Case #30, 20th Annual Uniformed Services Dermatology Seminar

Presenter: David S. Mezebish, LT, MC, USN

A 23 year old black female with a lifelong history of irregular "spattered and whorled" pigmentation on her chin, trunk, back, and left thigh. The patient states that her mother had similar pigment abnormalities, which completely resolved by late adolescence, but has persistent dental abnormalities and a patch of alopecia on the vertex of her scalp.

PE: Otherwise healthy appearing black female with dark brown spattered and whorled hyperpigmentation on her chin, trunk, back and left thigh, following Blaschko's lines. The patient also has a blue-black discoloration of her right central incisor tooth. There are no obvious hair or bony abnormalities.

Histopathologic findings: Hyperkeratosis, focal hypergranulosis, and marked pigmentary incontinence, consistent with late stage incontinentia pigmenti.