DefinitionThis section has been translated automatically.
Congenital or acquired in early childhood, homogeneous, pale-brown coloured, sharply defined, non-palpable brown or brown-yellow patches or spots (larger than 1,0 cm) -s.a. hyperpigmentations-. Single (up to 3) of these patches are found in 3% of all newborns and in 10-28% of all older children. 6 or > 6 café-au-lait spots of > 0.5 cm (prepubertal) and > 1.5 cm (postpubertal) indicate a systemic disease. Even in the case of very large café au lait stains in segmental or blaschkoid arrangement a malformation syndrome must be considered.
Occurrence/EpidemiologyThis section has been translated automatically.
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ManifestationThis section has been translated automatically.
congenital or acquired in early childhood.
LocalizationThis section has been translated automatically.
Torso and extremities.
Clinical featuresThis section has been translated automatically.
- Sharply defined, homogeneously coloured, circular or oval, not splashy, light brown (milky coffee-coloured), symptom-free spots and patches of various sizes. Rare is a naevoid arrangement in the Blaschko lines or a unilateral arrangement (indication of neurofibromatosis type V). A single very large café-au-lait patch does not seem to indicate a clinical syndrome. Associations of café-au-lait spots with childhood medulloblastoma have been reported.
- Clinical syndromes with café-au-lait spots (more than 6 café-au-lait spots indicate a syndromal constellation):
HistologyThis section has been translated automatically.
TherapyThis section has been translated automatically.
Usually not necessary, it is rather advised against a therapy. In case of cosmetic indication, possibly an attempt with laser treatment of superficial cryosurgery or dermabrasion. The risk of laser surgery is either recurrence, scarring, depigmentation or a spotty overall result. A study with complete regression through treatment with a pulsed dye laser is available (Goldberg DJ 1997).
Progression/forecastThis section has been translated automatically.
Café au lait stains have no tendency to develop malignancy. Especially during childhood size growth is observed
LiteratureThis section has been translated automatically.
- Goldberg DJ (1997) Laser treatment of pigmented lesions. Dermatol Clin. 15:397
- Kansal NK et al.(2017) Association of Piebaldism with Café-au-Lait Macules. Skinmed 15: 223-225.
- Langenbach N et al (1998) Naevi spili, Café-au-lait spots and melanocytic naevi aggregated alongside Blaschko's lines, with a review of segmental melanocytic lesions.Acta Derm Venereol 78:378-380.
- Lo FS et al (2017) Detection of Rare Somatic GNAS Mutation in McCune-Albright Syndrome Using a Novel Peptide Nucleic Acid Probe in a Single Tube. Molecules 22. pii: E1874.
- Marinău LD et al. (2017) Two girl patients with medulloblastoma. Case reports. Rom J Morphol Embryo 58:1103-1108.
- Nguyen JT et al (2004) Large solitary café au lait spots: a report of 5 cases and review of the literature. Cutis 73:311-314
- Shah KN (2010) The diagnostic and clinical significance of café-au-lait macules.pediatr Clin North Am 57:1131-11253. https://www.ncbi.nlm.nih.gov/pubmed/20888463
Incoming links (20)Bannayan-riley-ruvalcaba syndrome; Bloom syndrome; Fanconi anaemia; Fèvre-languepin syndrome; Hypomelanosis ito; Juvenile xanthogranuloma; Legius-syndrome; Lentigines syndromes; Lentiginosis partiale unilaterale; Lentigo simplex; ... Show all
Outgoing links (22)Ataxia teleangiectatica; Bannayan-riley-ruvalcaba syndrome; Becker's nevus; Berloque dermatitis; Bloom syndrome; Cobb syndrome; Cowden syndrome; Cryosurgery; Dermabrasion; Fèvre-languepin syndrome; ... Show all
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