Medscape is available in 5 Language Editions – Choose your Edition here.


Pediatric Hypomelanosis of Ito Treatment & Management

  • Author: Camila K Janniger, MD; Chief Editor: Dirk M Elston, MD  more...
Updated: Jun 24, 2016

Medical Care

No specific treatment is available for hypomelanosis of Ito (HI).

Treat seizures depending on the specific seizure type and epileptic syndrome (see Status Epilepticus, Complex Partial Seizures).

Partial seizures may respond to the usual anticonvulsant medications, such as carbamazepine, phenytoin, lamotrigine, gabapentin, and topiramate.

Infantile spasms should be treated accordingly with adrenocorticotropic hormone (ACTH), vigabatrin, valproic acid, or topiramate.

Approximately 30% of patients with seizures do not respond to anticonvulsant medications; therefore, these patients may need an appropriate evaluation to verify if they are good candidates for resective epilepsy surgery, ketogenic diet, or vagal nerve stimulation. In these patients, perform a prolonged video-EEG to document the zone of ictal onset.

Always offer the patient and parents genetic consultation.

Traditional depigmenting agents (eg, hydroquinone, corticosteroids, kojic acid) may be used.[25] In addition, active compounds isolated from plants (eg, arbutin, aloesin, gentisic acid, flavonoids, hesperidin, licorice, niacinamide, yeast derivatives, polyphenols) may inhibit melanogenesis without melanocytotoxicity and merit further evaluation.


Surgical Care

Approximately 30% of patients with seizures do not respond to anticonvulsant medications. In these patients, conduct an appropriate evaluation to verify if they are good candidates for resective epilepsy surgery or vagal nerve stimulation.

The patient may have large lesions and require a hemispherectomy for the treatment of their refractory epilepsy; however, in other cases, the removal of a more focal lesion may stop the seizures.

Cataracts and retinal detachment may produce loss of vision and can be successfully treated with surgery.

In patients with craniofacial malformation, such as cleft lip and palate, repair is done in the same fashion as in patients without hypomelanosis of Ito.



Consultation with an orthopedic specialist is indicated for patients with skeletal abnormalities.

Suggest consultation with an ophthalmologist for patients with ophthalmologic abnormalities.

Consultation with a nephrologist is recommended for patients with renal abnormalities.

Suggest a consultation with an endocrinologist for patients with associated abnormalities.

Patients with hypomelanosis of Ito who were initially seen by a dermatologist may benefit from a consultation with a neurologist; conversely, patients initially referred to a neurologist may benefit from a consultation with a dermatologist and geneticist. Always offer the patient and parents the option of a consultation with a geneticist.

Refer patients who have seizures that are not completely controlled by anticonvulsant medications to an appropriate tertiary center with a comprehensive epilepsy program for proper evaluation (video-EEG, single-photon emission computed tomography [SPECT] or positron emission tomography [PET], high-resolution MRI).



No dietary restrictions are indicated. Patients with seizures who are unresponsive to anticonvulsant medication may benefit from a high-fat, low-carbohydrate diet (ie, the ketogenic diet).



No restriction in activity is recommended.



Hypomelanosis of Ito (HI) cannot be prevented, except in rare cases of familial hypomelanosis of Ito.

Because familial hypomelanosis of Ito is autosomal dominant, genetic counseling is indicated as a way to prevent new cases in the same family. Nonetheless, most cases are a de novo occurrence.

Contributor Information and Disclosures

Camila K Janniger, MD Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, Rutgers New Jersey Medical School

Camila K Janniger, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.


Marcio Sotero de Menezes, MD Clinical Associate Professor, Department of Neurology, Division of Pediatric Neurology, Seattle Children's Hospital, University of Washington School of Medicine; Director, Pediatric Neuroscience Center and Genetic Epilepsy Clinic, Swedish Neuroscience Institute

Marcio Sotero de Menezes, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society

Disclosure: Received salary from Novartis for speaking and teaching; Received salary from Cyberonics for speaking and teaching; Received salary from Athena diagnostics for speaking and teaching.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Kevin P Connelly, DO Clinical Assistant Professor, Department of Pediatrics, Division of General Pediatrics and Emergency Care, Virginia Commonwealth University School of Medicine; 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, American Osteopathic Association

Disclosure: Nothing to disclose.

  1. Ito M. A singular case of naevus depigmentosus systematicus bilateralis. Jpn J Dermatol. 1951. 61:31-2.

  2. Hamada T, Saito T, Sugai T, Morita Y. "Incontinentia pigmenti achromians (Ito)". Arch Dermatol. 1967 Dec. 96(6):673-6. [Medline].

  3. Pascual-Castroviejo I, Lopez-Rodriguez L, de la Cruz Medina M, Salamanca-Maesso C, Roche Herrero C. Hypomelanosis of Ito. Neurological complications in 34 cases. Can J Neurol Sci. 1988 May. 15(2):124-9. [Medline].

  4. Ruiz-Maldonado R, Toussaint S, Tamayo L, Laterza A, del Castillo V. Hypomelanosis of Ito: diagnostic criteria and report of 41 cases. Pediatr Dermatol. 1992 Mar. 9(1):1-10. [Medline].

  5. Sybert VP. Hypomelanosis of Ito: a description, not a diagnosis. J Invest Dermatol. 1994 Nov. 103(5 Suppl):141S-143S. [Medline].

