Localized Scleroderma ILARIA GHERSETICH, MD l PATRIZIA TEOFOLI, MD MAURIZIO BENCI, MD l SILVIA INNOCENTI, MD TORELLO LOTTI, MD S cleroderma is a chronic disease of unknown eti- ology in which sclerosis of skin develops, with or without involvement of internal organs. It is pos- sible to differentiate between two major groups because by the extent, course, and prognosis of disease: localized scleroderma and progressive systemic sclero- derma. In this paper we describe the most relevant fea- tures of localized scleroderma. Localized scleroderma (LSJ,or morpheu, is a chronic be- nign condition characterized by indurated areas of skin that are faint purple at first but and eventually lose their color centrally, leaving ivory colored areas surrounded by a typical lilac ring. l The condition may be subdivided clinically into the following major types:* (a) circum- scribed plaques or bands; (b) linear morphea; and (c) frontoparietal lesions (en coup de sabre), with or without hemiatrophy of the face. Etiopathogenesis The etiology is unknown. Trauma is blamed as the pre- cipitating factor in individual cases.3 Morphea has oc- curred after BCG vaccination4 and at the site of rudiofher- upy for previous breast carcinoma.5 Hormonal factors may influence the disease: morphea may appear or grow worse during pregnancy. It has been proposed that Bowe- liu burgdorferi infection, which is the cause of a distinctive form of scleroderma (i.e., acrodermatitis chronica atroph- icans, ACA), causes some cases of true linear scleroderma and morphea.6 There are reports of raised levels of anti- bodies against this organism in patients with morphea compared with controls, and of the presence of viable From the Department of Dermatology I, University of FZorence, Florence Italy. S. Innocenti and T. Lotti are with the Department of Dermatology, Llni- versity of Siene, Italy. Address correspondence to Dr. Ilaria Ghersetich, Research Fellow of Dermatology, University of Florence, via P. Cupponi 21, 50132 Norence, Italy. 0 1994 by Elsevier Science Inc. l 0738-081x/94/$7.00 organisms in biopsies from morphea lesions.6 However, these results are unconfirmed, and several studies have not found abnormal levels of borrelial antibodies in pa- tients with scleroderma. T8 Recent studies have shown that morphea and ACA can be distinguished from each other in most cases on the basis of clinical, histologic, and immunohistochemical criteria.9 In fact, ACA involves acral body sites with lower temperatures, is seen mostly in elderly persons, and presents as a livid discoloration that is not sharply demarcated. Morphea affects any age and body site, and exhibits extension at the periphery of the lesion. Histologically, ACA shows atrophy of collagen and elastic fibers as well as hypertrophic basophilic elas- tic tissue, whereas in morphea, sclerosis and polarizing elastic tissue are prominent. Immunohistochemical test- ing with different lymphocyte markers showed differ- ences only in the expression of HLA-DR antigens.9 Re- cently the occurrence of morphea has been related to the implantation of silicon prothesis for breast augmenta- tion.‘O A familial incidence of morphea has been no- ticed,ll but significant HLA associations have not been demonstrated so far.12 Viral, toxic, neurogenic, or vascular factors might contribute, but this has not been proven (see Table 1).13 Cutaneous and systemic immunologic abnor- malities have been described. The pathogenesis of LS is currently considered the same as systemic sclerosis, and the two disorders can even occur together. Pathology The epidermis may be normal or flattened. The dermis at the very beginning is edematous, with swelling and de- generation of collagen fibrils, which become eosinophilic. There might be a moderate to dense perivascular lym- phocytic infiltrate (Figures 1 and 2). Later, the dermis becomes thickened, with dense collagen and fibro- blasts.” The elastic fibers are reduced in number. The dermal appendages are progressively lost. Electronmicro- scopic findings show regional neoformation of collagen. 237