Prognosis and management of scleroderma and scleroderma-like disorders in children.
Scleroderma is a spectrum of disorders of which juvenile scleroderma forms a part. Fortunately, most children develop one of the localised forms and systemic disease is late. The prognosis of any individual type of scleroderma is dependent upon the subset of the disease, its extent, and internal organ involvement. In childhood, the problem is usually not one of survival, but of morbidity due to atrophy of skin, muscle and bone, with the potential for eventual growth defects and deformity. Drug treatment, when required, is usually directed towards suppressing inflammation and collagen deposition; vascular therapy does not play a major role, but physical therapy is crucial in certain forms of the disease.
Black
C M
CM
Department of Rheumatology, Royal Free Hospital, London, UK.
D002648:Child; D003937:Diagnosis, Differential; D004802:Eosinophilia / Q000175:diagnosis; D005208:Fasciitis / Q000175:diagnosis; D006801:Humans; D011379:Prognosis; D012594:Scleroderma, Localized / Q000175:diagnosis / Q000628:therapy*; D012595:Scleroderma, Systemic / Q000175:diagnosis / Q000628:therapy*