Objective: The purpose of this study is to report a case of acquired angioedema and Factor V Leiden mutation in a woman who had started taking an oral contraceptive (OC), and to review the literature. 

Results: A 44-year-old nonobese and nonsmoking woman was admitted to our hospital because of pain and progressive erythematous lesion on her left groin. The patient had had a 6-week history of combined OC use. There was no family history of angioedema. With a diagnosis of necrotizing fasciitis, surgical debridement of all necrotic tissue was considered with fasciocutaneous flap. Histopathological analysis showed diffusely fresh bleeding areas with diffused subcutaneous necrosis. The culture of the necrotic tissue was negative. Postoperatively, she restarted combined OC, and then the painful petechial and rapidly disseminated ecchymotic lesions were seen. Oral contraceptive was stopped immediately. A venous Doppler ultrasonography of the lower extremities showed subacute thrombosis in the left popliteal vein. Complement C3 level was normal, but amounts of C4, C1 esterase inhibitor level and activity were low. Factor V Leiden was positive for the heterozygous presence of Factor V gene. 

Conclusions: Women with angioedema and Factor V Leiden should be counseled against using hormonal contraceptives containing estrogen, and because they may interact in a synergistic manner in the pathogenesis of cutaneous lesions and/or venous thrombosis, appropriate individualized therapy should be defined. Further studies are needed. (c) 2006 Elsevier Inc. All rights reserved.