Strabismus as the Initial Manifestation of En Coup De Sabre in Adult Patients

Patients with fronto-parietal linear scleroderma, also known as En Coup De Sabre (ECDS) may have multiple ocular manifestations. Few cases have been reported involving strabismus in ECDS, and a variety of mechanisms have been proposed.We present three cases of patients who developed progressive strabismus associated with ECDS. Each of these cases is believed to be caused by a different mechanism. Patients may also have systemic and neurologic involvement, necessitating neuroimaging and thorough evaluations by rheumatology and neurology.