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

J Am Acad Dermatol. 2002 Jul;47(1):47-52. doi: 10.1067/mjd.2002.120474.


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.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged
  • Esophageal Neoplasms / physiopathology
  • Female
  • Fingers / blood supply
  • Gangrene / diagnosis
  • Gangrene / epidemiology*
  • Humans
  • Incidence
  • Lymphoma / physiopathology
  • Male
  • Middle Aged
  • Paraneoplastic Syndromes / diagnosis*
  • Paraneoplastic Syndromes / epidemiology*
  • Prognosis
  • Risk Factors
  • Survival Rate
  • Syndrome
  • Vascular Diseases / diagnosis
  • Vascular Diseases / epidemiology*