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Linear porokeratosis

MedGen UID:
81293
•Concept ID:
C0302319
•
Disease or Syndrome
Synonym: Congenital facial linear porokeratosis (type)
SNOMED CT: Linear porokeratosis (238631008); Zosteriform porokeratosis (238631008)
 
Monarch Initiative: MONDO:0023246

Definition

Linear porokeratosis is a rare skin condition characterized by streaks of reddish-brown patches surrounded by a ridge-like border.The patches usually develop in infants or young children, but they sometimes develop in adults. [from MONDO]

Professional guidelines

PubMed

Porokeratosis: A Review of Its Pathophysiology, Clinical Manifestations, Diagnosis, and Treatment.
Vargas-Mora P, Morgado-Carrasco D, Fustà-Novell X
Actas Dermosifiliogr (Engl Ed) 2020 Sep;111(7):545-560. Epub 2020 May 13 doi: 10.1016/j.ad.2020.03.005. PMID: 32401728
Topical cholesterol/lovastatin for the treatment of porokeratosis: A pathogenesis-directed therapy.
Atzmony L, Lim YH, Hamilton C, Leventhal JS, Wagner A, Paller AS, Choate KA
J Am Acad Dermatol 2020 Jan;82(1):123-131. Epub 2019 Aug 23 doi: 10.1016/j.jaad.2019.08.043. PMID: 31449901Free PMC Article

Recent clinical studies

Etiology

Inflammatory linear verrucous epidermal nevus (ILVEN) encompasses a spectrum of inflammatory mosaic disorders.
Atzmony L, Ugwu N, Hamilton C, Paller AS, Zech L, Antaya RJ, Choate KA
Pediatr Dermatol 2022 Nov;39(6):903-907. Epub 2022 Jul 19 doi: 10.1111/pde.15094. PMID: 35853659Free PMC Article
Porokeratosis: A Review of Its Pathophysiology, Clinical Manifestations, Diagnosis, and Treatment.
Vargas-Mora P, Morgado-Carrasco D, Fustà-Novell X
Actas Dermosifiliogr (Engl Ed) 2020 Sep;111(7):545-560. Epub 2020 May 13 doi: 10.1016/j.ad.2020.03.005. PMID: 32401728
Topical cholesterol/lovastatin for the treatment of porokeratosis: A pathogenesis-directed therapy.
Atzmony L, Lim YH, Hamilton C, Leventhal JS, Wagner A, Paller AS, Choate KA
J Am Acad Dermatol 2020 Jan;82(1):123-131. Epub 2019 Aug 23 doi: 10.1016/j.jaad.2019.08.043. PMID: 31449901Free PMC Article
Second-Hit, Postzygotic PMVK and MVD Mutations in Linear Porokeratosis.
Atzmony L, Khan HM, Lim YH, Paller AS, Levinsohn JL, Holland KE, Mirza FN, Yin E, Ko CJ, Leventhal JS, Choate KA
JAMA Dermatol 2019 May 1;155(5):548-555. doi: 10.1001/jamadermatol.2019.0016. PMID: 30942823Free PMC Article
Linear porokeratosis.
Malhotra SK, Puri KJ, Goyal T, Chahal KS
Dermatol Online J 2007 Oct 13;13(4):15. PMID: 18319012

Diagnosis

Inflammatory linear verrucous epidermal nevus (ILVEN) encompasses a spectrum of inflammatory mosaic disorders.
Atzmony L, Ugwu N, Hamilton C, Paller AS, Zech L, Antaya RJ, Choate KA
Pediatr Dermatol 2022 Nov;39(6):903-907. Epub 2022 Jul 19 doi: 10.1111/pde.15094. PMID: 35853659Free PMC Article
Generalized Linear Porokeratosis.
Lu Y, Feng L
J Cutan Med Surg 2022 Nov-Dec;26(6):639. Epub 2022 Feb 8 doi: 10.1177/12034754221077925. PMID: 35134308
Porokeratosis: A Review of Its Pathophysiology, Clinical Manifestations, Diagnosis, and Treatment.
Vargas-Mora P, Morgado-Carrasco D, Fustà-Novell X
Actas Dermosifiliogr (Engl Ed) 2020 Sep;111(7):545-560. Epub 2020 May 13 doi: 10.1016/j.ad.2020.03.005. PMID: 32401728
Linear porokeratosis.
Malhotra SK, Puri KJ, Goyal T, Chahal KS
Dermatol Online J 2007 Oct 13;13(4):15. PMID: 18319012
Linear porokeratosis.
Kim C
Dermatol Online J 2005 Dec 30;11(4):22. PMID: 16403394