  6. Ponti G, Pellacani G, Tomasi A, Percesepe A, Guarneri C, Guerra A, et al. Hypomelanosis of Ito with a trisomy 2 mosaicism: a case report. J Med Case Rep. 2014 Oct 9. 8(1):333. [Medline].

  7. Palungwachira P, Palungwachira P. Incontinentia pigmenti achromians of Ito: an ultrastructural study. J Med Assoc Thai. 2006 Feb. 89(2):253-7. [Medline].

  8. Parisi L, Di Filippo T, Roccella M. Hypomelanosis of Ito: neurological and psychiatric pictures in developmental age. Minerva Pediatr. 2012 Feb. 64(1):65-70. [Medline].

  9. Okanari K, Miyahara H, Itoh M, Takahashi A, Aizaki K, Nakagawa E, et al. Hemimegalencephaly in a Patient With Coexisting Trisomy 21 and Hypomelanosis of Ito. J Child Neurol. 2012 Dec 23. [Medline].

  10. Degerliyurt A, Kantar A, Ceylaner S, Aysun S. Hypomelanosis of Ito and Sturge-Weber Syndrome Without Facial Nevus: An Association or a New Syndrome?. Pediatr Neurol. 2009 May. 40(5):395-397. [Medline].

  11. Inoue M, Fukuda M, Ishii E, Sayama K. Linear Leukoplakia and Central Nervous System Lesions: A Clinical Clue to the Diagnosis of Hypomelanosis of Ito. J Pediatr. 2015 Sep. 167 (3):771-771.e1. [Medline].

  12. Souza PV, Pinto WB, Calente FG, Burlin S, Pedroso JL, Oliveira AS, et al. Hypomelanosis of Ito presenting with adult-onset dementia and marked enlarged Virchow-Robin spaces. Arq Neuropsiquiatr. 2015 Apr. 73 (4):366-8. [Medline].

  13. Glover MT, Brett EM, Atherton DJ. Hypomelanosis of Ito: spectrum of the disease. J Pediatr. 1989 Jul. 115(1):75-80. [Medline].

  14. Pascual-Castroviejo I, Roche C, Martinez-Bermejo A, et al. Hypomelanosis of ITO. A study of 76 infantile cases. Brain Dev. 1998 Jan. 20(1):36-43. [Medline].

  15. Park JM, Kim HJ, Kim T, Chae HW, Kim DH, Lee MG. Sexual precocity in hypomelanosis of Ito: mosaicism-associated case report and literature review. Int J Dermatol. 2011 Feb. 50(2):168-74. [Medline].

  16. Pavone V, Signorelli SS, Praticò AD, Corsello G, Savasta S, Falsaperla R, et al. Total Hemi-overgrowth in Pigmentary Mosaicism of the (Hypomelanosis of) Ito Type: Eight Case Reports. Medicine (Baltimore). 2016 Mar. 95 (10):e2705. [Medline].

  17. Gatter N, Hoppe B, Nutzenadel F, Waldherr R, Querfeld U. A cutaneous disease with multisystem involvement: hypomelanosis of Ito may be associated with proteinuria, focal segmental glomerulosclerosis and end-stage renal disease. Nephrol Dial Transplant. 2007 Mar 8. [Medline].

  18. Tragardh M, Thomsen CR, Thorninger R, Moller-Madsen B. Hypomelanosis of Ito presenting with pediatric orthopedic issues: a case report. J Med Case Rep. 2014 May 19. 8:156. [Medline]. [Full Text].

  19. Jozwiak S, Schwartz RA, Janniger CK, Bielicka-Cymerman J. Usefulness of diagnostic criteria of tuberous sclerosis complex in pediatric patients. J Child Neurol. 2000 Oct. 15(10):652-9. [Medline].

  20. Sybert VP, Pagon RA, Donlan M, Bradley CM. Pigmentary abnormalities and mosaicism for chromosomal aberration: association with clinical features similar to hypomelanosis of Ito. J Pediatr. 1990 Apr. 116(4):581-6. [Medline].

  21. Rosman NP. Incontinentia Pigmenti. Gomez MR, ed. Neurocutaneous Diseases: A Practical Approach. Boston: Butterworths; 1987. 294-300.

  22. Huggins RH, Schwartz RA, Janniger CK. Vitiligo. Acta Dermatovenerol Alp Panonica Adriat. 2005 Dec. 14(4):137-42, 144-5. [Medline].

  23. Huggins RH, Janusz CA, Schwartz RA. Vitiligo: a sign of systemic disease. Indian J Dermatol Venereol Leprol. 2006 Jan-Feb. 72(1):68-71. [Medline].

  24. Jadotte YT, Janniger CK. Pityriasis alba revisited: perspectives on an enigmatic disorder of childhood. Cutis. 2011 Feb. 87(2):66-72. [Medline].

  25. Zhu W, Gao J. The use of botanical extracts as topical skin-lightening agents for the improvement of skin pigmentation disorders. J Investig Dermatol Symp Proc. 2008 Apr. 13(1):20-4. [Medline].

  26. Urban J, Toruniowa B, Janniger CK, Czelej D, Schwartz RA. Incontinentia pigmenti (Bloch-Sulzberger syndrome): multisystem disease observed in two generations. Cutis. 1996 Nov. 58(5):329-36. [Medline].

Hypomelanosis of Ito on the torso.
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.