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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

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
Cloutier-Bosworth A, Naert K, Kirshen C
J Cutan Med Surg 2017 Jul/Aug;21(4):356-358. Epub 2017 Mar 27 doi: 10.1177/1203475417702145. PMID: 28346010
Fernandes KA, Lima LA, Guedes JC, Lima RB, D'Acri AM, Martins CJ
An Bras Dermatol 2016 Sep-Oct;91(5 suppl 1):10-13. doi: 10.1590/abd1806-4841.20164619. PMID: 28300880Free 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

Diagnosis

Komzak K, McKelvie P, Goold L
Ophthalmic Plast Reconstr Surg 2022 Jul-Aug 01;38(4):e109-e111. Epub 2022 Mar 21 doi: 10.1097/IOP.0000000000002152. PMID: 35797673
Bellinato F, Maurelli M, Gisondi P, Girolomoni G
Clin Exp Dermatol 2022 Jan;47(1):80-85. Epub 2021 Aug 27 doi: 10.1111/ced.14851. PMID: 34260091
Vishwanath T, Nagpal A, Shinde G
JAMA Dermatol 2019 Dec 1;155(12):1418-1419. doi: 10.1001/jamadermatol.2019.2963. PMID: 31642871
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
Dourmishev A, Miteva L, Mitev V, Pramatarov K, Schwartz RA
Cutis 2000 Dec;66(6):420-4. PMID: 11138359

Therapy

Mouchard A, Tallegas M, Machet MC, Machet L, Cornillier H
Acta Derm Venereol 2018 Aug 29;98(8):822-823. doi: 10.2340/00015555-2971. PMID: 29774365
Cloutier-Bosworth A, Naert K, Kirshen C
J Cutan Med Surg 2017 Jul/Aug;21(4):356-358. Epub 2017 Mar 27 doi: 10.1177/1203475417702145. PMID: 28346010
Chabra IS, Grandinetti LM
Cutis 2014 Oct;94(4):E1-3. PMID: 25372260
Koraishy FM, Cohen RA, Israel GM, Dahl NK
Am J Kidney Dis 2013 Oct;62(4):806-9. Epub 2013 Jun 21 doi: 10.1053/j.ajkd.2013.04.017. PMID: 23796907
Lewis BK, Chern PL, Stone MS
J Am Acad Dermatol 2009 Apr;60(4):700-3. doi: 10.1016/j.jaad.2008.09.018. PMID: 19293020

Prognosis

Cloutier-Bosworth A, Naert K, Kirshen C
J Cutan Med Surg 2017 Jul/Aug;21(4):356-358. Epub 2017 Mar 27 doi: 10.1177/1203475417702145. PMID: 28346010
Boccaletti VP, Ricci R, De Panfilis G
Dermatol Online J 2011 May 15;17(5):12. PMID: 21635834
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
Kleine RT, Mendes R, Pugliese R, Miura I, Danesi V, Porta G
Clinics (Sao Paulo) 2012;67(3):231-5. doi: 10.6061/clinics/2012(03)05. PMID: 22473403Free PMC Article
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
Morgan MB, Truitt CA, Taira J, Somach S, Pitha JV, Everett MA
Am J Dermatopathol 1998 Apr;20(2):147-54. doi: 10.1097/00000372-199804000-00008. PMID: 9557783
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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