eMedicine Specialties > Dermatology > Pediatric Diseases

Incontinentia Pigmenti: Treatment & Medication

Author: Kara N Shah, MD, PhD, Assistant Professor, Department of Pediatrics, Section of Dermatology, Children's Hospital of Philadelphia
Contributor Information and Disclosures

Updated: Feb 18, 2008

Treatment

Medical Care

  • Treatment is not usually required for the cutaneous lesions. The vesicles of the inflammatory stage should be left intact, and the skin should be monitored for the development of secondary bacterial infections. Emollients and topical antibiotics may be used as needed.
  • Oral hygiene and regular dental care is necessary, and dental restoration may be indicated.
  • Seizures should be treated with anticonvulsants.
  • Routine neurodevelopmental assessments should be made, with referral to occupational and physical therapists as warranted.
  • Frequent ophthalmologic evaluations are required, especially during the first year of life, in order to diagnose and treat potential ophthalmologic complications.

Surgical Care

  • Abnormal retinal fibrovascular proliferation can be treated with xenon laser photocoagulation or cryosurgery.20
  • Retinal detachments may be treated using vitreoretinal surgery.

Consultations

  • Dermatologists may help in the initial evaluation and can perform a skin biopsy to aid in diagnosis.
  • Ophthalmologists can perform regular ophthalmologic examinations and manage any ophthalmologic sequelae.
  • Neurologists can perform a complete initial neurologic examination (including imaging studies and EEG), initiate and monitor anticonvulsant therapy in patients with seizures, and facilitate neurodevelopmental evaluation and intervention.
  • General dentists can provide regular dental care, screening for dental complications, and restorative dental care.
  • Geneticists can provide appropriate genetic counseling and genetic testing for the patient and his or her family.

Medication

In patients with seizures, anticonvulsant drugs are used. These agents have central and peripheral anticholinergic effects and sedative effects. They also block the active reuptake of norepinephrine and serotonin. A variety of anticonvulsants are available, and they should be selected at the discretion of the neurologist.

More on Incontinentia Pigmenti

Overview: Incontinentia Pigmenti
Differential Diagnoses & Workup: Incontinentia Pigmenti
Treatment & Medication: Incontinentia Pigmenti
Follow-up: Incontinentia Pigmenti
Multimedia: Incontinentia Pigmenti
References
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References

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Further Reading

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Keywords

IP, Bloch-Sulzberger syndrome, ectodermal dysplasia, neurocutaneous syndrome

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Contributor Information and Disclosures

Author

Kara N Shah, MD, PhD, Assistant Professor, Department of Pediatrics, Section of Dermatology, Children's Hospital of Philadelphia
Kara N Shah, MD, PhD is a member of the following medical societies: American Academy of Dermatology, American Academy of Pediatrics, and Society for Pediatric Dermatology
Disclosure: Nothing to disclose.

Medical Editor

Bernice R Krafchik, MBChB, FRCPC, Professor Emeritus, Department of Pediatrics, Section of Dermatology, University of Toronto
Bernice R Krafchik, MBChB, FRCPC is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, Canadian Medical Association, College of Physicians and Surgeons of Ontario, Royal College of Physicians and Surgeons of Canada, and Society for Pediatric Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Director Dermatology Residency Training Program, Scott and White Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: 3M Pharmaceutical Grant/research funds Other; Graceway Pharmaceuticals Grant/research funds Other

Managing Editor

Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of 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

Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital
Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons
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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