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Vesicular rash in a newborn girl

The Journal of Family Practice. 2015 November;64(11):729-731
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The appearance and pattern of this child’s rash led us to suspect a rare diagnosis. The findings of a cutaneous biopsy confirmed it.

No specific treatment for IP rash; carefully evaluate other symptoms

Because the skin manifestations of IP usually resolve spontaneously, no specific treatment is required. The prognosis of a patient with IP depends on the presence and severity of extracutaneous manifestations of the condition.

A referral to opthalmology is crucial; untreated retinal disorders have caused blindness in 7% to 23% of incontinentia pigmenti patients.

A patient with IP who has abnormal findings on a neurologic examination, vascular retinopathy, or both should undergo routine neurodevelopmental assessment and neuroimaging.5 Treatment of neurologic complications is symptomatic. Speech therapy may play an important role in management of these patients because dental and neurologic abnormalities may result in dysfunction of chewing, swallowing, speech, language, and hearing.

Ophthalmology referral is also crucial because untreated retinal disorders have been reported to cause blindness in 7% to 23% of patients with IP.3 Screening is recommended at birth (or at diagnosis), and the child is followed regularly during the first year of life.5

Morbidity and mortality primarily result from neurologic manifestations (most commonly seizures and mental retardation) and ophthalmologic complications, which can result in vision loss.

Our patient presented with a rash in the vesicular stage of IP that was transitioning to the verrucous stage. She was seen by Ophthalmology at 4 weeks of age and there were no concerning findings at that time. She will continue to follow up with Ophthalmology, Dermatology, and her primary care physician.

CORRESPONDENCE
John C. Browning, MD, Chief of Dermatology, Children’s Hospital of San Antonio, 333 N. Santa Rosa, San Antonio, TX, 78207; john.browning@bcm.edu