Core Dermatology Diagnoses / CategoriesNeoplastic Benign and Malignant

Café au lait spot

Author: Faculty Reviewer:

Publish date: Posted on
Last updated: February 2, 2022

Keywords #

café-au-lait
café-au-lait macules (CALMs)
coast of California
coast of Maine
genodermatosis

Diagnosis #

Café–au-lait macules (CALMs) are flat, hyperpigmented, irregularly-shaped skin lesions (macules or patches) that may be present at birth and may grow in number and size. Stemming from the French language, CALMs get their unique name from their typical coloring, which resembles “coffee with milk.” They appear most frequently on the trunk and extremities and are benign. However, some genodermatoses are associated with CALMs, the most common of which is neurofibromatosis 1 (NF1). Others include Legius syndrome, Watson syndrome, Noonan syndrome, McCune-Albright syndrome, ring chromosome syndromes, and constitutional mismatch repair deficiency.[1]

Key Concepts #
  • CALMs are benign skin lesions common in the general population. 
  • If a patient has 6 or more CALMs, or one especially large CALM, consider an underlying genetic disorder. 
Epidemiology #

CALMs are seen in 2.7% of newborns and 28% of school-age children. Three or more CALMs are found in 1% of children and 14% of adults.[3] 

Clinical Features #
  • Flat, tan, irregularly-shaped, hyperpigmented macules or patches that may be present at birth and may grow in number and size.[1] 
  • Appear more frequently on the trunk and extremities and rarely involve the face.[1] 
Differential Diagnoses #
Diagnostic Workup #

A skin biopsy can confirm the diagnosis, though this is rarely necessary. Solitary lesions are not associated with systemic disease, though the presence of multiple CALMs, or one especially large CALM, should elicit consideration of associated genetic syndromes. 

Treatment #

CALMs generally do not require treatment. Excision or laser approaches may be helpful if CALMs bother a patient cosmetically. 

References #
  1. Anderson, Sharon. “Café Au Lait Macules and Associated Genetic Syndromes.” Journal of Pediatric Health Care 34.1 (2020): 71-81. Web. 
  2. Hernández-Martín, A, and Duat-Rodríguez, A. “An Update on Neurofibromatosis Type 1: Not Just Café-au-Lait Spots, Freckling, and Neurofibromas. An Update. Part I. Dermatological Clinical Criteria Diagnostic of the Disease.” Dermatology (Actas Dermo-Sifiliográficas, English Edition) 107.6 (2016): 454-64. Web. 
  3. Ben-Shachar, Shay, Dubov, Tom, Toledano-Alhadef, Hagit, Mashiah, Jacob, Sprecher, Eli, Constantini, Shlomi, Leshno, Moshe, and Messiaen, Ludwine M. “Predicting Neurofibromatosis Type 1 Risk among Children with Isolated Café-au-lait Macules.” Journal of the American Academy of Dermatology 76.6 (2017): 1077-083.e3. Web. 
  4. Balaraman, Brundha, Ravanfar-Jordan, Parisa, and Friedman, Paul M. “Novel Use of Non-ablative Fractional Photothermolysis for Café-au-lait Macules in Darker Skin Types.” Lasers in Surgery and Medicine 49.1 (2017): 84-87. Web. 
Contents