Pediatric emergency medicine trisk 1349 1349
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Albright syndrome should be suspected when large, unilateral café-au-lait spots
with irregular borders occur in a segmental/Blaschkoid distribution.
Characteristics of this syndrome are bony abnormalities and precocious puberty.
Children who have large, swirling areas of pigmentation following lines of
Blaschko (lines representing planes of cutaneous embryogenesis) may have forms
of cutaneous mosaicism as can be seen in linear and whorled nevoid
hypermelanosis.
Melanocytic lesions are also important in the neonatal period. True malignant
melanoma is rare in children but can occur. Melanoma can arise de novo or within
a congenital melanocytic nevus. Giant congenital melanocytic nevi have a high
risk of malignant transformation, whereas small and medium have a much lower
risk of transformation. Patients with giant congenital melanocytic nevi are also at
an increased risk of neurocutaneous melanosis ( Fig. 69.18 ).
FIGURE 69.18 Congenital melanocytic nevus. Giant congenital melanocytic nevus on the
thigh of a neonate.
