Alessandra Bruns, Claudia Gennari Lacerda, Lúcia Stella S. de Assis Goulart, Virgínia Moça F. Trevisani
Division of Rheumatology
Universidade de Santo Amaro (UNISA)
Address: Rua Primeiro de Janeiro 450 ap.35, Vila Clementino, São Paulo – SP. CEP: 04044-060. Brazil.
Phone Number: 55 – 11 – 55876174
FaxNumber: 55 – 11 - 56682515
Key Words: localized scleroderma; childhood; treatment.
Localized scleroderma is a connective tissue disorder of unknown etiology which causes induration and discoloration of the skin as well as induration of subcutaneous tissue and muscles. It is a different disease from systemic scleroderma. It is subdividedinto morphea and linear scleroderma. There are still few reports on localized scleroderma in children. There are no studies that demonstrate a proven therapy for localized scleroderma. Even without specific
therapy, many different types of medications are used including corticosteroids, immunosuppressive and several other drugs with different possible mechanisms of actions. The disease’s ownvariable course and lack of standardization of outcome criteria make evaluation of the effectiveness of these treatments challenging.
INTRODUCTION AND CLINICAL CHARACTERISTICS
Localized scleroderma in children is a distinct entity from systemic sclerosis due to the absence of vasospasm, vascular lesions, and involvement of internal organs (1). It is classified into two subtypes:morphea and linear scleroderma. Linear scleroderma includes the typical linear lesions of localized scleroderma as well as “coup de sabre” lesions and facial hemiatrophy (Parry-Romberg Syndrome) (2). Morphea and linear scleroderma are clinically different, but histologically indistinguishable, and the lesions tend to improve in a period of 2 to 3 years (3). The linear form is frequently associated withgrowth deformities of the involved extremity. Rarely, either subtype may evolve to systemic sclerosis, although linear scleroderma can coexist with the systemic form or with another connective tissue disorder (4).
Morphea is characterized by the presence of one or more hardened and hypopigmented oval plaques on the face, trunk or extremities. In the initial phase, we can identify a reddishor violet inflammatory border. These plaques may be limited in number and size or may become extensive (generalized morphea).
In children, the localized form of scleroderma is more frequent than the systemic form but both of them are rare, constituting less than 3% of rheumatic diseases in childhood.. Approximately 1.5% of all scleroderma cases occur before 10 years of age (5,6). This illnessis present in all races and is more frequent in females (3:1). There is no significant familial incidence, and no consistent association with HLA antigens (1,5).
Different etiological factors have been implicated, including: 1.. trauma (6); 2. infectious agents, such as Borrelia burgdorferi (7); 3. environmental agents, such as toxic oil (8); 4. treatment with D-penicillamine (9); 5.bone marrow transplant (10); 6. oxygen-free radicals of endogenous and exogenous origin (11).
The main characteristics of linear scleroderma is the presence of one or more linear areas of involvement which affect the skin and can involve the underlying subcutaneous tissue, muscles and even the bones (Figure 1). The extremities, the face or the scalp might be affected. . Jointcontractures, functional limitations, digit atrophy, and cosmetic deformities are frequent complications (Figure 2). The denomination “scleroderma en coup de sabre” is used to designate the involvement of the face and/or the scalp and can be followed by profound tissue atrophy (Parry-Romberg Syndrome), most prominent in small children (Figure 3).
LABS AND OUTCOME MEASURES
Labs are of...