Types of scleroderma that occur in childhood

Types of scleroderma that occur in childhood

• Scleroderma is characterized by abnormally increased collagen deposition in the skin and occasionally in the internal organs.

• Linear scleroderma is three times more common than diffuse scleroderma in childhood. It occurs more commonly in children than adults. Localized scleroderma may take the form of morphea, with a single patch or multiple patches. Linear scleroderma may occur on the face, forehead and scalp (en coup de sabre), or progressive hemifacial atrophy (Parry–Romberg syndrome). It can also occur on the limb (en bande). ANA (50%), RF (10%–25%), and hypergammaglobulinemia can be seen. Arthritis can be seen especially in RF+ patients, and seizures and uveitis can be seen as part of the Parry–Romberg syndrome. For severe or progressive disease, treatment includes high-dose IV methylprednisolone initially followed by prednisone with taper over 3 to 6 months. Methotrexate is coadministered with the steroids and continued for at least 2 years. There is a 30% recurrence rate after methotrexate is stopped.

• Diffuse scleroderma (SSc) may be limited in its involvement, with a prolonged interval before the appearance of visceral stigmata, or as part of the CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias). Myositis occurs in 25% of patients and may be the presenting manifestation.

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