Encyclopedia of Pathology

Living Edition
| Editors: J.H.J.M. van Krieken

Naevus, Unius Lateris

  • Jacqueline E. van der WalEmail author
Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-28845-1_746-1



Nevus unius lateris typically involves the skin of one half of the body with linear, papillary, or verrucous lesions in a unilateral distribution, usually along the axis of a limb or across the trunk. The epidermal nevi are arranged in a whorled fashion in the pattern of Blaschko’s lines. When there is a bilateral widespread distribution of the nevi, the condition is termed ichthyosis hystrix.

Lesions are usually congenital and are frequently associated with sensory disturbances. Oral involvement is rare. The oral lesions are papillary, wartlike proliferations with the same color as the surrounding normal mucosa with a typical linear distribution. They may be associated with hypodontia, morphologic tooth abnormalities, and unerupted teeth (Brown and Gorlin 1960; Haberland-Carrodeguas et al. 2008).

Clinical Features

  • Incidence: Oral involvement is extremely rare. From the first report in 1960 by Brown and Gorlin until 2010, only about 37 cases have been reported.

  • Age: The lesions mainly develop at birth or during the first months of life and show a tendency to enlarge slowly during childhood and stabilize by adolescence. Adult onset is rare (Kim et al. 2012).

  • Sex: There is no sex preference.

  • Site: Oral nevus unius lateris have been reported on the lips, tongue, buccal mucosa, palate, and gingivae.

  • Treatment: No treatment is needed, but local surgical excision might be necessary to come to a definitive diagnosis.

  • Outcome: No recurrences have been reported in the only few reported cases. Extensive lesions were present for many years without causing functional problems or malignant transformation.


Microscopically, oral nevus unius lateris shows papilliferous and hyperplastic epithelium often without or with minimal keratinization. Within the lamina propria a mild, chronic inflammatory infiltrate may be present (Hickman et al. 1988; Tesi and Ficarra 2010).

Differential Diagnosis

  • Squamous papillomas are frequently keratinized and therefore appear white unlike the papillary epithelial nevi.

  • In papillary hyperplasia of the palate, the papillae are much coarser and diffuse rather than discrete and frequently inflamed. Papillary hyperplasia of the palate is more common in denture wearers.

  • Oral verruca vulgaris: These lesions enlarge rapidly in a short period of time, while nevus unius lateris/epidermal nevi slowly enlarge during somatic growth.

References and Further Reading

  1. Brown, H. M., & Gorlin, R. J. (1960). Oral mucosal involvement in nevus unius lateris (ichthyosis hystrix). Archives of Dermatology, 81, 509–515.CrossRefPubMedGoogle Scholar
  2. Haberland-Carrodeguas, C., Allen, C. M., Lovas, J. G. L., Hicks, J., Flaitz, C. M., Carlos, R., & Stal, S. (2008). Review of linear epidermal nevus with oral mucosal involvement – series of five new cases. Oral Diseases, 14, 131–137.CrossRefPubMedGoogle Scholar
  3. Hickman, R. E., Eveson, J. W., & Cawson, R. A. (1988). Nevus unius lateris and intraoral verrucous nevi. Oral Surgery, Oral Medicine, and Oral Pathology, 66, 226–229.CrossRefPubMedGoogle Scholar
  4. Kim, I. S., Choi, S. Y., Park, K. Y., Li, K., Kim, B. J., Seo, S. J., Kim, M. N., & Hong, C. K. (2012). Annals of Dermatology, 24, 480–481.CrossRefPubMedPubMedCentralGoogle Scholar
  5. Tesi, D., & Ficarra, G. (2010). Oral linear epidermal nevus: A review of the literature and report of two new cases. Head and Neck Pathology, 4, 139–143.CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Springer International Publishing AG 2018

Authors and Affiliations

  1. 1.Department of Pathology, Antoni van Leeuwenhoek HospitalThe Netherlands Cancer InstituteAmsterdamThe Netherlands