A Recalcitrant Case of Senear-Usher Syndrome Treated With Rituximab.

Area of Science:

• Dermatology
• Autoimmune Diseases
• Immunology

Background:

• Pemphigus erythematosus is a rare, chronic autoimmune blistering disease.
• It is characterized by autoantibodies targeting desmogleins, leading to skin blistering.
• This condition often presents with overlapping features of pemphigus foliaceus and lupus erythematosus.

Observation:

• A 51-year-old male presented with pruritic, scaly plaques and photosensitivity.
• Scalp biopsy revealed acantholysis consistent with pemphigus foliaceus.
• Laboratory tests showed elevated anti-desmoglein 1, positive ANA, anti-dsDNA, and anti-SS-A antibodies.

Findings:

• The patient was diagnosed with pemphigus erythematosus.
• Initial treatment with systemic corticosteroids and mycophenolate mofetil was suboptimal.
• Rituximab, administered under a rheumatoid arthritis protocol, led to complete resolution of cutaneous lesions within three months.

Implications:

• Rituximab demonstrates significant efficacy as a rescue therapy for refractory pemphigus erythematosus.
• This case highlights the potential of B-cell depletion therapy in managing severe autoimmune blistering diseases.
• Further research into rituximab's role in pemphigus erythematosus is warranted.