Treatment of pemphigus

Area of Science:

• Dermatology
• Immunology
• Pharmacology

Background:

• Pemphigus encompasses several autoimmune blistering diseases, including pemphigus vulgaris (PV), pemphigus foliaceus (PFG, PFL), and pemphigus erythematosus (PE).
• Treatment often involves corticosteroids, but some cases require additional immunosuppressants.

Purpose of the Study:

• To analyze the efficacy and outcomes of various treatment regimens for different types of pemphigus.
• To evaluate the role of cyclophosphamide and other immunosuppressants in corticosteroid-resistant pemphigus.

Main Methods:

• Retrospective analysis of 98 pemphigus cases treated between 1978-1987.
• Treatment strategies included corticosteroids alone, corticosteroids with dapsone or cyclophosphamide (CP), and other agents like gold sodiumthiomalate.
• Dosage and response were monitored for different pemphigus subtypes.

Main Results:

• Cyclophosphamide addition was effective for moderate to severe PV and PFG cases resistant to corticosteroids alone.
• Combination therapy with corticosteroids (60-120 mg/day) and initial CP cleared skin lesions within 2 months in 32 moderate and 14 severe cases.
• Eleven PFL and four PE cases showed good results with standard treatment. Intercellular antibody titers normalized in most cases.
• CP toxicity led to substitution with azathioprine or chlorambucil in three patients. Gold therapy provided partial or complete remission in refractory cases.

Conclusions:

• Combination therapy, particularly corticosteroids with cyclophosphamide, offers an effective treatment for pemphigus vulgaris and pemphigus foliaceus.
• Cyclophosphamide is a valuable addition for managing corticosteroid-resistant pemphigus.
• Localized pemphigus foliaceus and pemphigus erythematosus cases responded well to standard treatments.