Juvenile Scleroderma in Taiwanese Children-Experience of one Institution in Taipei

台北一醫學中心幼年型硬皮症病患之臨床經驗

10.6313/FJR.2006.20(1-2).06

高羽璇(Yu-Hsuan Kao)；徐世達(Shyh-Dar Shyur)；朱斯鴻(Szu-Hung Chu)；黃立心(Li-Hsin Huang)；王聰淇(Tsung-Chi Wang)

幼年型硬皮症 ； 硬斑病 ； 線狀硬皮病 ； 軍刀型硬皮症 ； Juvenile scleroderma ； linea ； morphea ； en coup de sabre

Formosan Journal of Rheumatology

20卷1&2期（2006 / 12 / 01）

39 - 48

英文

Scleroderma is rare in children who are more likely to have localized cutaneous scleroderma. In this study, we retrospectively reviewed the cases of eleven children with systemic and localized scleroderma. Materials and Methods. We reviewed the records of eleven children with systemic or localized scleroderma seen from March 1993 to June 2006 in the Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan. They were diagnosed according to American College of Rheumatology criteria and clinical manifestations of hard skin involvement. Data extracted from the records included gender, age at onset, age at diagnosis, clinical manifestations, laboratory data, family history, trauma history, treatment, and outcome. Results. The mean age at diagnosis was 9.2 years (range, 3 to 12 years). The mean age at onset was 6.5 years (range, birth to 11.7 years). Two children (1 girl and 1 boy) had systemic scleroderma (both with pulmonary involvement and 1 with renal involvement) and the other 6 girls and 3 boys had localized scleroderma, either morphea or a linear pattern. Antinuclear antibodies were positive in 10 at titers of 40x to 640x; 6 had a speckled pattern, 2 had a homogenous pattern, and 2 had a speckled-to-homogenous pattern. Tests for anti-Scl-70 antibodies were all negative. Serum levels of rheumatoid factor ranged from ＜20 to 60.2 IU/ml with only 3 children having positive levels (60.2 IU/ml in 1 girl with systemic scleroderma; 45.6 and 58.2 IU/ml in 1 girl and 1 boy with localized scleroderma, respectively). All patients had skin tightening but none had subcutaneous calcification. Raynaud's phenomenon with digital pitting was present only in the 2 patients with systemic scleroderma. Skin biopsy specimens from 2 boys and 1 girl showed hypertrophic collagen bundles with atrophic skin appendages and lymphocytic infiltration. All patients were treated with D-penicillamine with or without steroids and methotrexate. Only 1 boy with localized scleroderma recovered completely while the others with localized disease had a benign course with some skin softening. None of those 9 children with localized scleroderma progressed to systemic disease. Conclusion. Childhood scleroderma is more likely to be a localized cutaneous disease which, while it may not resolve, does not appear to progress to systemic disease.

Scleroderma is rare in children who are more likely to have localized cutaneous scleroderma. In this study, we retrospectively reviewed the cases of eleven children with systemic and localized scleroderma. Materials and Methods. We reviewed the records of eleven children with systemic or localized scleroderma seen from March 1993 to June 2006 in the Department of Pediatrics, Mackay Memorial Hospital, Taipei, Taiwan. They were diagnosed according to American College of Rheumatology criteria and clinical manifestations of hard skin involvement. Data extracted from the records included gender, age at onset, age at diagnosis, clinical manifestations, laboratory data, family history, trauma history, treatment, and outcome. Results. The mean age at diagnosis was 9.2 years (range, 3 to 12 years). The mean age at onset was 6.5 years (range, birth to 11.7 years). Two children (1 girl and 1 boy) had systemic scleroderma (both with pulmonary involvement and 1 with renal involvement) and the other 6 girls and 3 boys had localized scleroderma, either morphea or a linear pattern. Antinuclear antibodies were positive in 10 at titers of 40x to 640x; 6 had a speckled pattern, 2 had a homogenous pattern, and 2 had a speckled-to-homogenous pattern. Tests for anti-Scl-70 antibodies were all negative. Serum levels of rheumatoid factor ranged from ＜20 to 60.2 IU/ml with only 3 children having positive levels (60.2 IU/ml in 1 girl with systemic scleroderma; 45.6 and 58.2 IU/ml in 1 girl and 1 boy with localized scleroderma, respectively). All patients had skin tightening but none had subcutaneous calcification. Raynaud's phenomenon with digital pitting was present only in the 2 patients with systemic scleroderma. Skin biopsy specimens from 2 boys and 1 girl showed hypertrophic collagen bundles with atrophic skin appendages and lymphocytic infiltration. All patients were treated with D-penicillamine with or without steroids and methotrexate. Only 1 boy with localized scleroderma recovered completely while the others with localized disease had a benign course with some skin softening. None of those 9 children with localized scleroderma progressed to systemic disease. Conclusion. Childhood scleroderma is more likely to be a localized cutaneous disease which, while it may not resolve, does not appear to progress to systemic disease.