Episodic digital asphyxia caused by arterial insufficiency
Primary – Exaggerated vasospastic response in normal vessel – hypersensitivity of receptors in vessel wall
Bilateral, gangrene not a feature
Secondary – normal vasospastic response in abnormal vessel
Unilateral, gangrene can be a feature
Look for thoracic outlet obstruction, angiography
Young women in cool damp climates
Clinical
Sequential skin changes of pallor, cyanosis, rubor of fingers or toes after exposure to cold = Raynaud’s phenomenon.
Hands > feet, also affects nose, ears, nipples
White-blue-red color changes produced by excessive vasoconstriction, sluggish flow & reflex vasodilation
Sudden onset / progression suggest underlying arterial lesions → More aggressive
Associated CREST, SLE, Polymyositis, Thoracic outlet syndrome & occasionally others (cold agglutinins, CRF, neoplasia, vibration injury, drugs (OCP, ergotamine))
Acrocyanosis is a related chronic benign condition seen in young women, characterized by cold-induced cyanosis of the hands and feet ± with skin of calf/thigh/forearm displaying livedo reticularis
Investigations
Autoantibodies, Cryoglobulins, RhF, ESR, CRP, Electrophoresis
Management
Exclude primary causes
Systemic sclerosis, malignancy, SLE, drugs etc.
Lifestyle modification
Gloves, warm socks, hand and foot warmers
Stop smoking
Medication
Ca2+ Channel blockers - Oral Nifedipine
IVI Prostaglandin
Iloprost 6 hours per day for 3-5 days
Surgery
Sympathectomy for lower limb ONLY.
Amputation of non-viable digits/limbs.