Synopsis
Recognise painful mucosal and fragile cutaneous erosions, confirm intercellular autoimmunity with paired tissue and serology, and deliver rituximab-based specialist treatment while preventing infection and nutritional decline.
- Pemphigus vulgaris commonly begins with persistent painful oral erosions before skin disease; intact blisters may be rarely seen because the intraepidermal roof is fragile.
- Skin lesions are flaccid bullae or irregular raw erosions on normal or erythematous skin, often affecting scalp, face, chest, back and pressure sites.
- Gentle lateral pressure may extend an erosion, but Nikolsky sign is neither necessary nor specific and should not be repeatedly elicited on painful skin.
- Routine histology from a fresh blister edge shows suprabasal acantholysis; direct immunofluorescence from separate perilesional skin shows intercellular IgG and C3 in a net-like pattern.
- Serum desmoglein 3 antibodies align with mucosal disease and desmoglein 1 with skin involvement, but titres are interpreted alongside clinical activity and biopsy.
- Moderate-to-severe adult pemphigus vulgaris has a licensed rituximab regimen of 1000 mg intravenously on days 1 and 15 with a tapering glucocorticoid course, followed by defined maintenance when needed.
- Before rituximab, screen hepatitis B and other infection risk, update suitable vaccines, check immunoglobulins and blood counts and plan prophylaxis and reproductive safety.
- Use non-adherent wound care, bland oral products, potent topical corticosteroid for limited lesions, analgesia and dietetic support; antiseptic or antifungal treatment needs a defined indication.
- Disease control is absence of new lesions with healing of established erosions. Relapse and treatment infection can both produce deterioration, so culture and examination precede reflexive immunosuppression escalation.
Key red flags
Dysphagia, hoarseness, dyspnoea, eye pain, photophobia, reduced vision, inability to drink, rapidly increasing erosions, fever, hypotension or disproportionate skin pain requires urgent escalation and reconsideration of SJS/TEN or infection.
New purulence, malodour, warmth, fever, hypotension or disproportionate pain suggests bacterial, candidal or herpetic complication rather than immune activity alone.
Investigation priorities
Show the level of epidermal separation and acantholytic keratinocytes.
Management branches
Persistent painful mucosal erosions or fragile cutaneous bullae suggest pemphigus vulgaris.
- Map mouth, eyes, nose, pharynx, genital skin and total cutaneous erosions, assess intake and sepsis and photograph fresh lesions with consent.
- Obtain blister-edge tissue for routine histology and a separate intact perilesional sample for direct immunofluorescence before more treatment when clinically safe.
- Request desmoglein 1 and 3 serology and reconcile discordance with biopsy site, lesion age and alternative mucosal disease in a specialist service.
An adult has confirmed moderate-to-severe pemphigus vulgaris and no contraindication to B-cell depletion.
- Complete infection, hepatitis B, immunoglobulin, vaccination, blood, organ and reproductive screening and give required premedication in an infusion-capable setting.
- Administer rituximab 1000 mg intravenously on day 1 and day 15 with a tapering glucocorticoid regimen, plus PJP prophylaxis when indicated by local risk assessment.
- Assess new-lesion cessation, healing and infection; use 500 mg maintenance at months 12 and 18 and later six-monthly only when clinically needed under the product and specialist plan.
Key medicines
Rituximab intravenous infusionGive 1000 mg intravenously on day 1 and day 15 with tapering glucocorticoid; give 500 mg at months 12 and 18 and then every six months only if clinically needed.Administer with resuscitation facilities and premedication. Screen hepatitis B and infection, update vaccines beforehand, monitor immunoglobulins and late neutropenia, provide the alert card and withhold for serious infection or suspected PML.
Prednisolone with rituximab inductionUse the specialist severity-based oral regimen, commonly about 0.5 mg/kg daily in moderate or 1 mg/kg daily in severe disease, with a planned rapid response-led taper.Assess infection, diabetes, pressure, mood, bone, eye, gastrointestinal and adrenal risk; use prophylaxis where indicated, avoid abrupt cessation after sustained treatment and do not interpret infection deterioration as automatic flare.