The scleroderma neck sign.

The scleroderma neck sign, as described by Barnett, is a visible and palpable tight band over platysma in the hyperextended neck. A recent survey of 76 patients with scleroderma revealed that more than 90% had the scleroderma neck sign. Our study was performed using 15 patients with scleroderma and 30 controls including 3 with primary Raynaud's disease to examine the specificity of the scleroderma neck sign, and to look for a correlation between the presence of the scleroderma neck sign and histological changes of scleroderma in the skin overlying platysma. The scleroderma neck sign was present in 12 of the 15 patients with scleroderma but in none of the 30 controls. It was found both in patients with diffuse (5 out of 5) and limited (7 out of 10) scleroderma. In 10 of the 12 cases where the scleroderma neck sign was positive, there were characteristic histological changes of scleroderma on biopsy of the skin overlying platysma, in 1 there were nondiagnostic abnormalities, and in 1 the biopsy was unsatisfactory. The 3 patients with scleroderma in whom the scleroderma neck sign was absent had either nondiagnostic changes (1) or normal biopsies (2). The 3 patients with Raynaud's disease had normal skin biopsies. The scleroderma neck sign appears to be produced by scleroderma changes in the skin of the neck. In limited or early scleroderma where these changes are otherwise clinically inapparent, the scleroderma neck sign may be diagnostically useful.