprine steroid-sparing strategy.

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Frequently Asked Questions

What are the PDAI remission criteria?

What anti-desmoglein profile correlates with pemphigus type?

When is rituximab first-line for pemphigus?

What steroid dose is used for pemphigus induction?

How is bullous pemphigoid distinguished from pemphigus?