Corticosteroid treatment and growth of angiolipomas in patient with two rare diseases: Pfeifer-Weber-Christian disease and benign multiple subcutaneous angiolipomas

Abstract

Introduction. Pfeifer?Weber?Christian disease (PWCD) is a rare inflammatory disorder of the subcutaneous fatty tissue. Angiolipoma, is a benign adipocytic soft tissue tumor composed of mature adipose tissue and small vascular proliferations. Treatment with corticosteroids could lead to proliferation of fat tissue but the stimulation of angiolipoma growth during corticosteroid therapy is extremely rare. Case outline. We describe a case of a 46-year-old female patient with histopathological confirmation two rare diseases: PWCD and benign multiple subcutaneous non-infiltrative angiolipomas. Angiolipomas were treated conservatively. Treatment for PWCD was prednisone 20 mg/day. Due to poor control of PWCD and rapid angiolipomas growth on forearms, corticosteroids were discontinued after two months of use. Administration of oral cyclosporine A led to a rapid remission of the PWCD, and with no new growth of angiolipomas. Conclusion. The successful therapy with the Cyclosporine A supports the hypothesis that PWCD is a T cell mediated autoinflammatory condition. Rapid growth of angiolipoma during corticosteroid therapy is an extremely rare condition.