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Iran J Kidney Dis. 2019 Jan;13(1):67-70.

Multicystic Dysplastic Kidney and Incontinentia Pigmenti: Coexistence of 2 Rare Diseases.

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Departments of Pediatric Nephrology, Mersin University Faculty of Medicine, Mersin, Turkey.


Multicystic dysplastic kidney is a congenital kidney malformation consisting of multiple cysts of various sizes without a normal kidney morphology. Incontinentia pigmenti is a rare X-linked dominant genodermatosis, which is usually lethal in males, that presents clinically in 4 stages. Here, we report a case of multicystic dysplastic kidney with ureterovesical junction obstruction and incontinentia pigmenti. Coexistence of these two rare diseases may be a coincidental phenomenon or an association between the two may exist.

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