Advanced keratoconus in a child with juvenile scleroderma

An 18-year-old male presented with complaints of gradually decreasing vision in both eyes for 6 years. He was a diagnosed with juvenile scleroderma at the age of 11 years by a pediatric rheumatologist. Clinical slit lamp examination showed features of ectasia, thinning, Vogt's striae, and apical scarring in both eyes. Bulbar and tarsal conjunctiva was quiet and normal. Corneal tomography revealed mean keratometry 65.8 and 65.4 diopters, thinnest pachymetry of 351 and 224 microns in the right and left eye, respectively. There was no history of itching and eye rubbing in the past. Patients of juvenile scleroderma may have associated keratoconus. The management of advanced keratoconus presents challenges related to handling and insertion of contact lenses in this condition. Keratoplasty is an option in those patients when contact lenses are not acceptable.

Juvenile scleroderma: experience in one institution.

Background: Scleroderma is a chronic connective tissue disease characterized by hardened or scaly skin and widespread abnormalities of the viscera, which is rare in the pediatric age group. Objective: In this study, we retrospectively reviewed 23 pediatric patients suffering systemic (SSc) and localized (LS) scleroderma. Methods: Twenty-three patients were enrolled and were diagnosed with SSc or LS from March 1993 to September 2009 in the Department of Pediatrics at Mackay Memorial Hospital in Taipei, Taiwan. These diagnoses were based on the criteria of the American College of Rheumatology and the clinicalmanifestations of hard skin. Data recorded included sex, age-at-onset, age-at-diagnosis, laboratory data, family history, trauma history, treatment, and outcomes. Results: Three patients suffered SSc and 20 patients had LS, including 16 girls and 7 boys. Mean age-at-onset was 6.55±3.28 years old. Antinuclear antibodies were positive in 15 patients. Tests for anti-Scl-70 antibodies were positive in 1 patient with SSc. One boy had en coup de sabre combined with a posterior fossa tumor. Twenty-two patients were treated with D-penicillamine. Oral prednisolone and methotrexate were added, if indicated. One girl with LS developed proteinuria after Dpenicillamine treatment. All patients with localized disease ultimately documented a softening of their skin lesions. Conclusions: While scleroderma is rare in children, the prognosis of SSc is poor but better than for adults. The prognosis for LS is usually benign, however, the skin may become progressively indurated and it may not only be a skin disease. No progression from LS to SSc was observed in our study.