Systematized inflammatory epidermal nevus with symmetrical involvement: an unusual case of CHILD syndrome?

The CHILD syndrome (congenital hemidysplasia with ichthyosiform nevus and limb defects) is usually characterized by lateralization of all associated anomalies. It has been assumed that the event of X-inactivation coincides and interferes with a clone of organizer cells controlling a large developmental field. A 16-year-old girl with bilateral manifestations of CHILD syndrome is described. The inflammatory skin lesions affected the body folds (ptychotropism) in a symmetrical distribution, although only the right side of the neck was involved. In addition, absence of several facial muscles, vertebral defects, and shortening of the leg on the right side were noted, and a ventricular septum defect was present. This unusual case may be explained by the assumption that X-inactivation did not coincide with the origin of inducer cell clones controlling large morphogenetic fields on either side of the body.