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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

S2k guideline: Diagnosis and therapy of localized scleroderma.

Kreuter A, Moinzadeh P, Kinberger M, Horneff G, Worm M, Werner RN, Hammacher A, Krieg T, Wenzel J, Oeschger M, Weibel L, Müllegger R, Hunzelmann N
J Dtsch Dermatol Ges 2024 Apr;22(4):605-620. Epub 2024 Mar 1 doi: 10.1111/ddg.15328. PMID: 38426689

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

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 (65)

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 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

Systemic and localized scleroderma.

Chung L, Lin J, Furst DE, Fiorentino D
Clin Dermatol 2006 Sep-Oct;24(5):374-92. doi: 10.1016/j.clindermatol.2006.07.004. PMID: 16966019

See all (725)

Diagnosis

Nodular (keloidal) scleroderma.

Sanchez NG, Rebollo Domínguez N, Martínez Luna E, Villaseñor Ovies P
Reumatol Clin (Engl Ed) 2023 Oct;19(8):463-464. doi: 10.1016/j.reumae.2023.02.009. PMID: 37805259

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

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

Lipodermatosclerosis.

Miteva M, Romanelli P, Kirsner RS
Dermatol Ther 2010 Jul-Aug;23(4):375-88. doi: 10.1111/j.1529-8019.2010.01338.x. PMID: 20666825

See all (1169)

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

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

Lipodermatosclerosis.

Miteva M, Romanelli P, Kirsner RS
Dermatol Ther 2010 Jul-Aug;23(4):375-88. doi: 10.1111/j.1529-8019.2010.01338.x. PMID: 20666825

See all (799)

Prognosis

Systemic sclerosis associated myopathy.

Chaigne B, Léonard-Louis S, Mouthon L
Autoimmun Rev 2023 Feb;22(2):103261. Epub 2022 Dec 20 doi: 10.1016/j.autrev.2022.103261. PMID: 36549354

Gastrointestinal involvement in systemic sclerosis: An updated review.

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 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 (489)

Clinical prediction guides

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

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

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

Consensus-based recommendations for the management of juvenile systemic sclerosis.

Foeldvari I, Culpo R, Sperotto F, Anton J, Avcin T, Baildam E, Boros C, Chaitow J, Constantin T, Kasapcopur O, Knupp Feitosa de Oliveira S, Pilkington C, Toplak N, van Royen A, Saad Magalhaes C, Vastert SJ, Wulffraat N, Zulian F
Rheumatology (Oxford) 2021 Apr 6;60(4):1651-1658. doi: 10.1093/rheumatology/keaa584. PMID: 33147624

See all (543)

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

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

Development of morphea in patients receiving biologic therapies: A systematic review.

Maliyar K, Mufti A, Sachdeva M, Lytvyn Y, Salsberg J, Yeung J
J Am Acad Dermatol 2021 Apr;84(4):1081-1085. Epub 2020 Jul 17 doi: 10.1016/j.jaad.2020.06.1027. PMID: 32687966

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

Body Image in Children with Morphea: A Systematic Review.

Mills SD, Fox RS, Malcarne VL
Pediatr Dermatol 2015 Jul-Aug;32(4):e188-90. Epub 2015 May 13 doi: 10.1111/pde.12611. PMID: 25968244

See all (20)

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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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