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Localized scleroderma

MedGen UID:
48586
•Concept ID:
C0036420
•
Disease or Syndrome
Synonyms: Circumscribed Scleroderma; Localized Scleroderma; Scleroderma, Circumscribed; Scleroderma, Localized; Sclerodermas, Localized
SNOMED CT: Localized dermatosclerosis (201048007); Morphea scleroderma (201048007); Circumscribed scleroderma (201048007); Addison's keloid (201048007); Localized scleroderma (201048007); Localized morphea (201048007)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0019562
Orphanet: ORPHA90289

Definition

Localized scleroderma is the skin localized form of scleroderma (see this term) characterized by fibrosis of the skin causing cutaneous plaques or strips. [from ORDO]

Professional guidelines

PubMed

Clinical Treatment Options in Scleroderma: Recommendations and Comprehensive Review.
Zhao M, Wu J, Wu H, Sawalha AH, Lu Q
Clin Rev Allergy Immunol 2022 Apr;62(2):273-291. Epub 2021 Jan 15 doi: 10.1007/s12016-020-08831-4. PMID: 33449302
European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: localized scleroderma, systemic sclerosis and overlap syndromes.
Knobler R, Moinzadeh P, Hunzelmann N, Kreuter A, Cozzio A, Mouthon L, Cutolo M, Rongioletti F, Denton CP, Rudnicka L, Frasin LA, Smith V, Gabrielli A, Aberer E, Bagot M, Bali G, Bouaziz J, Braae Olesen A, Foeldvari I, Frances C, Jalili A, Just U, Kähäri V, Kárpáti S, Kofoed K, Krasowska D, Olszewska M, Orteu C, Panelius J, Parodi A, Petit A, Quaglino P, Ranki A, Sanchez Schmidt JM, Seneschal J, Skrok A, Sticherling M, Sunderkötter C, Taieb A, Tanew A, Wolf P, Worm M, Wutte NJ, Krieg T
J Eur Acad Dermatol Venereol 2017 Sep;31(9):1401-1424. Epub 2017 Aug 9 doi: 10.1111/jdv.14458. PMID: 28792092
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

Gastrointestinal involvement in systemic sclerosis: An updated review.
Nassar M, Ghernautan V, Nso N, Nyabera A, Castillo FC, Tu W, Medina L, Ciobanu C, Alfishawy M, Rizzo V, Eskaros S, Mahdi M, Khalifa M, El-Kassas M
Medicine (Baltimore) 2022 Nov 11;101(45):e31780. doi: 10.1097/MD.0000000000031780. PMID: 36397401Free PMC Article
Scleroderma in Children and Adolescents: Localized Scleroderma and Systemic Sclerosis.
Li SC
Pediatr Clin North Am 2018 Aug;65(4):757-781. doi: 10.1016/j.pcl.2018.04.002. PMID: 30031497
Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review.
Ferreli C, Gasparini G, Parodi A, Cozzani E, Rongioletti F, Atzori L
Clin Rev Allergy Immunol 2017 Dec;53(3):306-336. doi: 10.1007/s12016-017-8625-4. PMID: 28712039
Morphea and Eosinophilic Fasciitis: An Update.
Mertens JS, Seyger MMB, Thurlings RM, Radstake TRDJ, de Jong EMGJ
Am J Clin Dermatol 2017 Aug;18(4):491-512. doi: 10.1007/s40257-017-0269-x. PMID: 28303481Free PMC Article
Scleroderma mimics.
Nashel J, Steen V
Curr Rheumatol Rep 2012 Feb;14(1):39-46. doi: 10.1007/s11926-011-0220-8. PMID: 22131103

