We're unable to sign you in at this time. Please try again in a few minutes.
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Commentary |

The Skin Is Not the Predominant Problem in Incontinentia Pigmenti

Morton F. Goldberg, MD
Arch Dermatol. 2004;140(6):748-750. doi:10.1001/archderm.140.6.748.
Text Size: A A A
Published online


Clinical consternation occurs when characteristic vesiculobullous eruptions of incontinentia pigmenti (IP) appear neonatally. These distressing lesions almost never lead to serious long-term morbidity, although they often result in time-consuming, expensive, and typically fruitless diagnostic evaluations in an attempt to diagnose other diseases.

Blindness and psychomotor retardation, on the other hand, constitute the most serious acute and chronic complications of this disease124 (Figure 1). When they occur, they are tragedies for the patients as well as for their families. Ideally, therefore, these complications should be sought assiduously and, if possible, treated quickly, as soon as the diagnosis of IP is established. Because babies obviously cannot complain of reduced vision or of impaired cerebral function, a high index of suspicion by attending clinicians is warranted. Urgent consultations with ophthalmic and neurologic subspecialists (specifically, retinologists and pediatric neurologists) are often needed, both for diagnosis and treatment. Fortunately, not all patients have blinding or retarding symptoms owing to the variable clinical expression of this disease.

Figures in this Article

Sign in

Purchase Options

• Buy this article
• Subscribe to the journal
• Rent this article ?

First Page Preview

View Large
First page PDF preview


Place holder to copy figure label and caption

A, Infant with incontinentia pigmenti, showing marked pallor of optic nerve head (optic atrophy). There was profound and permanent loss of vision. B, An infant aged 12 days with incontinentia pigmenti and occlusion of central retinal artery, demonstrating macular cherry-red spot (black dot), cotton-wool ischemic infarcts (arrowheads), numerous occluded retinal blood vessels (solid arrow), and zones of nonperfusion (open arrow). Vision was permanently reduced to perception of gross movements. C, An infant aged 20 months with incontinentia pigmenti and extensive zones of retinal nonperfusion (black dots), hard exudates, and a large arteriovenous shunt (arrowhead). There was severe and permanent loss of vision. D, A child aged 27 months with incontinentia pigmenti and inoperable tractional retinal detachment of the macula. There was profound and permanent loss of vision.

Graphic Jump Location




Also Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
Please click the checkbox indicating that you have read the full article in order to submit your answers.
Your answers have been saved for later.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.


Some tools below are only available to our subscribers or users with an online account.

20 Citations

Sign in

Purchase Options

• Buy this article
• Subscribe to the journal
• Rent this article ?

Related Content

Customize your page view by dragging & repositioning the boxes below.

Articles Related By Topic
Related Collections
PubMed Articles