Child in hoerse- riding stance.

Abstract

A 6-year-old girl presented with progressive tightness of skin over pelvic and shoulder girdles resulting in stiffness of extremities giving her a fixed horse-riding stance [Figure 1]. It started as an ill-defined stony hard area noticed on the lateral side of right buttock at 2 years of age and there was a gradual progression of skin induration over proximal extremities. There was exaggerated lumbar lordosis, outer aspects of both the buttocks were flat and hollowed, and lower back and lumbar region showed mild hypertrichosis [Figures 1 and 2]. Skin on the involved body parts was rock-hard and not pinchable. Face, neck, hands, and feet were not involved. There was no history of Raynaud's phenomenon, arthralgia, dyspnea, or dysphagia. Hemogram, ESR, random blood sugar, renal function, and urinalysis were within normal limits. Antinuclear antibody test was negative. A deep biopsy was taken from an involved area of her left thigh including skin and underlying fascia.