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Elastosis perforans serpiginosa(EPS)

MedGen UID:
65137
Concept ID:
C0221271
Disease or Syndrome
Synonyms: Elastoma intrapapillare perforans verruciformis; EPS; Miescher elastoma
SNOMED CT: Perforating serpiginous elastosis (49428008); Miescher's elastoma (49428008); Keratosis follicularis serpiginosa (49428008); Perforating elastoma (49428008); Elastosis perforans serpiginosa (49428008); Elastosis perforans (49428008); Perforating elastosis (49428008)
 
Monarch Initiative: MONDO:0007529
OMIM®: 130100
Orphanet: ORPHA79148

Definition

A rare acquired dermis elastic tissue disorder with increased elastic tissue characterized by focal dermal elastosis and transepidermal elimination of abnormal elastic fibers, presenting as small keratotic papules or plaques arranged in groups in serpiginous or annular patterns on the neck, face, and arms, while other areas are less frequently affected. Although spontaneous regression is possible, the lesions often persist over longer periods of time. The condition typically occurs during childhood or early adulthood and is more frequent in men than in women. [from ORDO]

Clinical features

From HPO
Nevus
MedGen UID:
45074
Concept ID:
C0027960
Neoplastic Process
A nevus is a type of hamartoma that is a circumscribed stable malformation of the skin.
  • Abnormality of the integument

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVElastosis perforans serpiginosa
Follow this link to review classifications for Elastosis perforans serpiginosa in Orphanet.

Professional guidelines

PubMed

Kawakami T, Akiyama M, Ishida-Yamamoto A, Nakano H, Mitoma C, Yoneda K, Suga Y
J Dermatol 2020 Dec;47(12):1374-1382. Epub 2020 Oct 23 doi: 10.1111/1346-8138.15647. PMID: 33094871

Recent clinical studies

Etiology

Kawakami T, Akiyama M, Ishida-Yamamoto A, Nakano H, Mitoma C, Yoneda K, Suga Y
J Dermatol 2020 Dec;47(12):1374-1382. Epub 2020 Oct 23 doi: 10.1111/1346-8138.15647. PMID: 33094871
Valenzuela-Ubiña S, Jiménez-Gallo D, Russo-de la Torre F, Linares-Barrios M
Dermatol Ther 2020 Jul;33(4):e13692. Epub 2020 Jun 28 doi: 10.1111/dth.13692. PMID: 32458537
Montesu MA, Onnis G, Gunnella S, Lissia A, Satta R
Eur J Dermatol 2018 Aug 1;28(4):476-481. doi: 10.1684/ejd.2018.3355. PMID: 30129530
Quinlan C, Boggs J, Finan M, Mulligan N, Gulmann C, O'Kane M, Ralph N
Int J Dermatol 2018 Apr;57(4):470-472. Epub 2018 Jan 22 doi: 10.1111/ijd.13854. PMID: 29355938
Frew JW, Murrell DF, Haber RM
Int J Dermatol 2015 Oct;54(10):1109-23. Epub 2015 Jul 30 doi: 10.1111/ijd.12945. PMID: 26227781

Diagnosis

Andrés-Ramos I, Alegría-Landa V, Gimeno I, Pérez-Plaza A, Rütten A, Kutzner H, Requena L
Am J Dermatopathol 2019 Feb;41(2):85-117. doi: 10.1097/DAD.0000000000001275. PMID: 30688725
Menzies S, Kirby B
BMJ Case Rep 2015 Dec 7;2015 doi: 10.1136/bcr-2015-212482. PMID: 26643186Free PMC Article
Frew JW, Murrell DF, Haber RM
Int J Dermatol 2015 Oct;54(10):1109-23. Epub 2015 Jul 30 doi: 10.1111/ijd.12945. PMID: 26227781
Atzori L, Pinna AL, Pau M, Aste N
Dermatol Online J 2011 Apr 15;17(4):3. PMID: 21549078
Suneja T, Zelonis B, Hurley MY, Youker SR
Skinmed 2007 Sep-Oct;6(5):255-6. doi: 10.1111/j.1540-9740.2007.06438.x. PMID: 17786109

