Figure 1.

Clinical and radiologic features. (A) Front view showing limitation of the extension of the right elbow with accentuation of the cutaneous articular fold. (B) Back view demonstrating hypoplasia of the right scapula; note a small café-au-lait spot in the upper scapular region. Radiological findings of the right upper limb. (C) Thickening of the dermis of the lateral side of the arm at Magnetic Resonance Imaging (MRI) coronal view (arrow). (D) Thickened dermis at the scapular level (arrow). (E) Thickened dermis surrounding the arm muscles at axial view (arrows). (F) Plain radiograph documenting hypoplasia of the inferior part of the right scapula. Radiological findings of the left upper limb. Comparison with similar MRI sections of the affected (right) side demonstrated normal representation of the dermis at coronal (G), and upper (H) and lower (I) axial views. Muscles are underdeveloped in the right arm. (J) Normal left scapula at standard X-rays.