Mais conteúdo relacionado Semelhante a INCONTINENTIA PIGMENTI (20) Mais de Ibrahim Farag (19) INCONTINENTIA PIGMENTI6. Rare, X-linked, dominantly inherited disorder of
skin pigmentation that often is associated with
CNS, ocular and dental abnormalities
30. From birth to 2 weeks
with linear vesicles, pustules & bullae with
erythema whorled along the lines of Blaschko
32. between ages 2 and 8 weeks
with warty, keratotic linear papules and plaques
34. between ages 3–6 months
with macular hyperpigmentation in a swirled
pattern along the lines of Blaschko. These
changes often involve the nipples, axilla & groin
50. X-LINKED DOMINANT SYNDROMES
“BIG Child” with an X-linked dominant syndrome
B = Bazex syndrome
I = Incontinentia pigmenti
G = Goltz syndrome (focal dermal hypoplasia)
Child = CHILD syndrome
63. CBC frequently shows eosinophilia
Skin biopsy
CT scan or MRI of brain may show abnormalities
MOLECULAR GENETICS: NEMO gene
67. In stage 1
The main goal is to prevent secondary bacterial
infection of skin lesions & to monitor closely the
development of related problems
70. As IP is an X-linked dominant disease, GENETIC
COUNSELING regarding the risk of affected
offspring is very important