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Ulerythema ophryogenesis

MedGen UID:
1813086
Concept ID:
C5700076
Disease or Syndrome
Synonyms: Ulerythema ophryogenes; Ulerythema ophryogenes with multiple congenital anomalies
SNOMED CT: Ulerythema ophryogenes (400126005)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0018086
Orphanet: ORPHA3406

Definition

Ulerythema ophryogenesis is characterised by inflammatory keratotic papules occurring on the face, which may be followed by scars, atrophy and alopecia. Prevalence is unknown but the disease, affecting mainly children and young adults, is rare. Erythema with mild hyperkeratosis of the hair follicles resulting in rough papules is observed on the cheeks and lateral aspects of the eyebrows. The disorder occasionally extends to the adjacent scalp, ears and forehead and rarely to the extensor surfaces of the limbs. Symptoms regress with age, although loss of the lateral aspects of the eyebrows can occur. Many cases occur sporadically; autosomal dominant inheritance has also been reported. There is no particular treatment, but patients should avoid sun exposure without UV protection. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUlerythema ophryogenesis

Professional guidelines

PubMed

Kodali N, Patel VM, Schwartz RA
Ital J Dermatol Venerol 2023 Jun;158(3):217-223. Epub 2023 May 11 doi: 10.23736/S2784-8671.23.07594-1. PMID: 37166753
Kavamura MI, Leoni C, Neri G
Am J Med Genet C Semin Med Genet 2022 Dec;190(4):452-458. Epub 2022 Dec 21 doi: 10.1002/ajmg.c.32027. PMID: 36541891
Maghfour J, Ly S, Haidari W, Taylor SL, Feldman SR
J Dermatolog Treat 2022 May;33(3):1231-1242. Epub 2020 Sep 14 doi: 10.1080/09546634.2020.1818678. PMID: 32886029

Recent clinical studies

Etiology

Lan X, Qiao R, Sun J, Song H, Gao M, Mo R, Song Z, Yang Y, Jiang Y
J Dermatol 2024 Feb;51(2):253-260. Epub 2023 Dec 12 doi: 10.1111/1346-8138.17026. PMID: 38087855
Kus MM, Mulayim MK, Kus C, Doganer A, Ozturk P, Temiz F, Nazik H
J Cosmet Dermatol 2023 Oct;22(10):2791-2798. Epub 2023 May 2 doi: 10.1111/jocd.15773. PMID: 37128833
Pediatr Dermatol 2019 Nov;36(6):937-938. doi: 10.1111/pde.14062. PMID: 31778553
Haws RM, McIntee TJ, Green CB
Int J Dermatol 2019 Oct;58(10):1160-1164. Epub 2019 Feb 20 doi: 10.1111/ijd.14412. PMID: 30790276
Liakou AI, Esteves de Carvalho AV, Nazarenko LP
J Dermatol 2014 May;41(5):371-6. doi: 10.1111/1346-8138.12442. PMID: 24801913

Diagnosis

Kodali N, Patel VM, Schwartz RA
Ital J Dermatol Venerol 2023 Jun;158(3):217-223. Epub 2023 May 11 doi: 10.23736/S2784-8671.23.07594-1. PMID: 37166753
Drivenes JL, Vasilescu IC, Bygum A
Tidsskr Nor Laegeforen 2023 Mar 28;143(5) Epub 2023 Mar 7 doi: 10.4045/tidsskr.22.0513. PMID: 36987905
Kavamura MI, Leoni C, Neri G
Am J Med Genet C Semin Med Genet 2022 Dec;190(4):452-458. Epub 2022 Dec 21 doi: 10.1002/ajmg.c.32027. PMID: 36541891
Pediatr Dermatol 2019 Nov;36(6):e106-e107. doi: 10.1111/pde.14063. PMID: 31778571
Wang JF, Orlow SJ
Am J Clin Dermatol 2018 Oct;19(5):733-757. doi: 10.1007/s40257-018-0368-3. PMID: 30043128

Therapy

Greenzaid J, Nussbaum D, Friedman A
J Drugs Dermatol 2023 Oct 1;22(10):985-989. doi: 10.36849/JDD.7534. PMID: 37801530
Aramburu González A, Udondo González Del Tánago B, Orbea Sopeña A, González Hermosa MR
Actas Dermosifiliogr 2023 Oct;114(9):831-832. Epub 2022 Aug 10 doi: 10.1016/j.ad.2022.03.019. PMID: 35963327
Pediatr Dermatol 2019 Nov;36(6):937-938. doi: 10.1111/pde.14062. PMID: 31778553
Wang JF, Orlow SJ
Am J Clin Dermatol 2018 Oct;19(5):733-757. doi: 10.1007/s40257-018-0368-3. PMID: 30043128
Morton CM, Bhate C, Janniger CK, Schwartz RA
Cutis 2014 Feb;93(2):83-7. PMID: 24605344

Prognosis

Khan MU, Mohammad E, Abbas Y, Rezeq M, Mohammad B
Sci Rep 2023 Jun 22;13(1):10160. doi: 10.1038/s41598-023-36817-7. PMID: 37349344Free PMC Article
Lee YM, Song KH, Koo HS, Lee CS, Ko I, Lee SH, Huh KC
Gut Liver 2022 Sep 15;16(5):754-763. Epub 2022 Jan 7 doi: 10.5009/gnl210271. PMID: 35000932Free PMC Article
Pediatr Dermatol 2019 Nov;36(6):e106-e107. doi: 10.1111/pde.14063. PMID: 31778571
Pediatr Dermatol 2019 Nov;36(6):937-938. doi: 10.1111/pde.14062. PMID: 31778553
Ibrahim O, Khan M, Bolotin D, Dubina M, Nodzenski M, Disphanurat W, Kakar R, Yoo S, Whiting D, West DP, Poon E, Veledar E, Alam M
JAMA Dermatol 2015 Feb;151(2):187-91. doi: 10.1001/jamadermatol.2014.2211. PMID: 25372313

Clinical prediction guides

Lan X, Qiao R, Sun J, Song H, Gao M, Mo R, Song Z, Yang Y, Jiang Y
J Dermatol 2024 Feb;51(2):253-260. Epub 2023 Dec 12 doi: 10.1111/1346-8138.17026. PMID: 38087855
Lim PJ, Marcionelli G, Srikanthan P, Ndarugendamwo T, Pinner J, Rohrbach M, Giunta C
Front Endocrinol (Lausanne) 2023;14:1195704. Epub 2023 May 25 doi: 10.3389/fendo.2023.1195704. PMID: 37305034Free PMC Article
Suástegui-Rodríguez I, Camacho-Rosas LH, Peralta-Pedrero ML, Cruz FJ, Morales-Sánchez MA
Skinmed 2022;20(4):258-271. Epub 2022 Aug 31 PMID: 35976015
Landeck L, Visser M, Kezic S, John SM
Contact Dermatitis 2013 Mar;68(3):149-55. doi: 10.1111/j.1600-0536.2012.02171.x. PMID: 23421459
Silverberg NB
Cutis 2011 Mar;87(3):148-54. PMID: 21488574

Recent systematic reviews

Maghfour J, Ly S, Haidari W, Taylor SL, Feldman SR
J Dermatolog Treat 2022 May;33(3):1231-1242. Epub 2020 Sep 14 doi: 10.1080/09546634.2020.1818678. PMID: 32886029
Kechichian E, Jabbour S, El Hachem L, Tomb R, Helou J
Dermatol Surg 2020 Nov;46(11):1397-1402. doi: 10.1097/DSS.0000000000002441. PMID: 32804891

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