Бородавки-імунодефіцит-лімфедема-аногенітальна дисплазія синдром
ORPHA:568056· ICD-10 Q82.0· Warts-immunodeficiency-lymphedema-anogenital dysplasia syndrome
Визначення(English summary)
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.
- Поширеність
- <1 / 1 000 000
- Вік початку
- Infancy, Neonatal