As in SLE, changes in the skin are the most obvious manifestations of scleroderma in the head and neck. In systemic sclerosis, the skin changes can begin with a sometimes pruritic, edematous phase, as inflammatory cells and fibroblasts are activated and cytokines are released. The skin in the affected areas tightens, loses flexibility, and thickens. In systemic sclerosis, characteristic facies may be noted, with a pursed-lip appearance and reduction in the oral aperture (Fig. 4). After several years, thinning and atrophy occur. Pigmentation changes may occur as well, giving a "salt and pepper" appearance. The dramatic en coup de sabre lesion (Fig. 5) is rare and is a manifestation of localized scleroderma. A nondermatomal fibrotic band infiltrates skin, subcutaneous fat, fascia, and muscle in the midline of the face, giving the appearance of a sword wound. Telangiectasias may be prominent on the head and neck, including involvement of the palate, oral mucosa, and tongue (Fig. 6). Raynaud's phenomenon is a virtually universal feature of scleroderma and may transiently affect the tip of the nose and the pinnae. A variety of changes may occur in the nasal mucosa (16); nasal perforation can occur (17); and calcinosis has been reported rarely on the nose (18). Chondrodermatitis nodularis helicis has rarely been seen in
scleroderma (19). Sensorineural hearing loss has been reported to occur, but has not been found to correlate with other disease manifestations (20,21). Virtually the entire gastrointestinal tract can be affected in scleroderma. Swallowing dysfunction due to oropharyngeal involvement can occur (22) and increase the risk of aspiration. Esophageal dysmotility and nonobstructive dysphagia are seen in the majority of patients, making symptoms of gastroesophageal reflux very common.
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