More on Speckled Lentiginous Nevus Syndrome

Further Delineation of Speckled Lentiginous Nevus Syndrome

“…Risk of malignant melanoma transformation exists. Skeletal abnormalities have been reported in several cases [6][7][8][9] , but our patient appears to be the fi rst to disclose a marfanoid habitus. .…”

“…This dermatologic lesion is described in diff erent disorders: phacomatosis pigmentokeratotica, Schimmelpenning syndrome, segmental neurofi bromatosis, LEOPARD syndrome, Carney complex and FACES syndrome. Speckled lentiginous nevus syndrome (SLNS) was described by Happle as a neurocutaneous manifestation consisting of unilateral speckled lentiginous nevus, hyperhidrosis, muscular weakness and dysesthesia [ 4] , associated with median nerve paresis [ 5] and skeletal anomalies such as scoliosis, limb asymmetry and psychiatric manifestations [6][7][8][9] . Speckled lentiginous nevus syndrome was reported in 10 patients [4][5][6][7][8][9][10][11][12][13][14] .…”

Speckled lentiginous nevus: A rare presentation associated with motor neuropathy and muscular atrophy in a child

“…Muscular and neurologic abnormalities are rarely associated but have been reported, especially in SLNS . SLNS is an uncommon neurocutaneous syndrome that is described in the literature in a few case reports that typically present adults with segmental SLN in association with other symptoms such as ipsilateral hyperhidrosis, sensory neuropathy (dysesthesia, paresthesia, hyperalgesia), motor neuropathy, nerve palsy with thinning of the nerve, and spinal muscular atrophy with fasciculation …”

Speckled Lentiginous Nevus Syndrome: Central Nervous System Abnormalities as a Critical Diagnostic Feature

“…Second, torsion dystonia was at least initially unilateral, involving an upper limb (possibly the left one, also affected by the SLN, although it was not specified). Third, an ipsilateral peripheral anomaly may occur later on, even well after childhood (3).…”