Original article
Skin mapping for the classification of generalized morphea

https://doi.org/10.1016/j.jaad.2016.08.052Get rights and content

Background

Generalized morphea lacks cohesive clinical features, limiting its clinical and investigative utility.

Objective

We sought to use computerized lesion mapping to objectively subtype morphea.

Methods

We conducted a 2-part cross-sectional study. In part 1, we created a discovery cohort of patients with generalized morphea of whom lesion maps were created to characterize subsets. Clinical and demographic features were compared between proposed subsets to determine if they identified clinically relevant differences. In part 2, we created a validation cohort to determine if proposed criteria were applicable to different individuals.

Results

A total of 123 patients with generalized morphea were included. Mapping produced 2 distribution patterns that encompassed the majority in both cohorts: isomorphic (areas of skin friction) and symmetric (symmetrically distributed on trunk/extremities). In the discovery cohort, the isomorphic subset was older (55.6 ± 12.7 vs 42.2 ± 20.1 years, P < .001), all female (30/30 vs 38/43, P = .05), and more often had lichen sclerosus changes (12/43 vs 8/43, P = .02); involvement of the reticular dermis, subcutaneous fat, and/or fascia was more common in symmetric (10/43 vs 1/30) (P = .02). These features persisted in the validation cohort.

Limitations

Single cohort was a limitation.

Conclusions

Symmetric and isomorphic subsets possess distinctive demographic and clinical features, suggesting they more accurately define the phenotype of generalized morphea. Consideration should be given to revising classification.

Section snippets

Methods

This is a 2-part cross-sectional study of participants of the prospective MAC cohort at the time of their enrollment. First, computerized lesion mapping was used to characterize lesion distribution patterns (discovery cohort) in generalized morphea, which created 2 proposed subsets of generalized morphea. Secondly, these distribution patterns were applied to an independent set of participants at their enrollment into the MAC cohort (validation cohort).

To create the discovery cohort, a

Participants

In all, 73 participants met inclusion criteria for the discovery cohort (of 93 patients with generalized morphea enrolled in the MAC cohort at that time). We also identified 20 separate patients with multiple linear lesions meeting criteria for generalized morphea as defined by Laxer and Zulian5 (of 217 total patients with linear morphea). The clinical and demographic features of these patients were compared with the 73 participants with generalized, but not linear, lesions. The multiple linear

Discussion

The demographic and clinical features of generalized morphea are poorly defined, to the detriment of studies aimed at identifying genetic and mechanistic features. We performed a cross-sectional study using computerized lesion mapping to more accurately identify disease subsets based on distribution of cutaneous lesions, and identified 2 clinically relevant subsets of generalized morphea with distinct demographic and clinical features.

Initial analysis revealed that current criteria for

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Cited by (0)

Research for this manuscript was supported in part by National Institutes of Health (NIH) grant no. K23AR056303-5. This work was conducted with support from the University of Texas-Science and Technology Acquisition and Retention Program (UT-STAR), NIH/National Center for Research Resources (NCRR) grant number 4UL1TR001105-04/National Center for Advancing Translational Sciences grant no. UL1TR000451. The content is solely the responsibility of the authors and does not necessarily represent the official views of UT-STAR, University of Texas Southwestern Medical Center at Dallas and its affiliated academic and health care centers, NCRR, or NIH.

Conflicts of interest: None declared.

Reprints not available from the authors.

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