Further Delineation of Speckled Lentiginous Nevus Syndrome

Abstract

Speckled lentiginous nevus syndrome is a recently described condition, characterized by unilateral neurocutaneous manifestations consisting of: speckled lentiginous nevus, hyperhidrosis, muscular weakness and dysesthesia. Skeletal anomalies such as scoliosis, limb asymmetry, and psychiatric manifestations have been also reported. Herein, we report a case of speckled lentiginous nevus syndrome with skeletal anomalies and a pseudo-marfanoid phenotype.

Speckled lentiginous nevi in a young African‐American woman

“…Speckled lentiginous nevi may be an isolated finding or associated with syndromes such as speckled lentiginous nevus syndrome 3 . Speckled lentiginous nevus syndrome features the skin findings discussed above, along with neurological and musculoskeletal abnormalities such as hyperhidrosis, scoliosis, and muscle weakness and atrophy 2 . Our patient appears to have isolated speckled lentiginous nevi without an associated syndrome.…”

“…Our patient had the papular subtype. The distribution of nevi frequently follows the lines of Blaschko and likely represents a form of genetic mosaicism 2 . Speckled lentiginous nevi may be an isolated finding or associated with syndromes such as speckled lentiginous nevus syndrome 3 .…”