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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
Morphea: a practical review of its diagnosis, classification and treatment.
Rodríguez-Salgado P, García-Romero MT
Gac Med Mex 2019;155(5):483-491. doi: 10.24875/GMM.M20000336. PMID: 32091025
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

Scleroderma of the Hand: Evaluation and Treatment.
Beldner S, Rabinovich RV, Polatsch DB
J Am Acad Orthop Surg 2020 Aug 15;28(16):e686-e695. doi: 10.5435/JAAOS-D-19-00547. PMID: 32769717
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: nomenclature, etiology, pathogenesis, prognosis, and treatments: facts and controversies.
Fett N
Clin Dermatol 2013 Jul-Aug;31(4):432-437. doi: 10.1016/j.clindermatol.2013.01.010. PMID: 23806160

Diagnosis

Morphea: Current concepts.
Florez-Pollack S, Kunzler E, Jacobe HT
Clin Dermatol 2018 Jul-Aug;36(4):475-486. Epub 2018 Apr 10 doi: 10.1016/j.clindermatol.2018.04.005. PMID: 30047431
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
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
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
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
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

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 epidemiology update.
Calderon LM, Pope JE
Curr Opin Rheumatol 2021 Mar 1;33(2):122-127. doi: 10.1097/BOR.0000000000000785. PMID: 33481429
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
Systemic sclerosis: Current concepts of skin and systemic manifestations.
Pearson DR, Werth VP, Pappas-Taffer L
Clin Dermatol 2018 Jul-Aug;36(4):459-474. Epub 2018 Apr 12 doi: 10.1016/j.clindermatol.2018.04.004. PMID: 30047430
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
Neurologic involvement in scleroderma: a systematic review.
Amaral TN, Peres FA, Lapa AT, Marques-Neto JF, Appenzeller S
Semin Arthritis Rheum 2013 Dec;43(3):335-47. Epub 2013 Jul 1 doi: 10.1016/j.semarthrit.2013.05.002. PMID: 23827688

Recent systematic reviews

Standardised interpretation of capillaroscopy in autoimmune idiopathic inflammatory myopathies: A structured review on behalf of the EULAR study group on microcirculation in Rheumatic Diseases.
Piette Y, Reynaert V, Vanhaecke A, Bonroy C, Gutermuth J, Sulli A, Cutolo M, Smith V
Autoimmun Rev 2022 Jun;21(6):103087. Epub 2022 Apr 12 doi: 10.1016/j.autrev.2022.103087. PMID: 35421608
Efficacy and safety of rituximab therapy in patients with systemic sclerosis disease (SSc): systematic review and meta-analysis.
Moradzadeh M, Aghaei M, Mehrbakhsh Z, Arab-Bafrani Z, Abdollahi N
Clin Rheumatol 2021 Oct;40(10):3897-3918. Epub 2021 Apr 2 doi: 10.1007/s10067-021-05698-4. PMID: 33796953
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
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
Neurologic involvement in scleroderma: a systematic review.
Amaral TN, Peres FA, Lapa AT, Marques-Neto JF, Appenzeller S
Semin Arthritis Rheum 2013 Dec;43(3):335-47. Epub 2013 Jul 1 doi: 10.1016/j.semarthrit.2013.05.002. PMID: 23827688

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