A Clinical Study of Localized Scleroderma in Childhood

Eun, Chul; Lee, Chang Woo

Abstract

BACKGROUND: Localized scleroderma, a rare autoimmune disease, can be classified into three subtypes.linear scleroderma, morphea and generalized morphea. Localized scleroderma is usually limited to the skin and subcutaneous tissue but may result in permanent functional disability. Because of its rarity, there are few large scale clinical studies of pediatric patients with localized scleroderma in Korea.
OBJECTIVE
In this study we have examined 11 Korean pediatric patients with localized scleroderma registered at Hanyang university hospital. A review of the clinical presentation, laboratory data, therapy and complcations was carried out in detail.
METHODS
The medical records of 11 pediatric patients with localized scleroderma seen at the department at Hanyang University Hospital, between 1990 and 1996, were reviewed. The diagnoses were classified into one of the above three localized scleroderma subtypes based on the results of the physical examination, and all patients were examined periodically. The clinical features of the disease, laboratory results, treatment modalities, and complications were summerized from the review of the records.
RESULTS
Among the 11 patients, 8 were female and 3 were male; 7 had morphea and 4 had linear scleroderma. The average age at onset was 8.6 years. Antinuclear antibodies above the titer of 1:40 were present in 36%. One of 3 patients with linear scleroderma which involved the upper extremities had moderate degrees of joint contracture. Ten of 11 patients were treated with intralesional injection of corticosteroid and oral medications of hydroxychloroquine, prednisolone and/or nosteraidal anti-inflammatory drugs.
CONCLUSION
Localized scleroderma has no recognized internal organ involvements and is not thought to progress to systemic sclerosis. Since localized scleroderma may cause complications from local atrophy to joint contractures, patients will need to take adequate treatment. and follow-up examinations.