Etiket - Incontinentia Pigmenti

Incontinentia Pigmenti

Incontinentia Pigmenti (IP) is a genetic disorder that affects the skin, hair, teeth, and nails. It is also known as Bloch-Sulzberger disease[1]:548, Bloch Siemens syndrome, melanoblastosis cutis and naevus pigmentosus systematicus. The skin lesions evolve through characteristic stages: *blistering (from birth to about four months of age), *a wart-like rash (for several months), *swirling macular hyperpigmentation (from about six months of age into adulthood), followed by *linear hypopigmentation. Alopecia, hypodontia, abnormal tooth shape, and dystrophic nails are observed. Some patients have retinal vascular abnormalities predisposing [...]

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