Diagnosis

Gastrointestinal involvement in systemic sclerosis: An updated review.
Nassar M, Ghernautan V, Nso N, Nyabera A, Castillo FC, Tu W, Medina L, Ciobanu C, Alfishawy M, Rizzo V, Eskaros S, Mahdi M, Khalifa M, El-Kassas M
Medicine (Baltimore) 2022 Nov 11;101(45):e31780. doi: 10.1097/MD.0000000000031780. PMID: 36397401Free PMC Article
Scleroderma in Children and Adolescents: Localized Scleroderma and Systemic Sclerosis.
Li SC
Pediatr Clin North Am 2018 Aug;65(4):757-781. doi: 10.1016/j.pcl.2018.04.002. PMID: 30031497
Scleroderma with an update about clinico-pathological correlation.
Rongioletti F, Ferreli C, Atzori L, Bottoni U, Soda G
G Ital Dermatol Venereol 2018 Apr;153(2):208-215. Epub 2018 Jan 24 doi: 10.23736/S0392-0488.18.05922-9. PMID: 29368844
Morphea and Eosinophilic Fasciitis: An Update.
Mertens JS, Seyger MMB, Thurlings RM, Radstake TRDJ, de Jong EMGJ
Am J Clin Dermatol 2017 Aug;18(4):491-512. doi: 10.1007/s40257-017-0269-x. PMID: 28303481Free PMC Article
Localized scleroderma: clinical spectrum and therapeutic update.
Careta MF, Romiti R
An Bras Dermatol 2015 Jan-Feb;90(1):62-73. doi: 10.1590/abd1806-4841.20152890. PMID: 25672301Free PMC Article

Therapy

Clinical Treatment Options in Scleroderma: Recommendations and Comprehensive Review.
Zhao M, Wu J, Wu H, Sawalha AH, Lu Q
Clin Rev Allergy Immunol 2022 Apr;62(2):273-291. Epub 2021 Jan 15 doi: 10.1007/s12016-020-08831-4. PMID: 33449302
Upcoming treatments for morphea.
Wenzel D, Haddadi NS, Afshari K, Richmond JM, Rashighi M
Immun Inflamm Dis 2021 Dec;9(4):1101-1145. Epub 2021 Jul 17 doi: 10.1002/iid3.475. PMID: 34272836Free PMC Article
Scleroderma in Children and Adolescents: Localized Scleroderma and Systemic Sclerosis.
Li SC
Pediatr Clin North Am 2018 Aug;65(4):757-781. doi: 10.1016/j.pcl.2018.04.002. PMID: 30031497
Morphea and Eosinophilic Fasciitis: An Update.
Mertens JS, Seyger MMB, Thurlings RM, Radstake TRDJ, de Jong EMGJ
Am J Clin Dermatol 2017 Aug;18(4):491-512. doi: 10.1007/s40257-017-0269-x. PMID: 28303481Free PMC Article
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

Gastrointestinal involvement in systemic sclerosis: An updated review.
Nassar M, Ghernautan V, Nso N, Nyabera A, Castillo FC, Tu W, Medina L, Ciobanu C, Alfishawy M, Rizzo V, Eskaros S, Mahdi M, Khalifa M, El-Kassas M
Medicine (Baltimore) 2022 Nov 11;101(45):e31780. doi: 10.1097/MD.0000000000031780. PMID: 36397401Free PMC Article
Systemic sclerosis in adults. Part I: Clinical features and pathogenesis.
Jerjen R, Nikpour M, Krieg T, Denton CP, Saracino AM
J Am Acad Dermatol 2022 Nov;87(5):937-954. Epub 2022 Feb 4 doi: 10.1016/j.jaad.2021.10.065. PMID: 35131402
Upcoming treatments for morphea.
Wenzel D, Haddadi NS, Afshari K, Richmond JM, Rashighi M
Immun Inflamm Dis 2021 Dec;9(4):1101-1145. Epub 2021 Jul 17 doi: 10.1002/iid3.475. PMID: 34272836Free PMC Article
Scleroderma in Children and Adolescents: Localized Scleroderma and Systemic Sclerosis.
Li SC
Pediatr Clin North Am 2018 Aug;65(4):757-781. doi: 10.1016/j.pcl.2018.04.002. PMID: 30031497
Cutaneous Manifestations of Scleroderma and Scleroderma-Like Disorders: a Comprehensive Review.
Ferreli C, Gasparini G, Parodi A, Cozzani E, Rongioletti F, Atzori L
Clin Rev Allergy Immunol 2017 Dec;53(3):306-336. doi: 10.1007/s12016-017-8625-4. PMID: 28712039

