Site of morphea lesions predicts the risk of extracutaneous manifestations.
LAKE TAHOE, CALIF. -- 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.
"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.
Among all patients, the researchers found that left-extremity and extensor-extremity lesions had a stronger association with musculoskeletal involvement (odds ratios of 1.26 and 1.94, respectively). "The reasons for this are unclear," Dr. Chiu said. "We didn't assess handedness in our study, but that perhaps could explain it; 90% of the general population is right-hand dominant, so perhaps there's some sort of protective effect if you're using an extremity more. Joint contractures showed the greatest discrepancy between left and right extremity. So perhaps if you're using that one side more, you're less likely to have a joint contracture."
When the researchers limited the analysis to head lesions, they observed no significant difference in the lesions between the left and right head (OR, 0.72), but anterior head lesions had a stronger association with neurologic signs or symptoms, compared with posterior head lesions (OR, 2.56), as did superior head lesions, compared with inferior head lesions (OR, 2.23). The association between head lesion location and ophthalmologic involvement was not significant.
"The odds of extracutaneous manifestations vary by site of morphea lesions, with higher odds seen on the left extremity, extensor extremity, the anterior head, and the superior head," Dr. Chiu concluded. "Further research can be done to perhaps help us decide whether this necessitates difference in management or screening."
The project was funded by the Pediatric Dermatology Research Alliance and the SPD. Dr. Chiu reported having no relevant financial disclosures.
COMMENTARY BY DR. SIDBURY
STRATIFYING EXTRACUTANEOUS MANIFESTATIONS WITH LINEAR MORPHEA
Linear morphea has been associated with musculoskeletal and neurologic morbidity. Chiu et al. in the Pediatric Dermatology Research Alliance (PeDRA) conducted a multicenter, retrospective study to drill down on these associations.
While other types of morphea rarely have extracutaneous manifestations, the linear subtype had the highest incidence: 34% had musculoskeletal involvement, 24% neurologic, and 10% ophthalmologic. A fascinating and novel finding was the strong predilection for left extremity musculoskeletal morbidity, particularly contracture. Although handedness was not captured, a plausible explanation may be that increased utilization of an affected extremity may decrease morbidity. Similarly, anterior head involvement was more likely (odds ratio, 2.56) associated with neurologic sequelae, although reasons for this remain unclear.
All patients diagnosed with linear morphea should be screened for musculoskeletal, neurologic, and ophthalmologic morbidity, but Dr. Chiu's work will help pediatricians risk stratify.
BY DOUG BRUNK
Caption: DR. CHIU
Caption: A morphea lesion is evident on the thigh of this child.
Caption: A morphea lesion is seen on the thigh of this child, with purple marks indicating the extent of the lesion.
Please Note: Illustration(s) are not available due to copyright restrictions.
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|Date:||Jun 1, 2019|
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