Report
Pediatric morphea (localized scleroderma): Review of 136 patients

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Background

Morphea is an autoimmune inflammatory sclerosing disorder that may cause permanent functional disability and disfigurement.

Objectives

We sought to determine the clinical features of morphea in a large pediatric cohort.

Methods

We conducted a retrospective chart review of 136 pediatric patients with morphea from one center, 1989 to 2006.

Results

Most children showed linear morphea, with a disproportionately high number of Caucasian and female patients. Two patients with rapidly progressing generalized or extensive linear morphea and arthralgias developed restrictive pulmonary disease. Initial oral corticosteroid treatment and long-term methotrexate administration stabilized and/or led to disease improvement in most patients with aggressive disease.

Limitations

Retrospective analysis, relatively small sample size, and risk of a selected referral population to the single site are limitations.

Conclusions

These data suggest an increased prevalence of morphea in Caucasian girls, and support methotrexate as treatment for problematic forms. Visceral manifestations rarely occur; the presence of progressive problematic cutaneous disease and arthralgias should trigger closer patient monitoring.

Section snippets

Study design

An institutional review board–approved retrospective chart review was performed of children with morphea seen our division of dermatology from 1989 to 2006. These children were identified through a computerized search based on International Classification of Diseases, Ninth Revision diagnosis code for linear scleroderma (701.00). Patients with evidence of systemic sclerosis at baseline were excluded from the study. Information was obtained from each patient's chart about sex, ethnicity

Demographic data

The charts of 136 children with a diagnosis of morphea were available for examination. The female:male ratio was 2.42:1 (Table I). Affected individuals were predominantly Caucasian (82%); 12% were Hispanic, 3% were African American; and 3% were Asian.

Clinical presentation

The most common form of morphea was linear (51.4%) (Table I) with almost twice as many patients showing linear lesions on the extremities than the ECDS or PHA forms of linear morphea. Plaque type morphea was seen in only 37% of the children. The

Discussion

Morphea is an uncommon condition that comprises approximately 0.2% of patient referrals to pediatric dermatology clinics.14, 15, 16 The increased occurrence in girls has been well described, and our series concurs with this observation. We were intrigued, however, by the predominance of Caucasian children in our morphea population. The overall patient population in our children's hospital dermatology department is approximately 60% Caucasian, 15% Hispanic, 12.5% African American, and 12.5%

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    Funding sources: None.

    Conflicts of interest: None declared.

    Reprints not available from the authors.

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