Calcinosis cutis represents a major clinical problem in a number of rheumatic diseases but is especially recognised in dermatomyositis and systemic sclerosis. In dermatomyositis it is especially a feature of childhood onset disease and can be very disabling. In systemic sclerosis calcinosis occurs in several distinct contexts and these may require different approaches to management. The commonest form is that which is seen in limited cutaneous systemic sclerosis, previously termed CREST syndrome. This is most often seen in the fingers and at sites of local pressure or trauma. However calcinosis cutis also occurs in diffuse cutaneous systemic sclerosis and may have a more widespread distribution. It can occur very diffusely in fascial layers of the limbs and trunk. Another form of calcinosis occurs around joints and can lead to very severe restriction of movement. Finally there can be specific foci of more severe calcinosis in the soft tissue at sites of co-existent trauma. The mechanism underlying calcinosis is poorly understood but may reflect aberrant mesenchymal tissue repair. Biochemical analysis conforms that calcinosis deposits are formed from calcium hydroxyapatite. Treatment is challenging. There has been anecdotal benefit from some medical approaches including minocylcine as well as warfarin, calcium channel blockers and bisphosphonates. In addition anti-TNF agents and methotrexate have been reported as helpful especially in cases associated with significant local inflammation. Surgical removal can be effective bit deposits often recur. The natural history, frequency and patterns of calcinosis cutis need to be better defined so that more systematic approaches to treatment can be developed. Calcinosis cutis remains one of the most difficult and potentially disabling manifestations of the connective tissue disease in which it occurs.
Disclosure of Interest C. Denton Consultant for: Actelion, GSK, Pfizer, Sanofi-Aventis, Serono