Speckled Lentiginous Nevus Syndrome Associated with Musculoskeletal Abnormalities

Authors: Ramolia, Paras¹; Treadwell, Patricia²; Haggstrom, Anita³

Source: Pediatric Dermatology, Volume 26, Number 3, May/June 2009 , pp. 298-301(4)

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Abstract:

Speckled lentiginous nevus syndrome is a recently described neurocutaneous disorder characterized by speckled lentiginous nevus in association with hyperhidrosis, skin dysesthesia, and muscular weakness in an ipsilateral manner. We report a 9-year-old boy with a large speckled lentiginous nevus on his trunk, bilateral upper extremities, and neck associated with hyperhidrosis and nonspecific pain in the distribution of the nevus in addition to prominent upper extremity asymmetry and scoliosis. Similar musculoskeletal abnormalities have been reported in patients with phacomatosis pigmentovascularis and phacomatosis pigmentokeratotica which feature speckled lentiginous nevus; however, this patient demonstrates striking musculoskeletal abnormalities in the absence of coexisting vascular lesions or nevus sebaceous.

Document Type: Research article

DOI: http://dx.doi.org/10.1111/j.1525-1470.2009.00906.x

Affiliations: 1: Indiana University School of Medicine, Indianapolis, Indiana 2: Department of Pediatrics, Indiana University, Indianapolis, Indiana 3: Departments of Dermatology and Pediatrics, Indiana University, Indianapolis, Indiana

Publication date: 2009-05-01