Widespread painful oral erosions preceding skin blisters
47-year-old female
§Chief complaint
Painful sores throughout the mouth for 2 months, cannot eat
§Medical & dental history
- 01No relevant drug history
- 02Weight loss of 6 kg due to pain
- 03Unable to brush due to pain; heavy plaque accumulation
§Clinical examination
- 01Multiple ragged, irregular erosions on the buccal mucosa, soft palate and gingiva
- 02Intact blisters rare; positive Nikolsky sign
- 03Desquamative gingivitis
- 04Two flaccid bullae on the scalp appearing last week
§ figuresFigures (1)
Fig. 1All about the skin تدعمه Google ترجمةترجمة Search DermNet NZ Search Home About Topics A–Z CME Donate Browse Contact Images Jobs Book Home » Topics A–Z » Drug-induced pemphigus Drug-induced pemphigus Author: Brian Wu, MD
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§Differential diagnosis
§Final diagnosis
Pemphigus vulgaris with mucosal-dominant presentation
Fragile blisters that rupture immediately, a positive Nikolsky sign, and a suprabasal split with acantholysis on histology, plus intercellular IgG in a fishnet pattern on direct immunofluorescence, separate pemphigus from the subepithelial pemphigoid group.
§Investigations
- 01Perilesional incisional biopsy for H&E and a separate specimen in Michel medium for direct immunofluorescence
- 02Indirect immunofluorescence and anti-desmoglein 1 and 3 ELISA
- 03Baseline FBC, glucose, bone density before steroids
§Treatment plan
- 01Systemic corticosteroids (prednisolone) as first-line
- 02Steroid-sparing immunosuppression (azathioprine or mycophenolate); rituximab increasingly first-line
- 03Topical high-potency steroid for oral lesions plus antifungal cover
- 04Nutritional support and analgesia; dermatology co-management
§Histopathology report
Suprabasal intraepithelial split with acantholytic Tzanck cells and a row-of-tombstones basal layer. Direct immunofluorescence: intercellular IgG and C3 in a fishnet pattern.
§Follow-up & outcome
- 01Oral lesions healed by 8 weeks on prednisolone and rituximab
- 02Steroid tapered; on maintenance immunosuppression
- 03Regular monitoring for steroid complications
§Clinical pearls / learning points
- 01Oral lesions precede skin lesions in most cases of pemphigus vulgaris — dentists often diagnose it first.
- 02Always take a second perilesional biopsy for direct immunofluorescence; H&E alone is not enough.
- 03Untreated pemphigus vulgaris was historically fatal; prompt referral is essential.
§References
- Murrell DF et al. Diagnosis and management of pemphigus: consensus statement.
§Similar cases
41 cases in the library