Therapy

Valenzuela-Ubiña S, Jiménez-Gallo D, Russo-de la Torre F, Linares-Barrios M
Dermatol Ther 2020 Jul;33(4):e13692. Epub 2020 Jun 28 doi: 10.1111/dth.13692. PMID: 32458537
Montesu MA, Onnis G, Gunnella S, Lissia A, Satta R
Eur J Dermatol 2018 Aug 1;28(4):476-481. doi: 10.1684/ejd.2018.3355. PMID: 30129530
Alique-García S, Company-Quiroga J, Horcajada-Reales C, Echeverría-García B, Tardío-Dovao JC, Borbujo J
Photodiagnosis Photodyn Ther 2018 Mar;21:55-57. Epub 2017 Nov 10 doi: 10.1016/j.pdpdt.2017.11.001. PMID: 29129543
Menzies S, Kirby B
BMJ Case Rep 2015 Dec 7;2015 doi: 10.1136/bcr-2015-212482. PMID: 26643186Free PMC Article
Atzori L, Pinna AL, Pau M, Aste N
Dermatol Online J 2011 Apr 15;17(4):3. PMID: 21549078

Prognosis

Di Nuzzo S, Bertolani M, Casanova D, Manuguerra R, de Felici Del Giudice MB, Feliciani C
Clin Exp Dermatol 2021 Jan;46(1):219-222. Epub 2020 Sep 15 doi: 10.1111/ced.14436. PMID: 32931593
Kelati A, Lagrange S, Le Duff F, Lacour JP, Passeron T
JAMA Dermatol 2017 Oct 1;153(10):1063-1064. doi: 10.1001/jamadermatol.2017.2223. PMID: 28746703
Khatu SS, Dhurat RS, Nayak CS, Pereira RR, Kagne RB
Indian J Dermatol Venereol Leprol 2011 Jan-Feb;77(1):55-8. doi: 10.4103/0378-6323.74982. PMID: 21220881
Rath N, Bhardwaj A, Kar HK, Sharma PK, Bharadwaj M, Bharija SC
Indian J Dermatol Venereol Leprol 2005 May-Jun;71(3):182-5. doi: 10.4103/0378-6323.16234. PMID: 16394408
Sahn EE, Maize JC, Garen PD, Mullins SC, Silver RM
J Am Acad Dermatol 1989 May;20(5 Pt 2):979-88. doi: 10.1016/s0190-9622(89)70123-7. PMID: 2523911

Clinical prediction guides

Montesu MA, Onnis G, Gunnella S, Lissia A, Satta R
Eur J Dermatol 2018 Aug 1;28(4):476-481. doi: 10.1684/ejd.2018.3355. PMID: 30129530
Nasca MR, Lacarrubba F, Caltabiano R, Verzì AE, Micali G
J Cutan Pathol 2016 Nov;43(11):1021-1024. Epub 2016 Aug 12 doi: 10.1111/cup.12767. PMID: 27404989
Khatu SS, Dhurat RS, Nayak CS, Pereira RR, Kagne RB
Indian J Dermatol Venereol Leprol 2011 Jan-Feb;77(1):55-8. doi: 10.4103/0378-6323.74982. PMID: 21220881
Bécuwe C, Dalle S, Ronger-Savlé S, Skowron F, Balme B, Kanitakis J, Thomas L
Dermatology 2005;210(1):60-3. doi: 10.1159/000081487. PMID: 15604549
Langeveld-Wildschut EG, Toonstra J, van Vloten WA, Beemer FA
Arch Dermatol 1993 Feb;129(2):205-7. PMID: 7679569

Recent systematic reviews

Montesu MA, Onnis G, Gunnella S, Lissia A, Satta R
Eur J Dermatol 2018 Aug 1;28(4):476-481. doi: 10.1684/ejd.2018.3355. PMID: 30129530

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