Management of Hematemesis in Pemphigus

Diagnostic Approach

The British Journal of Dermatology recommends obtaining biopsies during endoscopy for direct immunofluorescence if the diagnosis of pemphigus is not yet established 1

The American College of Dermatology implies that high-dose prednisolone at 1-1.5 mg/kg/day should be initiated immediately upon diagnosis of esophageal bleeding, with a switch to pulsed intravenous methylprednisolone 250-1000 mg daily for 1-3 days if bleeding persists 2
Azathioprine 2-3 mg/kg/day or mycophenolate mofetil 2-3 g/day in divided doses are first-line adjuvants, with an expected 6-8 week latent period before demonstrating clinical effect 2, 3

The British Journal of Dermatology suggests maintaining vigilance for infection and sepsis, which are major causes of mortality in pemphigus, especially with escalated immunosuppression 1, 3
Daily washing with antibacterial products can decrease colonization in patients with extensive mucosal erosions 1

The British Journal of Dermatology recommends providing adequate analgesia for both acute and maintenance pain control, as esophageal involvement causes significant odynophagia 1

Rituximab 2 x 1000 mg infusions separated by 2 weeks can be considered as third-line therapy, achieving 89% complete remission at 2 years 2, 4
Dexamethasone-cyclophosphamide pulse (DCP) therapy is an alternative, with 86% remission rates, though it carries risks of amenorrhea, azoospermia, and hemorrhagic cystitis 2, 5

The American College of Dermatology implies that prednisolone should be reduced by 5-10 mg weekly initially, tapering more slowly below 20 mg daily, with a goal of ≤10 mg daily prednisolone while continuing adjuvant immunosuppression 2, 3