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Linear scleroderma

MedGen UID:
75518
•Concept ID:
C0263409
•
Disease or Syndrome
Synonym: Linear scleroderma (subtype)
SNOMED CT: Linear scleroderma (22784002); Linear morphea (22784002)
 
Monarch Initiative: MONDO:0043294

Definition

A type of localized scleroderma characterized by a long strip of indurated skin, which is typically found unilaterally on an arm or leg, and sometimes on the forehead or trunk. This disorder often affects the tissues beneath the skin, causing damage to bones, muscle or other organs. It can limit movement, alter growth, and disfigure the affected area. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLinear scleroderma

Professional guidelines

PubMed

Treatment in Juvenile Scleroderma.
Zulian F, Tirelli F
Curr Rheumatol Rep 2020 Jun 26;22(8):45. doi: 10.1007/s11926-020-00910-x. PMID: 32591919
Diagnosis and management of linear scleroderma in children.
Peña-Romero AG, García-Romero MT
Curr Opin Pediatr 2019 Aug;31(4):482-490. doi: 10.1097/MOP.0000000000000785. PMID: 31157654
Update on the classification and treatment of localized scleroderma.
Bielsa Marsol I
Actas Dermosifiliogr 2013 Oct;104(8):654-66. Epub 2013 Aug 13 doi: 10.1016/j.adengl.2012.10.012. PMID: 23948159

Recent clinical studies

Etiology

Juvenile Localized Scleroderma: Updates and Differences from Adult-Onset Disease.
Vasquez-Canizares N, Li SC
Rheum Dis Clin North Am 2021 Nov;47(4):737-755. Epub 2021 Sep 2 doi: 10.1016/j.rdc.2021.07.014. PMID: 34635302
Autoimmunity in Segmental Vitiligo.
Speeckaert R, Lambert J, Bulat V, Belpaire A, Speeckaert M, van Geel N
Front Immunol 2020;11:568447. Epub 2020 Oct 27 doi: 10.3389/fimmu.2020.568447. PMID: 33193342Free PMC Article
Treatment in Juvenile Scleroderma.
Zulian F, Tirelli F
Curr Rheumatol Rep 2020 Jun 26;22(8):45. doi: 10.1007/s11926-020-00910-x. PMID: 32591919
Overview of Juvenile localized scleroderma and its management.
Li SC, Zheng RJ
World J Pediatr 2020 Feb;16(1):5-18. Epub 2019 Nov 30 doi: 10.1007/s12519-019-00320-9. PMID: 31786801
Interventions for morphea.
Albuquerque JV, Andriolo BN, Vasconcellos MR, Civile VT, Lyddiatt A, Trevisani VF
Cochrane Database Syst Rev 2019 Jul 16;7(7):CD005027. doi: 10.1002/14651858.CD005027.pub5. PMID: 31309547Free PMC Article

Diagnosis

Morphological and clinical picture of the morphea in the oral cavity.
Pedowska M, Ptasiewicz M, Szumiło J, Chałas R
Folia Morphol (Warsz) 2022;81(3):544-550. Epub 2021 Oct 5 doi: 10.5603/FM.a2021.0083. PMID: 34608983
Interventions for morphea.
Albuquerque JV, Andriolo BN, Vasconcellos MR, Civile VT, Lyddiatt A, Trevisani VF
Cochrane Database Syst Rev 2019 Jul 16;7(7):CD005027. doi: 10.1002/14651858.CD005027.pub5. PMID: 31309547Free PMC Article
Diagnosis and management of linear scleroderma in children.
Peña-Romero AG, García-Romero MT
Curr Opin Pediatr 2019 Aug;31(4):482-490. doi: 10.1097/MOP.0000000000000785. PMID: 31157654
Ophthalmological manifestations of Parry-Romberg syndrome.
Bucher F, Fricke J, Neugebauer A, Cursiefen C, Heindl LM
Surv Ophthalmol 2016 Nov-Dec;61(6):693-701. Epub 2016 Apr 1 doi: 10.1016/j.survophthal.2016.03.009. PMID: 27045226
Progressive hemifacial atrophy: a review.
Tolkachjov SN, Patel NG, Tollefson MM
Orphanet J Rare Dis 2015 Apr 1;10:39. doi: 10.1186/s13023-015-0250-9. PMID: 25881068Free PMC Article

