The carpal tunnel syndrome, although a well-defined clinical entity, has been appreciated only recently as a manifestation of systemic diseases. Its special architecture may reflect diseases in which acral edema is a factor. Carpal tunnel syndrome associated with such conditions as dermatomyositis and scleroderma may be relieved symptomatically by the systemic use of corticosteroids. Observations on five cases are reported. Carpal tunnel syndrome coexisted with dermatomyositis in four cases and with scleroderma in one.