Abstract

Localized scleroderma (morphea) is a disorder characterized by thickening and induration of the skin and subcutaneous tissue due to excessive collagen deposition. The etiology is unknown. Its subtypes are classified according to their clinical presentation and depth of tissue involvement: Plaque, generalized, bullous, linear, and deep morphea. Localized scleroderma can range from just a few spots on the skin to involvement of the entire skin surface. Severe contractures and deformities may occur, when linear or deep morphea lesions cross the joint lines. Most often, the onset of lesions is insidious and subtle, although in some cases they can develop rapidly. Despite its limitation to the skin and subdermal tissue, isolate cases with internal organ involvement have been described. There are large number of skin disorders that may resemble localized scleroderma. First of all, localized scleroderma should be differentiated from systemic scleros is. Several treatments are currently used with only limited success in localized scleroderma. Most patients with plaque morphea experience spontaneous remission over 3-5 years and require no specific treatment, whereas patients with generalized, linear, and deep morphea require more aggressive systemic therapy because of the higher morbidity. Treatment with topical steroids, intralesional steroid injection, topical calcipotrien and tocoretinate are preferred in small lesions whereas systemic cortico steroids, D-penicillamin, and methotrexate are used in generalized and progressive lesions. Life expectancy of children with localized scleroderma is normal. Localized scleroderma may last from a few months to many years. The most serious morbidities of the disease are limb asymmetry, flexion contractures, growth retardation, and disability which occur in linear and deep morphea patients.