Warts-immunodeficiency-lymphedema-anogenital dysplasia syndrome


Engelsk navn: Warts-immunodeficiency-lymphedema-anogenital dysplasia syndrome
Engelske synonym: Disseminated warts-impaired cell-mediated immunity-primary lymphedema-anogenital dysplasia syndrome,WILD syndrome


Definisjon

A rare primary lymphedema characterized by extensive, multisegmental lymphedema, associated with persistent, widespread infections with various genital high- and low-risk human papillomaviruses, resulting in multifocal anogenital dysplasia. Laboratory examination shows abnormalities in lymphocyte subsets, in particular CD4+ T-cells. Epidermal nevi and capillary malformations have also been reported.

Fra Orphanet

ORPHA: 568056
Klassifiseringsnivå: Disorder
ICD-10: Q82.0

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