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Tufted angioma of skin

MedGen UID:
83402
Concept ID:
C0346073
Neoplastic Process
Synonym: Tufted angioma
SNOMED CT: Tufted angioma (705155008); Tufted angioma of skin (254786000)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
Concept ID:
C0600599
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
HPO: HP:0012329
Monarch Initiative: MONDO:0011927
OMIM®: 607859
Orphanet: ORPHA1063

Definition

Tufted angioma is a rare benign vascular lesion that predominantly affects children under 5 years of age but may occur in adulthood. Some cases of tufted angioma have been reported in the mother during pregnancy, whereas in other cases the tufted angioma may be congenital. The lesions occur predominantly on the neck, shoulders, and trunk and appear histologically in a 'cannonball' distribution of rounded nodules or tufts of capillary-sized vessels in the dermis, with lymphatic vessels present at the periphery. The natural history is slow progressive growth, after which it tends to remain stable in size. Regression has been reported in some cases. Tufted angioma should be distinguished from kaposiform hemangioendothelioma (KHE). Multiple tufted angioma and KHE may be associated with Kasabach-Merritt syndrome (141000), which is characterized by severe thrombocytopenia and consumption of coagulation factors (summary by Tille et al., 2003). [from OMIM]

Clinical features

From HPO
Tufted angioma of skin
MedGen UID:
83402
Concept ID:
C0346073
Neoplastic Process
Tufted angioma is a rare benign vascular lesion that predominantly affects children under 5 years of age but may occur in adulthood. Some cases of tufted angioma have been reported in the mother during pregnancy, whereas in other cases the tufted angioma may be congenital. The lesions occur predominantly on the neck, shoulders, and trunk and appear histologically in a 'cannonball' distribution of rounded nodules or tufts of capillary-sized vessels in the dermis, with lymphatic vessels present at the periphery. The natural history is slow progressive growth, after which it tends to remain stable in size. Regression has been reported in some cases. Tufted angioma should be distinguished from kaposiform hemangioendothelioma (KHE). Multiple tufted angioma and KHE may be associated with Kasabach-Merritt syndrome (141000), which is characterized by severe thrombocytopenia and consumption of coagulation factors (summary by Tille et al., 2003).
Vascular skin abnormality
MedGen UID:
102473
Concept ID:
C0162819
Disease or Syndrome
A disorder of the vasculature of the skin.
Abnormality of skin pigmentation
MedGen UID:
224697
Concept ID:
C1260926
Finding
An abnormality of the pigmentation of the skin.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTufted angioma of skin
Follow this link to review classifications for Tufted angioma of skin in Orphanet.

Conditions with this feature

Tufted angioma of skin
MedGen UID:
83402
Concept ID:
C0346073
Neoplastic Process
Tufted angioma is a rare benign vascular lesion that predominantly affects children under 5 years of age but may occur in adulthood. Some cases of tufted angioma have been reported in the mother during pregnancy, whereas in other cases the tufted angioma may be congenital. The lesions occur predominantly on the neck, shoulders, and trunk and appear histologically in a 'cannonball' distribution of rounded nodules or tufts of capillary-sized vessels in the dermis, with lymphatic vessels present at the periphery. The natural history is slow progressive growth, after which it tends to remain stable in size. Regression has been reported in some cases. Tufted angioma should be distinguished from kaposiform hemangioendothelioma (KHE). Multiple tufted angioma and KHE may be associated with Kasabach-Merritt syndrome (141000), which is characterized by severe thrombocytopenia and consumption of coagulation factors (summary by Tille et al., 2003).

Professional guidelines

PubMed

Borst AJ, Eng W, Griffin M, Ricci KW, Engel E, Adams DM, Dayneka J, Cohen-Cutler SJ, Andreoli SM, Wu MD, Wheeler AP, Heym KM, Crary SE, Nakano TA, Schulte RR, Setty BA, McLean TW, Pahl KS, Intzes S, Pateva I, Teitelbaum M, Zong Z, Li Y, Jeng MR
Pediatr Blood Cancer 2024 Mar;71(3):e30779. Epub 2023 Dec 10 doi: 10.1002/pbc.30779. PMID: 38073018
Goodarzi A, Mozafarpoor S, Dodangeh M, Seirafianpour F, Shahverdi MH
Dermatol Ther 2021 Mar;34(2):e14847. Epub 2021 Feb 13 doi: 10.1111/dth.14847. PMID: 33538017
Liu XH, Li JY, Qu XH, Yan WL, Zhang L, Yang C, Zheng JW
Int J Cancer 2016 Oct 1;139(7):1658-66. Epub 2016 Jun 28 doi: 10.1002/ijc.30216. PMID: 27252149

Recent clinical studies

Etiology

Goodarzi A, Mozafarpoor S, Dodangeh M, Seirafianpour F, Shahverdi MH
Dermatol Ther 2021 Mar;34(2):e14847. Epub 2021 Feb 13 doi: 10.1111/dth.14847. PMID: 33538017
Su X, Liu Y, Liu Y, Ma C
Indian J Dermatol Venereol Leprol 2020 Jan-Feb;86(1):24-32. doi: 10.4103/ijdvl.IJDVL_777_18. PMID: 31397400
Gong X, Ying H, Zhang Z, Wang L, Li J, Ding A, Zhou L, Lin X, Xiong P
J Dermatol 2019 Oct;46(10):835-842. Epub 2019 Aug 2 doi: 10.1111/1346-8138.15025. PMID: 31373042
Feito-Rodríguez M, Sánchez-Orta A, De Lucas R, López-Gutiérrez JC, Ruiz-Bravo E, Baselga E, Victoria AM, Hernández-Martín A, Campos-Domínguez M, Berenguer Fröhner B, Garnacho-Saucedo G, Vera Casaño A, Vicente Villa A, Bernabeu-Wittel J, Kutzner H, Requena L
Pediatr Dermatol 2018 Nov;35(6):808-816. Epub 2018 Oct 15 doi: 10.1111/pde.13683. PMID: 30318642
Croteau SE, Gupta D
Semin Cutan Med Surg 2016 Sep;35(3):147-52. doi: 10.12788/j.sder.2016.048. PMID: 27607323

