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Site of morphea lesions predicts risk of extracutaneous manifestations



– Morphea lesions on the extensor extremities, face, and superior head are associated with higher rates of extracutaneous involvement, results from a multicenter retrospective study showed.

Dr. Yvonne E. Chiu Doug Brunk/MDedge News

Dr. Yvonne E. Chiu

“We know that risk is highest with linear morphea,” lead study author Yvonne E. Chiu, MD, said at the annual meeting of the Society for Pediatric Dermatology. “Specifically, linear morphea on the head and neck is associated with neurologic issues, and linear morphea on a limb is associated with musculoskeletal issues. However, risk stratification within each of those sites has never really been studied before.”

Dr. Chiu, who is a pediatric dermatologist at the Medical College of Wisconsin and Children’s Hospital of Wisconsin in Milwaukee, and her associates carried out a 14-site retrospective study in an effort to characterize morphea lesional distribution and to determine which sites had the highest risk for extracutaneous manifestations. They limited the analysis to patients with pediatric-onset morphea before the age of 18 and adequate lesional photographs in their clinical record. Patients with extragenital lichen sclerosis and atrophoderma were included in the analysis, but those with pansclerotic morphea and eosinophilic fasciitis were excluded. The researchers used custom web-based software to map the morphea lesions, and linked those data to a REDCap database where demographic and clinical information was stored. From this, the researchers tracked neurologic symptoms such as seizures, migraine headaches, other headaches, or any other neurologic signs or symptoms; neurologic testing results from those who underwent MRI, CT, and EEG; musculoskeletal symptoms such as arthritis, arthralgias, joint contracture, leg length discrepancy, and other musculoskeletal issues, as well as ophthalmologic manifestations including uveitis and other ophthalmologic symptoms. Logistic regression was used to analyze association of body sites with extracutaneous involvement.

Dr. Chiu, who also directs the dermatology residency program at the Medical College of Wisconsin, reported findings from 826 patients with 2,467 skin lesions of morphea, or an average of about 1.92 lesions per patient. Consistent with prior reports, most patients were female (73%), and the most prevalent subtype was linear morphea (56%), followed by plaque (29%), generalized (8%), and mixed (7%).

The trunk was the single most commonly affected body site, seen in 36% of cases. “However, if you lumped all body sites together, the extremities were the most commonly affected site (44%), while 16% of lesions involved the head and 4% involved the neck,” Dr. Chiu said. Patients with linear morphea had the highest rate of extracutaneous involvement. Specifically, 34% had musculoskeletal involvement, 24% had neurologic involvement, and 10% had ophthalmologic involvement. There were small rates of extracutaneous manifestations in the other types of morphea as well.

The most common musculoskeletal complications among patients with linear morphea were arthralgias (20%) and joint contractures (17%), followed by other musculoskeletal complications (15%), leg length discrepancy (5%), and arthritis (2%). Contrary to previously published reports, nonmigraine headaches were more common than seizures among patients with linear morphea (17% vs. 4%, respectively), while 4% of subjects had migraine headaches. Of the 134 subjects who underwent neuroimaging, 19% had abnormal results. Ophthalmologic complications were rare among patients overall, with the exception of those who had linear morphea. Of these cases, 1% had uveitis, and 9% had some other ophthalmologic condition.

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