[Morphea or localized scleroderma and extragenital lichen sclerosus]

Abstract

Localized scleroderma (LoS) is a very heterogeneous connective tissue disease characterized by progressive sclerosis of the skin with possible involvement of extracutaneous structures. Both children and adults can be affected but show different frequencies for the individual subtypes of the disease. The clinical heterogeneity has already caused several modifications of existing classification criteria. Patients suffering from LoS can essentially be subdivided into five different subsets, which are defined by the horizontal and vertical extent of the tissue involvement. The quality of life of these patients is significantly impaired depending on the extent of the cutaneous and subcutaneous involvement. A causal treatment does not yet exist; however, patients should be treated with the currently available medications for progressive subtypes during the early phase of inflammation to reduce or avoid severe, cosmetic and functional impairments. Lichen sclerosus (LS) usually affects the genital as well as extragenital skin and both children and adults can be affected. This article focuses on the extragenital LS, which occurs more frequently in adults. The cause of the disease as well as causal treatment strategies are still lacking. Currently, treatment is adapted to the therapeutic strategies for LoS.

Keywords: Biopsy; Classification; Connective tissue diseases; Fibrosis; Inflammation.