Morphoea (Localized Scleroderma)

Abstract

Morphoea encompasses a group of related conditions characterized by varying degrees of sclerosis, fibrosis and atrophy in the skin and subcutaneous tissues, sometimes extending deeply into the muscle, bone, eye and brain. Extracutaneous manifestations occur in up to 25% of cases but in contrast to systemic sclerosis (SSc), no internal organ fibrosis or vascular changes occur. Antinuclear antibody positivity is common but the specific autoantibodies seen in SSc are rarely present. There is no increased mortality, but substantial morbidity may occur as a result of joint contractures, facial and limb asymmetry, extracutaneous manifestations and the psychological impact of the condition. Although previously considered self‐limiting, there is now emerging evidence that a protracted, relapsing–remitting course may be common. The key to successful treatment is the involvement of a multidisciplinary team and the initiation of therapy during the active inflammatory stage, before significant damage has occurred. Topical and phototherapies are appropriate in superficial forms of morphoea, whilst a majority of linear, generalized and deep forms require systemic therapy.