Background Joint hypermobility is a common but often poorly recognized connective tissue condition with joint laxity in the absence of any hereditary systemic disease. Acrocyanosis is symmetric, painless, blue discoloration in the distal parts of the body with aggravation by cold exposure, and frequent association with local hyperhidrosis of hands and feet. Primary acrocyanosis is mostly a disease of young adults, only few cases persist into middle age.
Methods A group of 200 consecutive rheumatology patients (<45 yrs. old) investigated for joint laxity and acrocyanosis. For each patient a Beighton score measured and those with a score of >4 regarded as benign joint hypermobility due to Brighton criteria. Acrocyanosis was diagnosed clinically by proper history and physical examination. Other relevant clinical findings were also recorded.
Results A total of 24 patients (F: M, 12:12) were diagnosed as hypermobility. Nearly all patients had a mild-moderate hypermobility. The mean Beighton score was 4.2±0.4. Of those patients, 11 had also acrocyanosis (%45.8). In the remaining group (176), only two cases of acrocyanosis detected (%0.01). In the group with acrocyanosis, hand and foot size proportionate to stature is larger than those without.
Conclusions It has been known that, patients with hypermobility may have some vascular problems, including Raynaud's phenomenon and varicose veins. Our observation points out that, besides joint hypermobility, vessel wall laxity might cause to passive engorgement of vascular structures, namely acrocyanosis.
Disclosure of Interest None declared