Overview

• Exaggerated phenomenon to cold temperature or emotional stress

• Sharp demarcated colour changes of the skin of the digits

Primary

• No associated cause

• Age 15 to 30

• Females > males

Risk factors

• Family history

• Smoking

• Manual occupation

• Migraine

• CV disease

Associated conditions

• Fibromyalgia

• Migraines

Secondary

• Systemic sclerosis, SLE, MCTD, Sjogren’s, Dermatomyositis/Polymyositis

• Various drugs and toxins including amphetamines

• Haematology abnormalities include cryoglobulinemia

• Hypothyroidism

Incidence

• Surveys estimate 3 to 20% in females and 3 to 14% in males

Features

• Digits, ear, nose, face, knees, nipples

• Typical episode is cold fingers, demarcated colours, white then blue then red

• White or blue lasts 15 to 20 minutes

Additional lab tests if concerns about secondary Raynaud’s

• CBC

• Urinalysis

• ANA

• TSH

• ESR/CRP

• If sclerodactylyl

  • AB for Ssc

• If suspected inflammatory myopathy

  • CK / ENA

• If suspected SLE or cryoglobulinemia

  • C3/C4

• If suspected paraproteinemia

  • SPEP

Vascular Imaging

• If single digit or asymmetric attacks, absent pulses, asymmetric BP or evidence of chronic ischaemia

DDx

• Excessive cold sensitivity

• External compression of blood vessels

• Peripheral neuropathy

• CRPS

• Occlusive vascular disease

• Acrocyanosis

• Chilblains

Treatment

• Avoidance of cold exposure

• Maintain whole body warmth and digital warmth

• Consider reducing caffeine

• Consider reducing cold exposure during surgery

• Avoid vasoconstricting jobs

• Smoking cessation

• Calcium channel blockers = Amlodipine

• PDE5

• Topical nitrates