Naevus-spilus-papulosus syndrome Q87.14

The nevus-spilus-papulosus sydrome, a neurocutaneous syndrome (Ramolia P et al. 2009), is characterized by a nevus spilus (speckled lentiginous nevus) of the papular type and by neurological anomalies, mostly ipsilaterally localized, in the form of motor neuropathies with muscle atrophies (Greywal T et al. 2018) or sensory neuropathies such as hyperhidrosis and/or dysesthesia (Happle R 2002).

mutation in the HRAS gene (11p15.5)

Mould penning syndrome; Naevus spilus papulosus

1. Greywal T et al (2018) Speckled lentiginous nevus: A rare presentation associated with motor neuropathy and muscular atrophy in a child. Pediatric Dermatol 35:e161-e162.
2. Happle R (2002) Speckled lentiginous nevus syndrome: delineation of a new distinct neurocutaneous phenotype. Eur J Dermatol 12:133-135.
3. Mendes M et al (2016) Speckled lentiginous nevus syndrome. Int J Dermatol 55:e602-e604.
4. Liu X et al (2015) Speckled lentiginous nevus syndrome associated with an abnormality of tongue. J Dermatol 42:433-434.
5. Ramolia P et al (2009) Speckled lentiginous nevus syndrome associated with musculoskeletal abnormalities. Pediatric dermatol 26:298-301.
6. Schaffer JV et al (2001) Speckled lentiginous nevus: within the spectrum of congenital melanocytic nevi. Arch Dermatol 137: 172-178