Coagulation factor V gene mutation associated with activated protein C resistance leading to recurrent thrombosis, leg ulcers, and lymphedema: successful treatment with intermittent compression

Activated protein C resistance is the most frequent cause of venous thrombosis. We describe a patient with extensive ulcerations and severe lymphedema of the legs after recurrent thrombosis. Laboratory tests revealed a pathologic activated protein C resistance and a reduced functional protein S. The...

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Bibliographic Details
Published in:Journal of the American Academy of Dermatology 1996-08, Vol.35 (2), p.306-309
Main Authors: Peus, Dominik, Schmiedeberg, Sherko v, Pier, Andreas, Scharf, Rüdiger E, Wehmeier, Artur, Ruzicka, Thomas, Krutmann, Jean
Format: Article
Language:English
Subjects:
Anti-Bacterial Agents - therapeutic use
Bandages
Biological and medical sciences
Blood Coagulation Disorders - genetics
Debridement
Factor V - genetics
Hematologic and hematopoietic diseases
Heterozygote
Humans
Leg Ulcer - etiology
Leg Ulcer - therapy
Lymphedema - etiology
Lymphedema - therapy
Male
Medical sciences
Middle Aged
Mutation - genetics
Platelet diseases and coagulopathies
Protein C - metabolism
Protein S - metabolism
Recurrence
Thrombophlebitis - etiology
Thrombophlebitis - genetics
Thrombophlebitis - therapy
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Summary:Activated protein C resistance is the most frequent cause of venous thrombosis. We describe a patient with extensive ulcerations and severe lymphedema of the legs after recurrent thrombosis. Laboratory tests revealed a pathologic activated protein C resistance and a reduced functional protein S. The underlying genetic defect was identified as a heterozygous coagulation factor V mutation. A combined therapeutic approach of intermittent compression, repeated debridements and systemic antibiotics resulted in marked improvement of both lymphedema and leg ulcers.
ISSN:0190-9622
1097-6787
DOI:10.1016/S0190-9622(96)90655-6