eMedicine Specialties > Pediatrics: General Medicine > Dermatology

Cafe Au Lait Spots: Treatment & Medication

Author: William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
Coauthor(s): Raj D Sheth, MD, Professor of Neurology, Mayo College of Medicine; Chief, Division of Pediatric Neurology, Nemours Children's Clinic; Nazanin Saedi, MD, Resident Physician, Department of Dermatology, University of California, Irvine, School of Medicine
Contributor Information and Disclosures

Updated: Jul 20, 2009

Treatment

Medical Care

Café au lait macules (CALMs) do not require medical care. When café au lait spots are associated with neurofibromatosis (NF) or another underlying condition, monitoring of associated conditions is required.

Although treatment of these lesions is not necessary, several lasers have been used to treat café au lait macules with variable responses. The risks of the procedures must be discussed with the patient and the family. The risks of laser surgery include transient hyperpigmentation, hypopigmentation, slight scarring, permanent hyperpigmentation, and recurrence.6 The data for the use of repeated Q-switched laser treatments are not consistent, with approximately 50% experiencing total clearance and with the other half developing recurrence and patchy pigmentation.7 The reported responses to frequency-doubled Nd:YAG vary.8 In one study, complete clearance of 34 café au lait macules was reported using a pulsed dye laser for 4-14 treatments, with no recurrences at 12 months follow-up.9

More on Cafe Au Lait Spots

Overview: Cafe Au Lait Spots
Differential Diagnoses & Workup: Cafe Au Lait Spots
Treatment & Medication: Cafe Au Lait Spots
Follow-up: Cafe Au Lait Spots
Multimedia: Cafe Au Lait Spots
References
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References

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  2. Abeliovich D, Gelman-Kohan Z, Silverstein S, et al. Familial cafe au lait spots: a variant of neurofibromatosis type 1. J Med Genet. Dec 1995;32(12):985-6. [Medline].

  3. Tekin M, Bodurtha JN, Riccardi VM. Cafe au lait spots: the pediatrician's perspective. Pediatr Rev. Mar 2001;22(3):82-90. [Medline].

  4. De Schepper S, Boucneau J, Vander Haeghen Y, et al. Cafe-au-lait spots in neurofibromatosis type 1 and in healthy control individuals: hyperpigmentation of a different kind?. Arch Dermatol Res. Apr 2006;297(10):439-49. [Medline].

  5. Medina YN, Rapaport R. Evolving diagnosis of McCune-Albright syndrome. atypical presentation and follow up. J Pediatr Endocrinol Metab. Apr 2009;22(4):373-7. [Medline].

  6. Carpo BG, Grevelink JM, Grevelink SV. Laser treatment of pigmented lesions in children. Seminars in Cutaneous Medicine and Surgery. 1999;18:233-243. [Medline].

  7. Stratigos AJ, Dover JS, Arndt KA. Laser treatment of pigmented lesions- 2000: how far have we gone?. Arch Dermatol. 2000;136(7):915-21. [Medline].

  8. Goldberg DJ. Laser treatment of pigmented lesions. Dermatol Clin. 1997;15:397-407. [Medline].

  9. Alster TS. Complete elimination of large cafe-au-lait birthmarks by the 510-nm pulsed-dye laser. Plast Reconstr Surg. 1995;96:1660-64. [Medline].

  10. [Guideline] Radtke HB, Sebold CD, Allison C, Haidle JL, Schneider G. Neurofibromatosis type 1 in genetic counseling practice: recommendations of the National Society of Genetic Counselors. J Genet Couns. Aug 2007;16(4):387-407. [Medline].

  11. Benedict PH, Szabo G, Fitzpatrick TB, Sinesi SJ. Melanotic macules in Albright's syndrome and in neurofibromatosis. JAMA. Aug 26 1968;205(9):618-26. [Medline].

  12. Cnossen MH, Moons KG, Garssen MP, et al. Minor disease features in neurofibromatosis type 1 (NF1) and their possible value in diagnosis of NF1 in children < or = 6 years and clinically suspected of having NF1. Neurofibromatosis team of Sophia Children's Hospital. J Med Genet. Aug 1998;35(8):624-7. [Medline].

  13. Cohen JB, Janniger CK, Schwartz RA. Cafe-au-lait spots. Cutis. Jul 2000;66(1):22-4. [Medline].

  14. De Schepper S, Boucneau J, Lambert J, et al. Pigment cell-related manifestation in neurofibromatosis type 1: an overview. Pigment Cell Research. 2005;18:13-24. [Medline].

  15. Dohil MA, Baugh WP, Eichenfield LF. Vascular and pigmented birthmarks. Pediatric Clinics of North America. 2000;47:783-812. [Medline].

