[Neumann type pemphigus vegetans with fatal outcome]

Area of Science:

• Dermatology
• Autoimmune Diseases
• Clinical Case Reports

Background:

• Pemphigus vegetans, Neumann type, is a rare autoimmune blistering disease.
• Management often involves systemic corticosteroids, which can lead to significant side effects with long-term use.

Observation:

• A 45-year-old patient with Neumann type pemphigus vegetans experienced disease progression despite various immunosuppressive therapies.
• Initial corticosteroid therapy halted disease progression but necessitated high doses.
• Long-term corticosteroid use resulted in severe complications including sepsis, thrombosis, and osteoporosis.

Findings:

• Attempts to reduce corticosteroid dosage with azathioprine, cyclophosphamide, or gold thiomalate were unsuccessful without disease exacerbation.
• Cyclophosphamide bolus therapy and gold thiomalate were used after discontinuing corticosteroids due to adverse effects.
• Subsequent treatment with methotrexate and dapsone for disease exacerbation failed to control skin lesions.

Implications:

• This case underscores the challenges in managing refractory pemphigus vegetans.
• It highlights the critical risks associated with long-term high-dose corticosteroid therapy in autoimmune conditions.
• Alternative or combination therapies may be necessary to mitigate corticosteroid toxicity while controlling disease activity.