Update on the classification and treatment of localized scleroderma

Abstract

Morphea or localized scleroderma is a distinctive inflammatory disease that leads to sclerosis of the skin and subcutaneous tissues. It comprises a number of subtypes differentiated according to their clinical presentation and the structure of the skin and underlying tissues involved in the fibrotic process. However, classification is difficult because the boundaries between the different types of morphea are blurred and different entities frequently overlap. The main subtypes are plaque morphea, linear scleroderma, generalized morphea, and pansclerotic morphea. With certain exceptions, the disorder does not have serious systemic repercussions, but it can cause considerable morbidity. In the case of lesions affecting the head, neurological and ocular complications may occur. There is no really effective and universal treatment so it is important to make a correct assessment of the extent and severity of the disease before deciding on a treatment approach.

Keywords: Atrofodermia de Pasini y Pierini; Atrophoderma of Pasini and Pierini; Deep morphea; Eosinophilic fasciitis; Fascitis eosinofílica; Hemiatrofia facial progresiva; Lichen sclerosus et atrophicus; Liquen escleroso y atrófico; Morfea en coup de sabre; Morfea profunda; Morphea en coup de sabre; Progressive facial hemiatrophy.