Atypical Café-au-Lait Macules in a Patient with Koolen-de Vries Syndrome (17q21.31 Microdeletion Syndrome).

Koolen-de Vries syndrome (KdVS), also referred to as the 17q21.31 microdeletion syndrome, is a rare genetic disorder characterized by developmental delay, typical facial dysmorphism, and congenital defects. Associated anomalies include many cutaneous findings.

Bilateral facial pigmentation.

A 38-year-old man presented with a chief complaint of blue-speckled secretions on his cheeks, brought on by exertion. Based on the clinical features, a diagnosis of apocrine chromhidrosis was made. Histopathologic exam further supported this diagnosis.