Figure 3

Patient 2 shows a senile appearance with hyper- or hypo-pigmentation, sclerodermatous changes and thin skin on the lower abdomen, buttocks, elbows and the lower extremities, lipodystrophy of the gluteal region and extremities (A-C); sparse scalp hair with easily visible veins (D); irregular and carious teeth and crowded teeth on the mandible (E,F); severe contractures at the interphalangeal joints and the hands with abnormal posture (G,H), and coarse and thickened skin around the ankles and on the back of feet in symmetry (I). X-ray findings of hand show flexion deformity of fingers of hands and delayed bone age (J). Radiological changes in chest reveal pyriform thorax , absence of right clavicle, and absence of mid-lateral part with thin interior part of left clavicle, and thin posterior parts of the 2^nd and 3^rd ribs on both sides with disconnection of posterior parts of the 3^rd rib on left side and the 2^nd and 3^rd on the right (K).