Diagnosis

Papke DJ Jr, Hornick JL
Virchows Arch 2020 Jan;476(1):17-28. Epub 2019 Aug 28 doi: 10.1007/s00428-019-02651-4. PMID: 31463731
Häußler SM, Uecker FC, Knopke S, Roßner F, Olze H, Böttcher A
HNO 2018 Jan;66(Suppl 1):1-6. doi: 10.1007/s00106-017-0375-3. PMID: 28667388
Marušić Z, Billings SD
Surg Pathol Clin 2017 Jun;10(2):345-366. Epub 2017 Mar 31 doi: 10.1016/j.path.2017.01.006. PMID: 28477885
Croteau SE, Gupta D
Semin Cutan Med Surg 2016 Sep;35(3):147-52. doi: 10.12788/j.sder.2016.048. PMID: 27607323
Liu XH, Li JY, Qu XH, Yan WL, Zhang L, Yang C, Zheng JW
Int J Cancer 2016 Oct 1;139(7):1658-66. Epub 2016 Jun 28 doi: 10.1002/ijc.30216. PMID: 27252149

Therapy

Goodarzi A, Mozafarpoor S, Dodangeh M, Seirafianpour F, Shahverdi MH
Dermatol Ther 2021 Mar;34(2):e14847. Epub 2021 Feb 13 doi: 10.1111/dth.14847. PMID: 33538017
Gong X, Ying H, Zhang Z, Wang L, Li J, Ding A, Zhou L, Lin X, Xiong P
J Dermatol 2019 Oct;46(10):835-842. Epub 2019 Aug 2 doi: 10.1111/1346-8138.15025. PMID: 31373042
Wang H, Guo X, Duan Y, Zheng B, Gao Y
Pediatr Dermatol 2018 Sep;35(5):635-638. Epub 2018 Jul 12 doi: 10.1111/pde.13600. PMID: 29999213
Croteau SE, Gupta D
Semin Cutan Med Surg 2016 Sep;35(3):147-52. doi: 10.12788/j.sder.2016.048. PMID: 27607323
Liu XH, Li JY, Qu XH, Yan WL, Zhang L, Yang C, Zheng JW
Int J Cancer 2016 Oct 1;139(7):1658-66. Epub 2016 Jun 28 doi: 10.1002/ijc.30216. PMID: 27252149

Prognosis

Feito-Rodríguez M, Sánchez-Orta A, De Lucas R, López-Gutiérrez JC, Ruiz-Bravo E, Baselga E, Victoria AM, Hernández-Martín A, Campos-Domínguez M, Berenguer Fröhner B, Garnacho-Saucedo G, Vera Casaño A, Vicente Villa A, Bernabeu-Wittel J, Kutzner H, Requena L
Pediatr Dermatol 2018 Nov;35(6):808-816. Epub 2018 Oct 15 doi: 10.1111/pde.13683. PMID: 30318642
Putra J, Gupta A
Pathology 2017 Jun;49(4):356-362. Epub 2017 Apr 21 doi: 10.1016/j.pathol.2017.03.001. PMID: 28438388
Croteau SE, Gupta D
Semin Cutan Med Surg 2016 Sep;35(3):147-52. doi: 10.12788/j.sder.2016.048. PMID: 27607323
Mittal R, Tripathy D
Diagn Pathol 2013 Sep 17;8:153. doi: 10.1186/1746-1596-8-153. PMID: 24044498Free PMC Article
Calonje E, Fletcher CD
Pathologica 1993 Jan-Feb;85(1095):1-15. PMID: 8390634

Clinical prediction guides

Feito-Rodríguez M, Sánchez-Orta A, De Lucas R, López-Gutiérrez JC, Ruiz-Bravo E, Baselga E, Victoria AM, Hernández-Martín A, Campos-Domínguez M, Berenguer Fröhner B, Garnacho-Saucedo G, Vera Casaño A, Vicente Villa A, Bernabeu-Wittel J, Kutzner H, Requena L
Pediatr Dermatol 2018 Nov;35(6):808-816. Epub 2018 Oct 15 doi: 10.1111/pde.13683. PMID: 30318642
Mittal R, Tripathy D
Diagn Pathol 2013 Sep 17;8:153. doi: 10.1186/1746-1596-8-153. PMID: 24044498Free PMC Article
Sadeghpour M, Antaya RJ, Lazova R, Ko CJ
Am J Dermatopathol 2012 Jun;34(4):400-3. doi: 10.1097/DAD.0b013e318234e720. PMID: 22307233
Wilmer A, Kaatz M, Bocker T, Wollina U
Eur J Dermatol 1999 Jan-Feb;9(1):51-3. PMID: 9920990
Calonje E, Fletcher CD
Pathologica 1993 Jan-Feb;85(1095):1-15. PMID: 8390634

Recent systematic reviews

Yao W, Li K, Wang Z, Pei J, Liu X, Zheng S, Dong K
Eur J Pediatr Surg 2019 Oct;29(5):401-407. Epub 2018 Oct 29 doi: 10.1055/s-0038-1673708. PMID: 30372769
Liu XH, Li JY, Qu XH, Yan WL, Zhang L, Yang C, Zheng JW
Int J Cancer 2016 Oct 1;139(7):1658-66. Epub 2016 Jun 28 doi: 10.1002/ijc.30216. PMID: 27252149

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