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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

Myelodysplastic syndromes: 2023 update on diagnosis, risk-stratification, and management.
Garcia-Manero G
Am J Hematol 2023 Aug;98(8):1307-1325. Epub 2023 Jun 8 doi: 10.1002/ajh.26984. PMID: 37288607
Diagnosis and Treatment of Myelodysplastic Syndromes: A Review.
Sekeres MA, Taylor J
JAMA 2022 Sep 6;328(9):872-880. doi: 10.1001/jama.2022.14578. PMID: 36066514
Chronic myelomonocytic leukemia: 2022 update on diagnosis, risk stratification, and management.
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

Ectodermal dysplasias: Classification and organization by phenotype, genotype and molecular pathway.
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
Short stature in genetic syndromes: Selected issues.
Łaczmańska I, Kuliczkowska-Płaksej J, Stembalska A
Adv Clin Exp Med 2018 Mar;27(3):409-414. doi: 10.17219/acem/67051. PMID: 29558022
Genetic short stature.
Grunauer M, Jorge AAL
Growth Horm IGF Res 2018 Feb;38:29-33. Epub 2017 Dec 6 doi: 10.1016/j.ghir.2017.12.003. PMID: 29249624
Proteus syndrome.
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
Metaphyseal chondrodysplasia.
Spranger JW
Postgrad Med J 1977 Aug;53(622):480-7. doi: 10.1136/pgmj.53.622.480. PMID: 335375Free PMC Article

Diagnosis

Hereditary Syndromes of Sudden Cardiac Death.
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
Septo-optic dysplasia.
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
Short stature in genetic syndromes: Selected issues.
Łaczmańska I, Kuliczkowska-Płaksej J, Stembalska A
Adv Clin Exp Med 2018 Mar;27(3):409-414. doi: 10.17219/acem/67051. PMID: 29558022
Genetics of patella hypoplasia/agenesis.
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
Metaphyseal chondrodysplasia.
Spranger JW
Postgrad Med J 1977 Aug;53(622):480-7. doi: 10.1136/pgmj.53.622.480. PMID: 335375Free PMC Article

Therapy

Myelodysplastic syndromes: 2023 update on diagnosis, risk-stratification, and management.
Garcia-Manero G
Am J Hematol 2023 Aug;98(8):1307-1325. Epub 2023 Jun 8 doi: 10.1002/ajh.26984. PMID: 37288607
Molecular Targeted Therapy and Immunotherapy for Myelodysplastic Syndrome.
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
Novel Surfactant Administration Techniques: Will They Change Outcome?
Barkhuff WD, Soll RF
Neonatology 2019;115(4):411-422. Epub 2019 Apr 11 doi: 10.1159/000497328. PMID: 30974437
Elosulfase alfa.
Haddley K
Drugs Today (Barc) 2014 Jul;50(7):475-83. doi: 10.1358/dot.2014.50.7.2177904. PMID: 25101330
The preleukemic syndrome (hemopoietic dysplasia).
Linman JW, Bagby GC Jr
Cancer 1978 Aug;42(2 Suppl):854-64. doi: 10.1002/1097-0142(197808)42:2+<854::aid-cncr2820420707>3.0.co;2-w. PMID: 356956

Prognosis

Yunis-Varon Syndrome.
Siddique AW, Ahmed Z, Haider A, Khalid H, Karim T
J Ayub Med Coll Abbottabad 2019 Apr-Jun;31(2):290-292. PMID: 31094135
Autoimmune manifestations associated with myelodysplastic syndromes.
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
Glenoid Dysplasia.
Abboud JA, Bateman DK, Barlow J
J Am Acad Orthop Surg 2016 May;24(5):327-36. doi: 10.5435/JAAOS-D-15-00032. PMID: 27055054
Yunis-Varon syndrome.
Bhatia S, Holla RG
Indian Pediatr 2005 Apr;42(4):373-5. PMID: 15876600
The preleukemic syndrome (hemopoietic dysplasia).
Linman JW, Bagby GC Jr
Cancer 1978 Aug;42(2 Suppl):854-64. doi: 10.1002/1097-0142(197808)42:2+<854::aid-cncr2820420707>3.0.co;2-w. PMID: 356956

Clinical prediction guides

Inborn errors of immunity associated with characteristic phenotypes.
Bardou MLD, Henriques MT, Grumach AS
J Pediatr (Rio J) 2021 Mar-Apr;97 Suppl 1(Suppl 1):S75-S83. Epub 2020 Dec 19 doi: 10.1016/j.jped.2020.10.015. PMID: 33347837Free PMC Article
Trichoscopy in Hair Shaft Disorders.
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
Genetics of patella hypoplasia/agenesis.
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
Multimodality Imaging for Spontaneous Coronary Artery Dissection in Women.
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
Congenital glenoid dysplasia.
Currarino G, Sheffield E, Twickler D
Pediatr Radiol 1998 Jan;28(1):30-7. doi: 10.1007/s002470050287. PMID: 9426270

Recent systematic reviews

Surfactant delivery via thin catheter in preterm infants: A systematic review and meta-analysis.
Yeung TY, Zhou Q, Kanmaz Kutman HG, Pandita A, Philippopoulos E, Jasani B
PLoS One 2023;18(4):e0284792. Epub 2023 Apr 26 doi: 10.1371/journal.pone.0284792. PMID: 37099568Free PMC Article
Orthodontic and dentofacial orthopedic treatments in patients with ectodermal dysplasia: a systematic review.
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
Selecting End Points for Disease-Modification Trials in Inflammatory Bowel Disease: the SPIRIT Consensus From the IOIBD.
Le Berre C, Peyrin-Biroulet L; SPIRIT-IOIBD study group
Gastroenterology 2021 Apr;160(5):1452-1460.e21. Epub 2021 Jan 6 doi: 10.1053/j.gastro.2020.10.065. PMID: 33421515
A systematic review of randomized controlled trials for the prevention of bronchopulmonary dysplasia in infants.
Beam KS, Aliaga S, Ahlfeld SK, Cohen-Wolkowiez M, Smith PB, Laughon MM
J Perinatol 2014 Sep;34(9):705-10. Epub 2014 Jul 10 doi: 10.1038/jp.2014.126. PMID: 25010224Free PMC Article
Swaddling: a systematic review.
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

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