SYSTEMIC OR LIMITED IS HEMISCLERODERMA OF FACE IN A PERSON WITH UVEITIS? EXPERIENCE OF 10 CASES OF UVEITIS IN HEMISCLERODERMA OF FACE FROM ONE RHEUMATOLOGY CENTER.

Abstract

Linear scleroderma of head and face (LSH) in children is a severe disorder, that results in hemiatrophy of skin, subcutaneuse tissue, bones with functional disabilities, neurologic disorders and uveal involvement. The aim of the research was to establish uveal involvement in children with hemifacial scleroderma. Materials and methods: A retrospective analysis was done in a group of 110 children with hemifacial scleroderma. A comprehensive clinical, laboratory and instrumental examination was performed, including MRI of the brain, EEG, and an ophthalmologist's examination, which included visometry, biomicroscopy, and ophthalmoscopy. Results: 10 cases of uveal involvement were detected (9% of 110 pt). 9 patients had anterior segment inflammation (iridocyclitis), in 2 iridocyclitis was combined with retinal changes (in 1- peripheral focal chorioretinitis, in 1- iridocyclitis and central focal chorioretinitis). In one case, iridocyclitis was combined with optic neuropathy. In 3 children uveitis appeared at the disease debute, in the others 3-10 years later. Uveal inflammation in all cases was on the side of scleroderma skin involvement. In 3 children uveitis was bilateral. Seizures and concomittant foci in white matter of the brain were detected in 2 children with uveitis. 90% of the group had positive antinuclear factor. Persistent decrease in visual acuity developed in 3 patients. Соnclusion: Patients with LSH must undergo routine eye examination using basic ophthalmological techniques (visometry, biomicroscopy, ophthalmoscopy) every 6 months and highly necessary in case of relapse of scleroderma We assume that patients with UI in LSH must be defined as patients with JSS and treated intensively with systemicglucocorticoids, cytostatics and even biologics in case of resistance.