ABANO TERME, ITALY — Juvenile localized scleroderma—traditionally considered a relatively benign condition with manifestations limited to skin and subcutaneous tissue—is not just simply a skin disease in close to one-quarter of affected children, Dr. Francesco Zulian said at a congress on skin, rheumatism, and autoimmunity.
Investigations of a worldwide database of 727 patients have shown that 22.4% of patients have extracutaneous disease manifestations, said Dr. Zulian of the University of Padua (Italy).
Among patients with extracutaneous manifestations, 66% had the linear subtype of juvenile scleroderma, while 25% had the plaque subtype. An additional 7% had generalized morphea, and 2% had deep morphea. “The mean age of onset was 7 years, but we have 17 patients with onset in the first year of life and 6 with congenital lesions,” he said. There was a family history of autoimmune disease in 12%, and a recent history of trauma in 14%. Juvenile localized scleroderma still was not well recognized, and the mean delay in diagnosis was 18 months.
The most common extracutaneous manifestation was arthritis, reported in 12.1%. Neurologic involvement was seen in 4.4%, vascular involvement in 2.4%, and ocular involvement in 2.1%.
The arthritis often is seen on the same side of the body as the cutaneous lesion, which raises the possibility that linear bands of sclerosis spreading across joints could cause inflammation by local mechanisms (Arthritis Rheum. 2005;52:2873–81).
But the observation that articular involvement sometimes occurred on the opposite side “makes us suspect that some systemic inflammation was going on,” Dr. Zulian said.
Neurologic manifestations were seen primarily among children with linear scleroderma of the face. Accordingly, any child with this presentation should be evaluated with an electroencephalogram and CT or MRI, particularly because abnormalities on MRI were found in seven of the children who had no neurologic symptoms, he said.
Treatment of the condition was addressed in a separate session by Dr. Davide Meneghesso, one of Dr. Zulian's colleagues at the University of Padua.
“There is no universally accepted treatment,” Dr. Meneghesso said. Topical, oral, and parenteral steroids have been used, as has D-penicillamine, vitamin D, and nonsteroidal anti-inflammatory drugs. Various supportive treatments such as anticonvulsants also have been used in patients with extracutaneous complications (Rheumatology 2005 Dec. 20 [Epub ahead of print];doi 10.1093/rheumatology/kei251).
But now, a prospective trial evaluating methotrexate, 15 mg/m
Mean age of the patients was 8.3 years, mean disease duration was 49 months, and the female to male ratio was 2.8:1.0. Nineteen children had linear scleroderma and eight had generalized morphea, he said.
Only two patients did not respond to the treatment. The remaining 11 patients remained stable during 12 months of follow-up, he said. Histologic evaluation in 16 of the patients found improvements in inflammation and fibrosis in nine and stable disease in seven. Side effects were seen in 55% of patients, but none were severe.
Among children with extracutaneous manifestations of scleroderma, the linear subtype (left) was found in two-thirds. Generalized morphea (right) is much more uncommon. PHOTOS COURTESY DR. FRANCESCO ZULIAN
