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Porokeratotic eccrine ostial and dermal duct nevus

MedGen UID:
96880
Concept ID:
C0473579
Neoplastic Process
Synonyms: Comedo nevus of the palm; comedo nevus of the palm; Linear eccrine nevi with comedones; Linear Eccrine Nevus with Comedones; linear eccrine Nevus with comedones; Porokeratotic eccrine duct and hair follicle nevus; Porokeratotic Eccrine Duct and Hair Follicle Nevus; Porokeratotic eccrine duct and hair follicle Nevus; Porokeratotic eccrine nevus; Porokeratotic Eccrine Ostial and Dermal Duct Nevus; porokeratotic eccrine ostial and dermal duct nevus
SNOMED CT: Linear eccrine nevi with comedones (239118007); Porokeratotic eccrine duct and hair follicle nevus (239118007); Porokeratotic eccrine ostial and dermal duct nevus (239118007)
 
Monarch Initiative: MONDO:0015635
Orphanet: ORPHA166286

Definition

A rare, congenital disorder of the eccrine sweat ducts that presents as grouped keratotic papules and plaques with a linear distribution and/or multiple punctate pits filled with tiny keratotic plugs resembling comedones. The lesion are usually located on the acral portion of a limb. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPorokeratotic eccrine ostial and dermal duct nevus
Follow this link to review classifications for Porokeratotic eccrine ostial and dermal duct nevus in Orphanet.

Recent clinical studies

Etiology

Levinsohn JL, McNiff JM, Antaya RJ, Choate KA
JAMA Dermatol 2015 Jun;151(6):638-41. doi: 10.1001/jamadermatol.2014.5069. PMID: 25692760
Bandoyopadhyay D, Saha A
Dermatol Online J 2014 Jun 15;20(6) PMID: 24945650

Diagnosis

Karataş A, Seçkin D
Int J Dermatol 2020 May;59(5):617-619. Epub 2020 Jan 23 doi: 10.1111/ijd.14775. PMID: 31975368
Pathak D, Kubba R, Kubba A
Indian J Dermatol Venereol Leprol 2011 Mar-Apr;77(2):174-6. doi: 10.4103/0378-6323.77457. PMID: 21393947
Sassmannshausen J, Bogomilsky J, Chaffins M
J Am Acad Dermatol 2000 Aug;43(2 Pt 2):364-7. doi: 10.1067/mjd.2000.100536. PMID: 10901725
Moreno A, Pujol RM, Salvatella N, Alomar A, de Moragas JM
J Cutan Pathol 1988 Feb;15(1):43-8. doi: 10.1111/j.1600-0560.1988.tb00515.x. PMID: 3280630
Aloi FG, Pippione M
Arch Dermatol 1986 Aug;122(8):892-5. PMID: 3740872

Therapy

Alomran H, Kanitakis J
Dermatol Online J 2020 Feb 15;26(2) PMID: 32239895
Bhunia D, Ghosh S, Rudra O, Biswas SK
Dermatol Online J 2014 Sep 16;20(9) PMID: 25244169
Jain S, Sardana K, Garg VK
Pediatr Dermatol 2013 Mar-Apr;30(2):264-6. Epub 2012 Feb 16 doi: 10.1111/j.1525-1470.2011.01659.x. PMID: 22339989
Lazic T, Li Q, Frank M, Uitto J, Zhou LH
Pediatr Dermatol 2012 May-Jun;29(3):349-57. Epub 2011 Oct 20 doi: 10.1111/j.1525-1470.2011.01425.x. PMID: 22011219
Wong JW, Summers EM, Taylor MB, Harris RM
Dermatol Online J 2011 Sep 15;17(9):10. PMID: 21971275

Prognosis

Bergman R, Lichtig C, Cohen A, Friedman-Birnbaum R
Am J Dermatopathol 1992 Aug;14(4):319-22. doi: 10.1097/00000372-199208000-00006. PMID: 1380208

Clinical prediction guides

Levinsohn JL, McNiff JM, Antaya RJ, Choate KA
JAMA Dermatol 2015 Jun;151(6):638-41. doi: 10.1001/jamadermatol.2014.5069. PMID: 25692760
Goddard DS, Rogers M, Frieden IJ, Krol AL, White CR Jr, Jayaraman AG, Robinson-Bostom L, Bruckner AL, Ruben BS
J Am Acad Dermatol 2009 Dec;61(6):1060.e1-14. Epub 2009 Aug 6 doi: 10.1016/j.jaad.2009.03.036. PMID: 19664847
Rasi A, Tajziechi L
Arch Iran Med 2008 Mar;11(2):218-20. PMID: 18298304
Bergman R, Lichtig C, Cohen A, Friedman-Birnbaum R
Am J Dermatopathol 1992 Aug;14(4):319-22. doi: 10.1097/00000372-199208000-00006. PMID: 1380208

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