Lentiginosis centrofacialisQ82.8

Author:Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 05.12.2021

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Synonym(s)

centrofacial lentiginosis; lentiginosis centrofacial

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HistoryThis section has been translated automatically.

Touraine described this disease for the first time (Touraine, 1941). He discovered a total of 32 cases in 17 families studied. In 9 of the families, one parent and one or more children were affected. In 5 families with a total of 15 cases, only 2 or more siblings were affected. Mental retardation was frequently present. Dociu later compiled a series of 40 cases and emphasized that no other lentigines were found except in the face (Dociu I et al. 1976).

DefinitionThis section has been translated automatically.

Rare, probably autosomal-dominantly inherited syndrome with facial lentigines and associated malformations. The genetic defect of this (neuro-)ectodermal dysplasia is unknown so far.

ManifestationThis section has been translated automatically.

Occurs in the 1st year of life, increase during childhood.

LocalizationThis section has been translated automatically.

Located in the center of the face. Lateral cheek areas and mucous membranes are free.

Clinical featuresThis section has been translated automatically.

Small brownish or black spots.

Associated symptoms: Spina bifida, hypertrichosis sacralis, kyphoscoliosis, funnel chest, absence of maxillary central incisors, mental retardation with intelligence reduction, primary keratoses, epilepsy.

Differential diagnosisThis section has been translated automatically.

Ephelides

Peutz-Jeghers syndrome

Carney complex

TherapyThis section has been translated automatically.

No causal therapy possible, if necessary cosmetic cover (e.g. Dermacolor).

LiteratureThis section has been translated automatically.

  1. Dociu I et al (1976) Centrofacial lentiginosis. Brit J Derm 94: 39-43.

  2. Touraine A (1941) Lentiginosis centro-faciale et dysplasies associées. Bull Soc Fr Dermatol Syphiligr 48: 518-522.
  3. Touraine A (1941) Une nouvelle neuro-ectodermose congenitale: la lentiginose centro-faciale et ses dysplasies associées. Ann Dermatol Syphiligr (Paris) 8: 453-473.

  4. Vakos J et al (1961) Neurodysraphic centro-facial lentiginosis. Presse Med 69:1349-1351.

Authors

Last updated on: 05.12.2021

Author: Prof. Dr. med. Peter Altmeyer