Eleven‐year‐old girl with skin hardening and restricted mobility in the lower limbs

A healthy 11-year-old Caucasian girl came to our Clinic because of a hardening of the skin in both legs, first noticed 5 years ago. She had been born from healthy nonconsanguineous parents and had developed well. The process was first noticed on the anterior left thigh and had progressed distally, involving the other extremity and the lower left abdomen as well; the hard consistency had stabilized after a few months and showed no progression thereafter. At the time of presentation, the patient was diagnosed with scleroderma and was treated with oral corticosteroids for 3 months without improvement. On physical examination, there was a stone-hard induration of the subcutaneous tissue of both lower limbs, more noticeable on the left thigh; the overlying skin showed no sclerosis or discoloration. The skin hardening clearly spared the inguinal areas (fig. 1) and was unremarkable on the knees and feet. The patient also had a striking lumbar lordosis, and a slight flexion contracture of the left lower limb which caused a minor difficulty in walking (fig. 2). Histologic study showed dermal fibrosis, with thickened collagen bundles within the deep dermis that extended into the subcutaneous fat. There was no inflammatory infiltrate, and the hair follicles and sweat glands were well preserved (figs 3–5). The fascia was not