Poszepczynska-Guigné E, Viguier M, Chosidow O, Orcel B, Emmerich J, Dubertret L Paraneoplastic acral vascular syndrome: epidemiologic features, clinical manifestations, and disease sequelae. [Case Reports, Journal Article, Review] J Am Acad Dermatol 2002 Jul; 47(1):47-52.
BACKGROUND: Acral vascular syndromes associated with malignancy have rarely been reported. OBJECTIVE: Our purpose was to assess the clinical and evolving features of paraneoplastic acral vascular syndromes. Patients and Methods: Two cases of paraneoplastic gangrene are described and analyzed together with previously reported cases identified by a MEDLINE search. RESULTS: Among the 68 patients identified, 40 had gangrene, 16 had acrocyanosis, and 12 had Raynaud's phenomenon. The male to female ratio was 0.89; median age was 59 years. Fingers were affected in 94%. Adenocarcinomas were the predominant associated malignancies (41%), and metastases were observed in 41%. The acral vascular syndromes in 48% of the patients definitively regressed after tumor treatment. Forty-four percent of the patients died within 2 years. A favorable cutaneous outcome was obtained with prostacyclin infusions in 6 patients. CONCLUSION: A neoplastic origin of acral vascular syndrome should be considered in elderly patients, especially men, in the absence of usual causative conditions.
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