Warts-immunodeficiency-lymphedema-anogenital dysplasia syndrome
ORPHA:568056· ICD-10 Q82.0
Definition
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.
- Prevalence
- <1 / 1 000 000
- Age of onset
- Infancy, Neonatal