Paraneoplastic acral vascular syndrome: epidemiologic features, clinical manifestations, and disease sequelae.
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.


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.


CONCLUSIONS
A neoplastic origin of acral vascular syndrome should be considered in elderly patients, especially men, in the absence of usual causative conditions.
Poszepczynska-Guigné
Ewa
E
Department of Dermatology, Hôpital Saint-Louis, 1 avenue Claude-Vellefaux, 75475 Paris Cedex 10, France.
Viguier
Manuelle
M
Chosidow
Olivier
O
Orcel
Brigitte
B
Emmerich
Joseph
J
Dubertret
Louis
L
D000368:Aged; D004938:Esophageal Neoplasms / Q000503:physiopathology; D005260:Female; D005385:Fingers / Q000098:blood supply; D005734:Gangrene / Q000175:diagnosis / Q000453:epidemiology*; D006801:Humans; D015994:Incidence; D008223:Lymphoma / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D010257:Paraneoplastic Syndromes / Q000175:diagnosis* / Q000453:epidemiology*; D011379:Prognosis; D012307:Risk Factors; D015996:Survival Rate; D013577:Syndrome; D014652:Vascular Diseases / Q000175:diagnosis / Q000453:epidemiology*