Key points

• Understanding of the nomenclature used for the classification of vasospastic disorders into primary and secondary Raynaud's phenomena is essential

• The management of these disorders requires a multidisciplinary team approach involving physicians, rheumatologists and vascular specialists

• Treatment of Raynaud's includes general supportive measures, pharmacotherapy and correction of underlying disorders

• Vasculitis is associated with a range of medical conditions and can present as digital ischaemia

• A diagnosis of vasculitis is suggested by constitutional symptoms and confirmed by raised inflammatory markers, autoantibodies and biopsy of the skin lesions

• Immunosuppressive therapy is the mainstay of treatment in vasculitic disorders

Introduction

Raynaud's phenomenon refers to a clinical state characterised by episodic vasospasm, usually involving the distal small arteries of the upper limb although sometimes toes and feet are also affected. In addition, there are other vascular disorders characterized by inflammatory changes in the arterial wall, known as ‘vasculitidies’, which can present with digital ischaemia.

Vasospasm (Raynaud's phenomenon)

Maurice Raynaud first described this clinical picture in 1862. The classical presentation of Raynaud's phenomenon is characterised by a sequence of colour changes in the following order:

• pallor, reflecting initial vasospasm;

• cyanosis as a result of deoxygenation of stagnant blood during maximum vasospasm;

• rubor, representing inflow of oxygenated blood and reactive hyperaemia as the vasospasm subsides.

Episodes usually last for 30–60 min. However, some patients present with only cold hands and do not exhibit the classical triple colour response although they demonstrate a similar blood flow pattern to classical vasospasm.