U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Warts-immunodeficiency-lymphedema-anogenital dysplasia syndrome

MedGen UID:
1799992
Concept ID:
C5568569
Disease or Syndrome
Synonyms: Disseminated warts, impaired cell-mediated immunity, primary lymphedema, anogenital dysplasia syndrome; Disseminated warts-impaired cell-mediated immunity-primary lymphedema-anogenital dysplasia syndrome; disseminated warts-impaired cell-mediated immunity-primary lymphedema-anogenital dysplasia syndrome; Warts, immunodeficiency, lymphedema, anogenital dysplasia syndrome; warts-immunodeficiency-lymphedema-anogenital dysplasia syndrome; WILD (warts, immunodeficiency, lymphedema, anogenital dysplasia) syndrome; WILD syndrome
SNOMED CT: WILD syndrome (1186725001); Warts, immunodeficiency, lymphedema, anogenital dysplasia syndrome (1186725001); WILD (warts, immunodeficiency, lymphedema, anogenital dysplasia) syndrome (1186725001); Disseminated warts, impaired cell-mediated immunity, primary lymphedema, anogenital dysplasia syndrome (1186725001)
 
Monarch Initiative: MONDO:0035473
Orphanet: ORPHA568056

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. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVWarts-immunodeficiency-lymphedema-anogenital dysplasia syndrome

Professional guidelines

PubMed

Garcia-Manero G
Am J Hematol 2023 Aug;98(8):1307-1325. Epub 2023 Jun 8 doi: 10.1002/ajh.26984. PMID: 37288607
Sekeres MA, Taylor J
JAMA 2022 Sep 6;328(9):872-880. doi: 10.1001/jama.2022.14578. PMID: 36066514
Patnaik MM, Tefferi A
Am J Hematol 2022 Mar 1;97(3):352-372. Epub 2022 Jan 27 doi: 10.1002/ajh.26455. PMID: 34985762

Recent clinical studies

Etiology

Berberian JG
Emerg Med Clin North Am 2022 Nov;40(4):651-662. Epub 2022 Oct 7 doi: 10.1016/j.emc.2022.06.005. PMID: 36396213
Wright JT, Fete M, Schneider H, Zinser M, Koster MI, Clarke AJ, Hadj-Rabia S, Tadini G, Pagnan N, Visinoni AF, Bergendal B, Abbott B, Fete T, Stanford C, Butcher C, D'Souza RN, Sybert VP, Morasso MI
Am J Med Genet A 2019 Mar;179(3):442-447. Epub 2019 Jan 31 doi: 10.1002/ajmg.a.61045. PMID: 30703280Free PMC Article
Torban E, Goodyer P
Annu Rev Med 2009;60:339-53. doi: 10.1146/annurev.med.60.052307.120752. PMID: 18976115
Kloeppel R, Rothe K, Hoermann D, Schmidt F, Bennek J, Kahn T
J Comput Assist Tomogr 2002 Mar-Apr;26(2):262-5. doi: 10.1097/00004728-200203000-00017. PMID: 11884784
Spranger JW
Postgrad Med J 1977 Aug;53(622):480-7. doi: 10.1136/pgmj.53.622.480. PMID: 335375Free PMC Article

Diagnosis

Berberian JG
Emerg Med Clin North Am 2022 Nov;40(4):651-662. Epub 2022 Oct 7 doi: 10.1016/j.emc.2022.06.005. PMID: 36396213
Sataite I, Cudlip S, Jayamohan J, Ganau M
Handb Clin Neurol 2021;181:51-64. doi: 10.1016/B978-0-12-820683-6.00005-1. PMID: 34238479
Vanlerberghe C, Boutry N, Petit F
Clin Genet 2018 Jul;94(1):43-53. Epub 2018 Apr 11 doi: 10.1111/cge.13209. PMID: 29322497
Agrawal A, Agrawal R, Singh R, Agrawal R, Agrawal S
Indian J Dent Res 2014 Mar-Apr;25(2):243-7. doi: 10.4103/0970-9290.135935. PMID: 24992861
Kim G, Shin H
Dermatol Online J 2004 Nov 30;10(3):23. PMID: 15748593

Therapy

Garcia-Manero G
Am J Hematol 2023 Aug;98(8):1307-1325. Epub 2023 Jun 8 doi: 10.1002/ajh.26984. PMID: 37288607
Lee P, Yim R, Yung Y, Chu HT, Yip PK, Gill H
Int J Mol Sci 2021 Sep 23;22(19) doi: 10.3390/ijms221910232. PMID: 34638574Free PMC Article
Miao L, Zhang Z, Ren Z, Li Y
Front Immunol 2021;12:663201. Epub 2021 Apr 28 doi: 10.3389/fimmu.2021.663201. PMID: 33995389Free PMC Article
Behnke J, Lemyre B, Czernik C, Zimmer KP, Ehrhardt H, Waitz M
Dtsch Arztebl Int 2019 Mar 8;116(11):177-183. doi: 10.3238/arztebl.2019.0177. PMID: 31014448Free PMC Article
Tanaka TN, Bejar R
Blood 2019 Mar 7;133(10):1086-1095. Epub 2019 Jan 22 doi: 10.1182/blood-2018-10-844670. PMID: 30670443

