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Incontinentia Pigmenti
How is it treated?
In the early months of life, management aims to reduce the risk of infection of the blisters which can be treated by topical (locally applied) medications and discomfort relieving baths. However, such treatment does not shorten the period in which blisters occur.
Pigmented areas can be treated with skin camouflage (see "Visible Difference" in section, Procedures and Management), cosmetics or obscuring clothes. These areas fade over time. In the case of scalp alopecia, a dermatologist can prescribe wigs from a hospital medical appliance department or a prescription can be used for a reduction of the cost of a wig from a commercial retailer.
Numerous eye abnormalities in IP have been reported but the majority of them result from retinal vascular defects of the retinal blood vessels. These abnormalities, which can lead to retinal detachment, represent the major cause of severe visual defects in IP. The mechanisms causing the changes in blood vessels are not fully understood. The natural history of retinal disease in IP is also unknown and optimal treatment of the retinal problems is not clear at present. There are reports of successful treatment of proliferative retinopathy (overgrowth in the retinal blood vessels) with cryotherapy (freezing treatments) and laser.
An ocular examination should be performed as soon as possible after birth and IP patients should be followed carefully by an ophthalmologist. Parents may be advised of the possibility of retinal detachment and the need to have any apparent changes checked promptly. A study from Sweden of 30 patients with IP recommended eye screening for all newly diagnosed infants with IP and the female offspring of affected women. The following scheme was suggested: ocular examination as soon as possible after birth, monthly until three to four months, three monthly until one year of age, and then bi-annually until three years of age.
Our knowledge of the natural history of the retinal changes in IP is not complete and the duration of screening remains unclear and therefore ophthalmology review should perhaps be continued on an annual basis throughout childhood. An ocular assessment can be arranged by referral to the local ophthalmology department from a GP, clinical geneticist, paediatrician/neonatologist or dermatologist.
Continued evaluation by a dentist should take place and referral to a speech therapist for help with speech and feeding should be part of the management/treatment process.
How is it diagnosed?
| Inheritance patterns and prenatal diagnosis 
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