History: A one year old Caucasian female presented to the clinic because of the presence of a discoloration of the skin in her axillae and groin. The child had respiratory distress at birth secondary to choanal stenosis requiring tracheostomy. In addition, she had unicoronal stenosis and hydrocephalus necessitating a VP shunt.

Physical Exam: The patient showed acrocephaly ( premature closure of lamboid and coronal sutures resulting in an abnormally high peaked skull), a beak-shaped nose, hypoplastic maxilla, a protruding lower lip and hypertelorism Figures 1 &2)

1. 2.

A velvety, yellowish discoloration of the skin of both axillae ( Figure 3) and the groin was present.


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