Speckled lentiginous nevus L81.4

Vaguely defined disease term for a unilateral or segmental lentiginosis without further indication of other systemic dysplasias (Schaffer JV et al. 2001). The term is sometimes equated with "nevus spilus" (Schaffer JV et al. 2001). It is possible that the initial description was also based on a (segmental) neurofibromatosis.

To what extent the "speckledlentiginous nevus" syndrome described by R.Happle, which is characterized by speckled lentiginous nevi in association with hyperhidrosis, muscle weakness, dysesthesia or other neurological abnormalities, has an independent character, remains to be seen in further clinical observations.

1. Happle R (2002) Speckled lentiginous nevus syndrome: delineation of a new distinct neurocutaneous phenotype. Eur J Dermatol12:133-135.
2. Schaffer JV et al (2001) Speckled lentiginous nevus: within the spectrum of congenital melanocytic nevi. Arch Dermatol 137:172-178.
3. Touraine, A. Une nouvelle neuro-ectodermose congenitale: la lentiginose centro-faciale et ses dysplasies associees. Ann. Derm. Syph. 8: 453-473, 1941.