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Angiokeratoma circumscriptum naeviforme

MedGen UID:
1841777
Concept ID:
C5826379
Neoplastic Process
HPO: HP:0034410

Definition

A type of angiokeratoma that typically presents in childhood as a large, mostly linear and unilateral hyperkeratotic plaque which is composed of confluent keratotic papules. The size ranges from a few centimeters up to a major part of the body surface. Initially the lesions present as multiple reddish macules, clinically similar to a nevus flammeus. Within several years they develop into acanthokeratotic papules that agrregate into plaques which may have a hyperkeratotic surface. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAngiokeratoma circumscriptum naeviforme

Professional guidelines

PubMed

Yang CH, Ohara K
Dermatol Surg 2002 Oct;28(10):913-19; discussion 920. doi: 10.1046/j.1524-4725.2002.02061.x. PMID: 12410675

Recent clinical studies

Etiology

Abbenante D, Raone B, Baraldi C, Anna Carpanese M, Patrizi A
Acta Dermatovenerol Croat 2021 Dec;29(3):169-170. PMID: 34990348
Das A, Mohanty S, Gharami RC, Chowdhury SN, Kumar P, Das NK, Bandyopadhyay D
Clin Exp Dermatol 2021 Dec;46(8):1452-1461. Epub 2021 Jul 6 doi: 10.1111/ced.14753. PMID: 34022084
Buehler S, Itin P
Dermatology 2015;231(3):213-6. Epub 2015 Jul 24 doi: 10.1159/000435810. PMID: 26228617

Diagnosis

Chhabra G, Verma P
J Cutan Med Surg 2022 May-Jun;26(3):311. Epub 2021 May 12 doi: 10.1177/12034754211016289. PMID: 33980054
Abbenante D, Raone B, Baraldi C, Anna Carpanese M, Patrizi A
Acta Dermatovenerol Croat 2021 Dec;29(3):169-170. PMID: 34990348
Das A, Mohanty S, Gharami RC, Chowdhury SN, Kumar P, Das NK, Bandyopadhyay D
Clin Exp Dermatol 2021 Dec;46(8):1452-1461. Epub 2021 Jul 6 doi: 10.1111/ced.14753. PMID: 34022084
del Pozo J, Fonseca E
Dermatol Surg 2005 Feb;31(2):232-6. doi: 10.1111/j.1524-4725.2005.31048. PMID: 15762221
Schiller PI, Itin PH
Dermatology 1996;193(4):275-82. doi: 10.1159/000246270. PMID: 8993949

Prognosis

Schiller PI, Itin PH
Dermatology 1996;193(4):275-82. doi: 10.1159/000246270. PMID: 8993949

Clinical prediction guides

del Pozo J, Fonseca E
Dermatol Surg 2005 Feb;31(2):232-6. doi: 10.1111/j.1524-4725.2005.31048. PMID: 15762221

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