Pediatric emergency medicine trisk 2947 2947

FIGURE 101.3 Hemiatrophy of left leg because of linear scleroderma. Note normal appearing size,
muscle mass, and overall bulk of normal right leg. (Courtesy of Shriners Hospitals for Children,
Houston, Texas.)

The far more serious form, JSSc, is also very rare, occurring in fewer than 1,000
children nationwide. JSSc is a chronic, multisystem, connective tissue disorder, where
hardening of the skin is combined with fibrous changes in the internal organs. JSSc
often presents with cutaneous changes such as RP (90% of patients), edema, induration,
increased pigmentation, and tightening of the skin. Some of these children may also
develop arthritis resembling JIA, muscle weakness resembling juvenile
dermatomyositis (JDM), and nodules along tendon sheaths. If these features are seen,
one should consider the possibility of an overlap syndrome, in which features of SLE,
JSSc, JDM, and JIA intermingle.