Café-au-lait stain L81.3

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 11.12.2021

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Synonym(s)

Café-au-lait-macule; Cafe-au lait stain; Cafe-au-lait stains; Café au lait stains; CALM; Coffee latte stain; Lactic coffee colours

Definition
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Congenital or acquired in early childhood, homogeneous, pale-brown, sharply demarcated, non-palpable brown or brown-yellow spots or patches (= spots >1.0 cm ) -s.a. hyperpigmentation.

Single (up to 3) of these patches are found in 3% of all newborns and in 10-28% of all older children.

If there are 6 or > 6 café au lait spots of > 0.5 cm (prepubertal) and > 1.5 cm (postpubertal), there is evidence of systemic disease. Also in case of very large café au lait spots in segmental or blaschcoid arrangement a malformation syndrome (see below) has to be considered.

Occurrence/Epidemiology
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Common: 10-20% of the normal population.

Manifestation
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congenital or acquired in early childhood.

Localization
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Torso and extremities.

Clinical features
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Sharply demarcated, homogeneously colored, circular or oval, nonsplattering, light brown (milk coffee colored), asymptomatic spots and patches of variable size. Rarely, there is a naevoid arrangement in the Blaschko lines or a unilateral arrangement (suggestive of neurofibromatosis type V). A single very large café-au-lait patch does not appear to indicate a clinical syndrome. Reported associations of café-au-lait spots with childhood medulloblastomas.

Clinical syndromes with café-au-lait spots (more than 6 café-au-lait spots indicate a syndromal constellation):

Histology
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S.a. Lentigo simplex. Homogeneous hyperpigmentation of the str. basale (Melan A representation), increased melanocyte numbers, giant melanosomes.

Therapy
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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/forecast
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Café au lait stains have no tendency to develop malignancy. Especially during childhood size growth is observed

Literature
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  1. Goldberg DJ (1997) Laser treatment of pigmented lesions. Dermatol Clin. 15:397
  2. Kansal NK et al.(2017) Association of piebaldism with café-au-lait macules. Skinmed 15: 223-225.
  3. Langenbach N et al. (1998) Nevi 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.
  4. 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.
  5. Marinău LD et al (2017) Two girl patients with medulloblastoma. Case reports. Rome J Morphol Embryol 58:1103-1108.
  6. 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
  7. Shah KN (2010) The diagnostic and clinical significance of café-au-lait macules.Pediatr Clin North Am 57:1131-11253 .

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 11.12.2021