Prognosis

Wang X, Li Y, Shi T, Bont LJ, Chu HY, Zar HJ, Wahi-Singh B, Ma Y, Cong B, Sharland E, Riley RD, Deng J, Figueras-Aloy J, Heikkinen T, Jones MH, Liese JG, Markić J, Mejias A, Nunes MC, Resch B, Satav A, Yeo KT, Simões EAF, Nair H; Respiratory Virus Global Epidemiology Network; RESCEU investigators
Lancet 2024 Mar 30;403(10433):1241-1253. Epub 2024 Feb 14 doi: 10.1016/S0140-6736(24)00138-7. PMID: 38367641
Siddique AW, Ahmed Z, Haider A, Khalid H, Karim T
J Ayub Med Coll Abbottabad 2019 Apr-Jun;31(2):290-292. PMID: 31094135
Grignano E, Jachiet V, Fenaux P, Ades L, Fain O, Mekinian A
Ann Hematol 2018 Nov;97(11):2015-2023. Epub 2018 Aug 8 doi: 10.1007/s00277-018-3472-9. PMID: 30091023
Kulkarni ML, Navaz SR, Vani HN, Manjunath KS, Matani D
Indian J Pediatr 2006 Mar;73(3):245-7. doi: 10.1007/BF02825493. PMID: 16567923
Bhatia S, Holla RG
Indian Pediatr 2005 Apr;42(4):373-5. PMID: 15876600

Clinical prediction guides

Raimondi F, Migliaro F, Corsini I, Meneghin F, Dolce P, Pierri L, Perri A, Aversa S, Nobile S, Lama S, Varano S, Savoia M, Gatto S, Leonardi V, Capasso L, Carnielli VP, Mosca F, Dani C, Vento G, Lista G
Pediatrics 2021 Apr;147(4) Epub 2021 Mar 9 doi: 10.1542/peds.2020-030528. PMID: 33688032
Rudnicka L, Olszewska M, Waśkiel A, Rakowska A
Dermatol Clin 2018 Oct;36(4):421-430. Epub 2018 Aug 16 doi: 10.1016/j.det.2018.05.009. PMID: 30201151
Vanlerberghe C, Boutry N, Petit F
Clin Genet 2018 Jul;94(1):43-53. Epub 2018 Apr 11 doi: 10.1111/cge.13209. PMID: 29322497
Tweet MS, Gulati R, Williamson EE, Vrtiska TJ, Hayes SN
JACC Cardiovasc Imaging 2016 Apr;9(4):436-50. doi: 10.1016/j.jcmg.2016.01.009. PMID: 27056163
Currarino G, Sheffield E, Twickler D
Pediatr Radiol 1998 Jan;28(1):30-7. doi: 10.1007/s002470050287. PMID: 9426270

Recent systematic reviews

Wang X, Li Y, Shi T, Bont LJ, Chu HY, Zar HJ, Wahi-Singh B, Ma Y, Cong B, Sharland E, Riley RD, Deng J, Figueras-Aloy J, Heikkinen T, Jones MH, Liese JG, Markić J, Mejias A, Nunes MC, Resch B, Satav A, Yeo KT, Simões EAF, Nair H; Respiratory Virus Global Epidemiology Network; RESCEU investigators
Lancet 2024 Mar 30;403(10433):1241-1253. Epub 2024 Feb 14 doi: 10.1016/S0140-6736(24)00138-7. PMID: 38367641
Silveira RC, Panceri C, Munõz NP, Carvalho MB, Fraga AC, Procianoy RS
J Pediatr (Rio J) 2024 Jan-Feb;100(1):8-24. Epub 2023 Jun 21 doi: 10.1016/j.jped.2023.05.008. PMID: 37353207Free PMC Article
Cerezo-Cayuelas M, Pérez-Silva A, Serna-Muñoz C, Vicente A, Martínez-Beneyto Y, Cabello-Malagón I, Ortiz-Ruiz AJ
Orphanet J Rare Dis 2022 Oct 17;17(1):376. doi: 10.1186/s13023-022-02533-0. PMID: 36253866Free PMC Article
Ho JJ, Subramaniam P, Davis PG
Cochrane Database Syst Rev 2020 Oct 15;10(10):CD002271. doi: 10.1002/14651858.CD002271.pub3. PMID: 33058208Free PMC Article
van Sleuwen BE, Engelberts AC, Boere-Boonekamp MM, Kuis W, Schulpen TW, L'Hoir MP
Pediatrics 2007 Oct;120(4):e1097-106. doi: 10.1542/peds.2006-2083. PMID: 17908730

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...