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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]

Term Hierarchy

GTR
MeSH
Orphanet

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVLocalized scleroderma

Pathological Conditions, Signs and Symptoms
- Pathological process
  - Disease
    - Disorder by Site
      - Connective and Soft Tissue Disorder
        Connective tissue disorder
        Localized scleroderma
        Circumscribed Morphea
        Deep Circumscribed Morphea
        Plaque morphea
        Disabling pansclerotic morphea of childhood
        Generalized morphea
        Linear scleroderma
        Coup de sabre scleroderma
        Mixed Morphea
        Pansclerotic Morphea

Follow this link to review classifications for Localized scleroderma in Orphanet.

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

See all (64)

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: 36397401 Free PMC Article

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: 28303481 Free PMC Article

See all (711)

Diagnosis

Gastrointestinal involvement in systemic sclerosis: An updated review.

Morfea: revisión práctica de su diagnóstico, clasificación y tratamiento.

Rodríguez-Salgado P, García-Romero MT
Gac Med Mex 2019;155(5):522-531. doi: 10.24875/GMM.18004288. PMID: 31695234

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

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: 25672301 Free PMC Article

See all (1148)

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: 34272836 Free 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: 28303481 Free 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

See all (788)

Prognosis

Gastrointestinal involvement in systemic sclerosis: pathogenesis, assessment and treatment.

Volkmann ER, McMahan Z
Curr Opin Rheumatol 2022 Nov 1;34(6):328-336. Epub 2022 Aug 19 doi: 10.1097/BOR.0000000000000899. PMID: 35993874 Free 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: 34272836 Free 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

See all (480)

Clinical prediction guides

Non-invasive imaging and clinical skin scores in juvenile localized scleroderma.

Pain CE, Murray A, Dinsdale G, Marsden A, Manning J, Riley P, Leone V, Amin T, Zulian F, Herrick AL
Rheumatology (Oxford) 2024 May 2;63(5):1332-1340. doi: 10.1093/rheumatology/kead380. PMID: 37531286

Renal involvement in systemic sclerosis.

Scheen M, Dominati A, Olivier V, Nasr S, De Seigneux S, Mekinian A, Issa N, Haidar F
Autoimmun Rev 2023 Jun;22(6):103330. Epub 2023 Apr 7 doi: 10.1016/j.autrev.2023.103330. PMID: 37031831

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

Diagnosis and management of morphoea in children: an overview.

Weibel L
Clin Exp Dermatol 2021 Apr;46(3):487-494. Epub 2021 Mar 10 doi: 10.1111/ced.14562. PMID: 33751628

See all (535)

Recent systematic reviews

Utility of phototherapy in patients with systemic sclerosis: Systematic review.

Miziołek B, Tworek M, Łapczyńska E, Tekielak A, Kochanowska J, Polak K, Bergler-Czop B
Dermatol Ther 2022 Jun;35(6):e15478. Epub 2022 Apr 12 doi: 10.1111/dth.15478. PMID: 35357072

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

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: 31309547 Free PMC Article

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

See all (19)

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Localized scleroderma

Definition

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Recent systematic reviews

Supplemental Content

Table of contents

Clinical resources

Practice guidelines

Consumer resources

Reviews

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