Therapy

Pathogenesis-directed treatment of linear porokeratosis with topical cholesterol-lovastatin.
Maredia H, Hand JL, Tollefson MM
Pediatr Dermatol 2024 Mar-Apr;41(2):296-297. Epub 2023 Sep 19 doi: 10.1111/pde.15427. PMID: 37726979
Inflammatory linear verrucous epidermal nevus (ILVEN) encompasses a spectrum of inflammatory mosaic disorders.
Atzmony L, Ugwu N, Hamilton C, Paller AS, Zech L, Antaya RJ, Choate KA
Pediatr Dermatol 2022 Nov;39(6):903-907. Epub 2022 Jul 19 doi: 10.1111/pde.15094. PMID: 35853659Free PMC Article
Successful Clearance of Linear Porokeratosis With Aminolevulinic Acid and Pulsed Dye Laser.
Casale F, Tchanque-Fossuo CN, Durkin JR
Dermatol Surg 2021 Sep 1;47(9):1300-1301. doi: 10.1097/DSS.0000000000002996. PMID: 34448766
Topical cholesterol/lovastatin for the treatment of porokeratosis: A pathogenesis-directed therapy.
Atzmony L, Lim YH, Hamilton C, Leventhal JS, Wagner A, Paller AS, Choate KA
J Am Acad Dermatol 2020 Jan;82(1):123-131. Epub 2019 Aug 23 doi: 10.1016/j.jaad.2019.08.043. PMID: 31449901Free PMC Article
Generalized linear porokeratosis.
Dervis E, Demirkesen C
Int J Dermatol 2006 Sep;45(9):1077-9. doi: 10.1111/j.1365-4632.2004.02490.x. PMID: 16961514

Prognosis

Long-term clearance of linear porokeratosis with tacrolimus, 0.1%, ointment.
Parks AC, Conner KJ, Armstrong CA
JAMA Dermatol 2014 Feb;150(2):194-6. doi: 10.1001/jamadermatol.2013.8526. PMID: 24553841
A case of linear porokeratosis treated with photodynamic therapy with confocal microscopy surveillance.
Curkova AK, Hegyi J, Kozub P, Szep Z, D'Erme AM, Simaljakova M
Dermatol Ther 2014 May-Jun;27(3):144-7. Epub 2013 Oct 1 doi: 10.1111/dth.12097. PMID: 24119172
Giant ulcerating squamous cell carcinoma arising from linear porokeratosis: a case study.
Vivas AC, Maderal AD, Kirsner RS
Ostomy Wound Manage 2012 Nov;58(11):18-20. PMID: 23134898
Linear porokeratosis: excellent response to photodynamic therapy.
García-Navarro X, Garcés JR, Baselga E, Alomar A
Arch Dermatol 2009 May;145(5):526-7. doi: 10.1001/archdermatol.2009.45. PMID: 19451495
Multiple squamous cell carcinomas complicating linear porokeratosis.
Curnow P, Foley P, Baker C
Australas J Dermatol 2003 May;44(2):136-9. doi: 10.1046/j.1440-0960.2003.00661.x. PMID: 12752189

Clinical prediction guides

Dermoscopy of Zosteriform and Swirling Pattern Type 1 Segmental Darier Disease.
Balić A, Garces Rene J, Radoš J
Acta Dermatovenerol Croat 2022 Nov;30(3):191-193. PMID: 36812281
A case of linear porokeratosis treated with photodynamic therapy with confocal microscopy surveillance.
Curkova AK, Hegyi J, Kozub P, Szep Z, D'Erme AM, Simaljakova M
Dermatol Ther 2014 May-Jun;27(3):144-7. Epub 2013 Oct 1 doi: 10.1111/dth.12097. PMID: 24119172
Porokeratosis in Singapore: an Asian perspective.
Tan LS, Chong WS
Australas J Dermatol 2012 May;53(2):e40-4. Epub 2012 Jan 19 doi: 10.1111/j.1440-0960.2011.00856.x. PMID: 22571584
Generalized linear porokeratosis.
Dervis E, Demirkesen C
Int J Dermatol 2006 Sep;45(9):1077-9. doi: 10.1111/j.1365-4632.2004.02490.x. PMID: 16961514
Linear porokeratosis superimposed on disseminated superficial actinic porokeratosis: report of two cases exemplifying the concept of type 2 segmental manifestation of autosomal dominant skin disorders.
Freyschmidt-Paul P, Hoffmann R, König A, Happle R
J Am Acad Dermatol 1999 Oct;41(4):644-7. PMID: 10495390

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