006Efficacy of photochemotherapy and UVA-1 therapy in patients with morphea or lichen sclerosus

Abstract

Morphea and lichen sclerosus are inflammatory skin diseases of unknown aetiology. Morphea can be subdivided into plaque morphea, linear morphea and disabling or generalized morphea. In most patients morphea leads to superficial or deep sclerosis of the skin. The characteristic features of lichen sclerosus which often affects the genital area are edema of upper dermis, inflammatory infiltration and hyalinisation to the dermis at advanced stages. Patients with morphea or lichen sclerosus suffer especially from scar formation and morphea may lead to severe disfigurement, contractures and reduction of quality of life. Skin sclerosis seems to be the result of vascular damage, T cell activation and altered connective tissue production. Various therapies have been reported for lichen sclerosus and morphea. Whereas the topical use of ultrapotent corticosteroids is well established for genital lichen sclerosus, immunosuppressive agents are normally not successful in resolving extragenital skin sclerosis. In a retrospective study we confirmed the efficacy of phototherapy in more than 50 patients with morphea. Fourty treatments with 30 J/cm2 UVA-1 or PUVA-bath photochemotherapy resulted in a significant improvement, reduced skin thickness, as determined by high frequency ultrasound and reconstitution of functional mobility of the skin and even the underlying fasciae. In lichen sclerosus phototherapy was successful only in some patients. Thus for lichen sclerosus the use of topical corticosteroids is the first choice therapy, while phototherapy using either PUVA-bath or medium dose UVA-1 are the most effective treatments for morphea.