Café au Lait Macule

Image and content excerpted from the VisualDx clinical decision support system.

Image of Café au Lait Macule

VisualDx images show variation in age, skin color, and disease stage. VisualDx has 12 images of Café au Lait Macule.

Full text and additional images for Café au Lait Macule are available in the following VisualDx packages:

Pediatrics Complete

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ICD Codes

709.00 – Dyschromia unspecified

L81.3 – Café au lait spots


Café au lait macules (CALMs) are localized areas of hyperpigmentation. The term refers to the characteristic homogeneous color of "coffee with milk," which may be light to dark brown. Onset is usually evident in early childhood, although they may be present at birth; lesions may be solitary or multiple and increase in size with age. Single lesions are present in 10–20% of the US population. One percent of healthy young adults have up to 3 CALMs. Lesions are more common in darker-skinned races (3% of Hispanic and 18% of black newborns have them) and have a darker "espresso" pigmentation compared to those seen in whites.

They are located anywhere on the skin surface and vary in size but are usually 2–5 cm in adults. Multiple CALMs should alert the clinician to the possibility of an underlying systemic disease.

Disorders associated with multiple CALM are as follows – Neurofibromatosis (with 6 or more CALM), McCune-Albright syndrome, tuberous sclerosis, Fanconi anemia, Watson syndrome (a subset of neurofibromatosis associated with pulmonic stenosis), Noonan syndrome, cardio-facio-cutaneous syndrome, Jaffe-Campanacci syndrome, juvenile xanthogranulomas, Westerhof syndrome, piebaldism, Mukamel syndrome, LEOPARD (or Moynahan's) syndrome, Bannayan-Riley-Ruvalcaba syndrome, partial unilateral lentiginosis, Cowden's disease, gastrocutaneous syndrome, ataxia-telangiectasia, Bloom syndrome, multiple endocrine neoplasia, Johnson-McMillin syndrome, Tay syndrome, Silver-Russell syndrome, and Leschke's syndrome.

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