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Incontinentia pigment - Coggle Diagram
Incontinentia pigment
Definition
ia a rare X-ray linked dominant genetic disorder that affects the skin, hair, teeth, nails and central nervous system.
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pathophysiology
cells expressing the mutated X chromosomes selectively eliminate around the time of birth; therefore, females with incontinentia pigmenti have an extremely skewed X-inactiveivation pattern
in female continential pigmenti patients, iyonization resultsin functional mosaicism of x-linked genes, which is manifested by the blaschkoid distribution of cutaneous lesions.
normal X chromosomes are active in unaffected skin, and mutated X chromosomes are active in skin affected with incontinentia pigmenti.
involvement of the skin, hair, teeth, and nails is seen in conjuction with neurologic and ophthalmologic anomalities.
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nursing management
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temporary relief may be obtained from a cold compress or directing airflow from a fan on to wet skin.
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References
Ardelean D, Pope E. Incontinentia pigmenti in boys: a series and review of the literature. Pediatr Dermatol. 2006 Nov-Dec;23(6):523-7
Bruckner AL. Incontinentia pigmenti: a window to the role of NF-kappaB function. Semin Cutan Med Surg. 2004 Jun;23(2):116-24