Clinical prediction guides

Extragenital lichen sclerosus: A comprehensive review.
Arif T, Fatima R, Sami M
Australas J Dermatol 2022 Nov;63(4):452-462. Epub 2022 Aug 11 doi: 10.1111/ajd.13890. PMID: 35950883
Systemic sclerosis in adults. Part I: Clinical features and pathogenesis.
Jerjen R, Nikpour M, Krieg T, Denton CP, Saracino AM
J Am Acad Dermatol 2022 Nov;87(5):937-954. Epub 2022 Feb 4 doi: 10.1016/j.jaad.2021.10.065. PMID: 35131402
Scleroderma in children.
Zulian F
Best Pract Res Clin Rheumatol 2017 Aug;31(4):576-595. Epub 2018 Mar 27 doi: 10.1016/j.berh.2018.02.004. PMID: 29773274
European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: localized scleroderma, systemic sclerosis and overlap syndromes.
Knobler R, Moinzadeh P, Hunzelmann N, Kreuter A, Cozzio A, Mouthon L, Cutolo M, Rongioletti F, Denton CP, Rudnicka L, Frasin LA, Smith V, Gabrielli A, Aberer E, Bagot M, Bali G, Bouaziz J, Braae Olesen A, Foeldvari I, Frances C, Jalili A, Just U, Kähäri V, Kárpáti S, Kofoed K, Krasowska D, Olszewska M, Orteu C, Panelius J, Parodi A, Petit A, Quaglino P, Ranki A, Sanchez Schmidt JM, Seneschal J, Skrok A, Sticherling M, Sunderkötter C, Taieb A, Tanew A, Wolf P, Worm M, Wutte NJ, Krieg T
J Eur Acad Dermatol Venereol 2017 Sep;31(9):1401-1424. Epub 2017 Aug 9 doi: 10.1111/jdv.14458. PMID: 28792092
Localized scleroderma.
Kreuter A
Dermatol Ther 2012 Mar-Apr;25(2):135-47. doi: 10.1111/j.1529-8019.2012.01479.x. PMID: 22741933

Recent systematic reviews

Immunologic underpinnings and treatment of morphea.
LaChance AH, Goldman N, Kassamali B, Vleugels RA
Expert Rev Clin Immunol 2022 May;18(5):461-483. Epub 2022 May 4 doi: 10.1080/1744666X.2022.2063841. PMID: 35403538
Coexistence of systemic and localized scleroderma: a systematic literature review and observational cohort study.
Vanhaecke A, De Schepper S, Paolino S, Heeman L, Callens H, Gutermuth J, Nguyen S, Cutolo M, Smith V
Rheumatology (Oxford) 2020 Oct 1;59(10):2725-2733. doi: 10.1093/rheumatology/keaa297. PMID: 32653905
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
Development of minimum standards of care for juvenile localized scleroderma.
Constantin T, Foeldvari I, Pain CE, Pálinkás A, Höger P, Moll M, Nemkova D, Weibel L, Laczkovszki M, Clements P, Torok KS
Eur J Pediatr 2018 Jul;177(7):961-977. Epub 2018 May 4 doi: 10.1007/s00431-018-3144-8. PMID: 29728839
A systematic review of tools for determining activity of localized scleroderma in paediatric and adult patients.
Lis-Święty A, Janicka I, Skrzypek-Salamon A, Brzezińska-Wcisło L
J Eur Acad Dermatol Venereol 2017 Jan;31(1):30-37. Epub 2016 Jul 12 doi: 10.1111/jdv.13790. PMID: 27401109

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