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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
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
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 in a Bilateral Distribution: An Unusual Presentation.
Yadav P, Sanke S, Mendiratta V, Chander R
Skinmed 2022;20(3):220-223. Epub 2022 Jun 30 PMID: 35779030
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.
Kim C
Dermatol Online J 2005 Dec 30;11(4):22. PMID: 16403394

Therapy

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
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
Linear porokeratosis of Mibelli.
Eyre WG, Carson WE
Arch Dermatol 1972 Mar;105(3):426-9. PMID: 5012146

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
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
A case of extensive linear porokeratosis with evaluation of topical tretinoin versus 5-flourouracil as treatment modalities.
Grover C, Goel A, Nanda S, Khurana N, Reddy BS
J Dermatol 2005 Dec;32(12):1000-4. doi: 10.1111/j.1346-8138.2005.tb00890.x. PMID: 16471466
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
Linear porokeratosis: treatment with the carbon dioxide laser.
Barnett JH
J Am Acad Dermatol 1986 May;14(5 Pt 2):902-4. doi: 10.1016/s0190-9622(86)70108-4. PMID: 3086391

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
Squamous cell carcinoma in a renal transplant recipient with linear porokeratosis.
Anzai S, Takeo N, Yamaguchi T, Sato T, Takasaki S, Terashi H, Takayasu S, Takahashi S
J Dermatol 1999 Apr;26(4):244-7. doi: 10.1111/j.1346-8138.1999.tb03465.x. PMID: 10343471

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