eMedicine Specialties > Dermatology > Pediatric Diseases

Incontinentia Pigmenti: Follow-up

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

Follow-up

Further Outpatient Care

  • The presence of variable disease expression in an affected family makes monitoring for potential complications important. Regular follow-up with a neurologist, ophthalmologist, dentist, and dermatologist should be coordinated as needed.

Complications

  • Secondary bacterial infection can develop during the vesicular stage, but this is rare.
  • Seizures and mental retardation are common in patients with structural brain malformations or evidence of ischemic brain injury.
  • Ophthalmologic complications can lead to reduced visual acuity and blindness.

Prognosis

  • The prognosis of IP is generally good.
  • Morbidity and mortality are related to neurologic and ophthalmologic sequelae, including seizures, visual impairment, and mental retardation.
  • Patients with structural brain abnormalities and neonatal seizures are at greater risk for motor and intellectual impairment.

Patient Education

  • Inform parents that delayed eruption of both deciduous and permanent teeth is common.
  • Reassure parents that if no evidence of CNS involvement or seizures is seen in their infant, the neurodevelopmental prognosis is excellent.
  • Genetic counseling should be offered to the family.
  • Counsel parents on the expected course of cutaneous manifestations.

Miscellaneous

Medicolegal Pitfalls

  • Failure to make a correct diagnosis based on cutaneous findings and ocular, CNS, and dental abnormalities
  • Failure to perform skin biopsy of vesicular or verrucous lesions to confirm a diagnosis of IP
  • Failure to refer patients to neurologists and ophthalmologists for evaluation and treatment
  • Failure to provide appropriate genetic counseling

Special Concerns

  • Examine the mother and all female relatives of a child with IP for clinical features of IP. Carefully look for dental abnormalities and stage 4 cutaneous changes (eg, atrophic/hypopigmented lesions involving the lower legs).
 


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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