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Paraneoplastic acral vascular syndrome: epidemiologic features, clinical manifestations, and disease sequelae
Ewa Poszepczynska-Guigné1, Manuelle Viguier, Olivier Chosidow
1Department of Dermatology, Hôpital Saint-Louis, 1 avenue Claude-Vellefaux, 75475 Paris Cedex 10, France.
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.