These observations warrant a search for PAN in young patients affected by FMF and showing signs of vasculitis. A patient infected by HBV developed a PAN demonstrated by clinical symptoms and pathology. Outcome was also unusual in the patient who did not respond to the association of plasma exchanges and antiviral agents and was only slightly improved by steroids and cyclophosphamide. HBV-related PAN is considered to be an immune complex disorder. This case reports underlines the heterogeneity of the PAN group of vasculitis and the probable role for various pathogenetic mechanisms.
Corticosteroids are an alternative therapy.
A case of cutaneous PAN followed for 6 years is reported. Dapsone, colchicin, corticosteroid and cyclophosphamide have failed to control cutaneous lesions. Some cases of necrotizing vasculitis have been treated with intravenous immunoglobulins. This is the first reported case of cutaneous PAN treated with intravenous immunoglobulins. Because of the lack of controlled studies, the indications of intravenous immunoglobulins in vasculitis are restricted to those resistant to corticosteroids and immunosuppressive therapy.