Segmental stiff skin syndrome (SSS): Two additional cases with a positive response to mycophenolate mofetil and physical therapy

To the Editor: Recently, Myers et al1 described a distinct subtype of the stiff skin syndrome (SSS) characterized by a segmental distribution, delayed onset, and reduced morbidity. Reviewing their cohort of 4 patients and an additional 48 cases from the literature, the authors reported that segmental SSS comprises up to one third of all SSS cases. Histopathologic assessments from their patients revealed thickened and horizontally oriented collagen and adipocyte entrapment without inflammation, features that typify the widespread form and are useful to differentiate the disorder from diagnostic mimickers with linear and segmental patterns, such as morphea and connective tissue nevi.

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