Unilateral generalized morphea is a rare variant of localized scleroderma

Abstract

Localized scleroderma (LS) is a rare connective tissue disorder generally involving the entire dermis and usually limited to the subcutaneous tissue. However, it may progress to large indurated plaques, growth retardation, muscle atrophy, and even to flexion deformities or poorly healing ulcerations. LS has been classified as plaque, generalized, bullous, linear, and deep forms exhibiting different clinical subtypes. Recently, an unusual case of unilateral generalized morphea (UGM) in childhood extending from the middle dermis to the subcutaneous fat tissue has been reported. We here describe four young patients exhibiting a similar subtype of LS. All patients demonstrated a prominent unilateral skin involvement starting in childhood or adolescence. Histology revealed prominent accentuation of intradermal involvement. Except for positive anti-nuclear antibodies, no specific antibody pattern could be observed. In presenting these clinically homogenous cases we hereby introduce UGM as an extreme variant of the linear form of LS in childhood. As the onset of UGM usually occurs in pediatric patients, pediatricians should be cognizant of the presentation of this uncommon condition. Treatment with combined low-dose methotrexate and pulsed high-dose corticosteroid therapy might represent a promising treatment option for UGM.