  16. Eichenfield LF, Gibbs NF. Hyperpigmentation disorders. In: Textbook of Neonatal Dermatology. 2000:370.

  17. Friedman JM. Epidemiology of neurofibromatosis type 1. Am J Med Genet. Mar 26 1999;89(1):1-6. [Medline].

  18. Friedman JM, Birch PH. Type 1 neurofibromatosis: a descriptive analysis of the disorder in 1,728 patients. Am J Med Genet. May 16 1997;70(2):138-43. [Medline].

  19. Hofman KJ, Boehm CD. Familial neurofibromatosis type 1: clinical experience with DNA testing. J Pediatr. Mar 1992;120(3):394-8. [Medline].

  20. Hofman KJ, Harris EL, Bryan RN, Denckla MB. Neurofibromatosis type 1: the cognitive phenotype. J Pediatr. Apr 1994;124(4):S1-8. [Medline].

  21. Johnson BL, Charneco DR. Cafe au lait spot in neurofibromatosis and in normal individuals. Arch Dermatol. Oct 1970;102(4):442-6. [Medline].

  22. Legius E, Descheemaeker MJ, Steyaert J, et al. Neurofibromatosis type 1 in childhood: correlation of MRI findings with intelligence. J Neurol Neurosurg Psychiatry. Dec 1995;59(6):638-40. [Medline].

  23. Listernick R, Darling C, Greenwald M, et al. Optic pathway tumors in children: the effect of neurofibromatosis type 1 on clinical manifestations and natural history. J Pediatr. Nov 1995;127(5):718-22. [Medline].

  24. Nguyen JT, Yan AC, James WD. Large solitary cafe au lait spots: a report of 5 cases and review of the literature. Cutis. May 2004;73(5):311-4, 316. [Medline].

  25. Orion E, Matz H, Wolf D, Wolf R. Cafe au lait has hue of its own. Dermatology Online Journal. 2003;9:8. [Medline].

  26. Pinson S, Wolkenstein P. Neurofibromatosis type 1 or Von Recklinghausen's disease. La Revue de Medecine Interne. 2005;26:196-215. [Medline].

  27. Ruggieri M, Huson SM. The neurofibromatoses. An overview. Ital J Neurol Sci. Apr 1999;20(2):89-108. [Medline].

  28. Thami GP, Garg G. Cafe-au-lait macules: are these markers of neoplasia of genetic origin?. Dermatology. 2004;208:234-5. [Medline].

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Further Reading

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Keywords

café au lait spots, cafe au lait spots, pigmented macules, axillary freckling, neurofibromatosis, neurofibromatosis type 1, NF1, neurofibromatosis type 2, NF2, Von Recklinghausen disease, peripheral neurofibromatosis, NF2, McCune-Albright syndrome, tuberous sclerosis, Fanconi anemia, optic glioma, Lisch nodules, pseudarthroses, congenital glaucoma, sphenoid wing dysplasia, precocious puberty, aplastic anemia, mental retardation, ash leaf spots, angiofibromas

hemangiomas, cardiac rhabdomyomas, shagreen patches, Russell-Silver syndrome, Silver-Russell syndrome, ataxia telangiectasia, Bloom syndrome, Gaucher disease, Gaucher syndrome, Chiak-Higashi syndrome, Hunter syndrome, Maffucci syndrome, Watson syndrome, Crowe sign, scoliosis, hypoplastic bowing of the legs, osseous lesions in the ribs, pseudoarthritis of the tibia, spina bifida, scalloping of the vertebral body, neurofibromas, lipomata, angiomata, ptosis, pheochromocytoma

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Contributor Information and Disclosures

Author

William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology
Disclosure: elsevier Royalty Other; american college of physicians Honoraria Other

Coauthor(s)

Raj D Sheth, MD, Professor of Neurology, Mayo College of Medicine; Chief, Division of Pediatric Neurology, Nemours Children's Clinic
Raj D Sheth, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, American Neurological Association, and Child Neurology Society
Disclosure: Nothing to disclose.

Nazanin Saedi, MD, Resident Physician, Department of Dermatology, University of California, Irvine, School of Medicine
Nazanin Saedi, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Medical Editor

Kevin P Connelly, DO, Clinical Assistant Professor, Department of Pediatrics, Division of General Pediatrics and Emergency Care, Virginia Commonwealth University; Medical Director, Paws for Health Pet Visitation Program of the Richmond SPCA; Pediatric Emergency Physician, Emergency Consultants Inc, Chippenham Medical Center
Kevin P Connelly, DO is a member of the following medical societies: American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

CME Editor

Merrily P M Poth, MD, Professor, Department of Pediatrics and Neuroscience, Uniformed Services University of the Health Sciences
Merrily P M Poth, MD is a member of the following medical societies: American Academy of Pediatrics, Endocrine Society, and Lawson-Wilkins Pediatric Endocrine Society
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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