Abstract

Incontinentia Pigmenti, also known as Bloch-Sulzberger syndrome, is a rare X-linked dominant disorder considered to be lethal in males. The syndrome usually presents at birth or shortly thereafter and the skin rash evolves in the classic four stages of vesicular, verrucous, hyperpigmented, and hypopigmented lesions following a blaschko linear distribution. Here we present an unusual case of Incontinentia Pigmenti presenting with papules and plaques preceding the classic evolution of eruptions.