44 - Morphea and Lichen Sclerosus

Abstract

Morphea and lichen sclerosus (LS) are inflammatory skin diseases that lead to cutaneous sclerosis and, in a number of patients, significant morbidity. The underlying pathogenesis is a combination of vascular damage and infiltration of T cells that release cytokines including IL-4 and TGF-β. In morphea, activated fibroblasts produce altered collagen and in LS, a loss of elastic fibers is accompanied by altered collagen fibers. Morphea can be subdivided into several subgroups: plaque-type/circumscribed (the most common form) which can become generalized, linear (including en coup de sabre), pansclerotic (children), guttate, deep, nodular/keloidal, and bullous. A variant of linear morphea is Parry–Romberg syndrome in which there is progressive hemifacial atrophy. The disability associated with morphea usually results from extension of the sclerosis into the subcutaneous tissues or over joints. In LS, the superficial sclerosis and atrophy are most debilitating when there is genital involvement. In some patients, the two disorders coexist. Superpotent topical corticosteroids represent the treatment of choice for anogenital LS. For LS in other sites, therapeutic options are limited. Standard treatments for the various forms of morphea include phototherapy (UVA1, PUVA), methotrexate, and systemic corticosteroids.