A Recalcitrant Case of Senear-Usher Syndrome Treated With Rituximab

A 51-year-old uninsured, otherwise healthy male who works in the fishing industry presented with a two-month history of pruritic scaly plaques on his face, scalp, and trunk and mild photosensitivity. A biopsy of a scalp lesion revealed acantholysis consistent with pemphigus foliaceus. Laboratory testing demonstrated elevated anti-desmoglein 1, positive antinuclear antibodies (ANA and anti-dsDNA), and elevated Sjögren's anti-SS-A antibodies. The patient was diagnosed with pemphigus erythematosus. The patient was not optimally responsive and was unable to discontinue systemic corticosteroids despite a maximum dosage of mycophenolate mofetil of 3000 mg/day. Hence, rituximab was added as a rescue treatment with the rheumatoid arthritis protocol. Three months after starting rituximab, there was a marked improvement in symptoms with complete resolution of cutaneous lesions.