Therapy

Treatment in Juvenile Scleroderma.
Zulian F, Tirelli F
Curr Rheumatol Rep 2020 Jun 26;22(8):45. doi: 10.1007/s11926-020-00910-x. PMID: 32591919
Interventions for morphea.
Albuquerque JV, Andriolo BN, Vasconcellos MR, Civile VT, Lyddiatt A, Trevisani VF
Cochrane Database Syst Rev 2019 Jul 16;7(7):CD005027. doi: 10.1002/14651858.CD005027.pub5. PMID: 31309547Free PMC Article
Juvenile Scleroderma-What has Changed in the Meantime?
Adrovic A, Sahin S, Barut K, Kasapcopur O
Curr Rheumatol Rev 2018;14(3):219-225. doi: 10.2174/1573397114666180423105056. PMID: 29692255
Morphea in Childhood: An Update.
Aranegui B, Jiménez-Reyes J
Actas Dermosifiliogr (Engl Ed) 2018 May;109(4):312-322. Epub 2017 Dec 14 doi: 10.1016/j.ad.2017.06.021. PMID: 29248149
Update on the classification and treatment of localized scleroderma.
Bielsa Marsol I
Actas Dermosifiliogr 2013 Oct;104(8):654-66. Epub 2013 Aug 13 doi: 10.1016/j.adengl.2012.10.012. PMID: 23948159

Prognosis

Diagnosis and management of linear scleroderma in children.
Peña-Romero AG, García-Romero MT
Curr Opin Pediatr 2019 Aug;31(4):482-490. doi: 10.1097/MOP.0000000000000785. PMID: 31157654
Juvenile Scleroderma-What has Changed in the Meantime?
Adrovic A, Sahin S, Barut K, Kasapcopur O
Curr Rheumatol Rev 2018;14(3):219-225. doi: 10.2174/1573397114666180423105056. PMID: 29692255
Morphea in Childhood: An Update.
Aranegui B, Jiménez-Reyes J
Actas Dermosifiliogr (Engl Ed) 2018 May;109(4):312-322. Epub 2017 Dec 14 doi: 10.1016/j.ad.2017.06.021. PMID: 29248149
Progressive hemifacial atrophy: a review.
Tolkachjov SN, Patel NG, Tollefson MM
Orphanet J Rare Dis 2015 Apr 1;10:39. doi: 10.1186/s13023-015-0250-9. PMID: 25881068Free PMC Article
Localized scleroderma.
Tuffanelli DL
Semin Cutan Med Surg 1998 Mar;17(1):27-33. doi: 10.1016/s1085-5629(98)80059-x. PMID: 9512104

Clinical prediction guides

Linear alopecia in pediatrics: RCM and dermoscopy reveal diagnostic cues.
Wang Y, Chen L, Qin B, Li Q
Skin Res Technol 2023 Nov;29(11):e13523. doi: 10.1111/srt.13523. PMID: 38009024Free PMC Article
Autoimmunity in Segmental Vitiligo.
Speeckaert R, Lambert J, Bulat V, Belpaire A, Speeckaert M, van Geel N
Front Immunol 2020;11:568447. Epub 2020 Oct 27 doi: 10.3389/fimmu.2020.568447. PMID: 33193342Free PMC Article
Treatment in Juvenile Scleroderma.
Zulian F, Tirelli F
Curr Rheumatol Rep 2020 Jun 26;22(8):45. doi: 10.1007/s11926-020-00910-x. PMID: 32591919
Interventions for morphea.
Albuquerque JV, Andriolo BN, Vasconcellos MR, Civile VT, Lyddiatt A, Trevisani VF
Cochrane Database Syst Rev 2019 Jul 16;7(7):CD005027. doi: 10.1002/14651858.CD005027.pub5. PMID: 31309547Free PMC Article
Hair disorders associated with autoimmune connective tissue diseases.
Cassano N, Amerio P, D'Ovidio R, Vena GA
G Ital Dermatol Venereol 2014 Oct;149(5):555-65. Epub 2014 Jun 30 PMID: 24975949

Recent systematic reviews

Systematic Review of Health-Related Quality of Life Impact in Juvenile Localized Scleroderma.
Sanchez-Espino LF, Luca N, Pope E, Laxer RM, Knight AM, Sibbald C
Arthritis Care Res (Hoboken) 2024 Mar;76(3):340-349. Epub 2024 Jan 7 doi: 10.1002/acr.25243. PMID: 37750181
Epilepsy in Parry-Romberg syndrome and linear scleroderma en coup de sabre: Case series and systematic review including 140 patients.
Hixon AM, Christensen E, Hamilton R, Drees C
Epilepsy Behav 2021 Aug;121(Pt A):108068. Epub 2021 May 28 doi: 10.1016/j.yebeh.2021.108068. PMID: 34052630Free PMC Article
Interventions for morphea.
Albuquerque JV, Andriolo BN, Vasconcellos MR, Civile VT, Lyddiatt A, Trevisani VF
Cochrane Database Syst Rev 2019 Jul 16;7(7):CD005027. doi: 10.1002/14651858.CD005027.pub5. PMID: 31309547Free PMC Article

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