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Limited cutaneous systemic sclerosis

MedGen UID:
148187
•Concept ID:
C0748540
•
Disease or Syndrome
Synonym: Limited scleroderma
SNOMED CT: Limited systemic sclerosis (299276009); Limited scleroderma (299276009); Systemic sclerosis, limited (299276009)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
•Concept ID:
C0600599
•
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Non-Mendelian inheritance (Orphanet)
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0016358
Orphanet: ORPHA220402

Definition

Limited cutaneous systemic sclerosis (lcSSc) is a subtype of systemic sclerosis (SSc; see this term) characterized by the association of Raynaud's phenomenon with skin fibrosis limited to the hands, face, feet and forearms. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLimited cutaneous systemic sclerosis

Professional guidelines

PubMed

Management of Systemic Sclerosis-Related Skin Disease: A Review of Existing and Experimental Therapeutic Approaches.
Volkmann ER, Furst DE
Rheum Dis Clin North Am 2015 Aug;41(3):399-417. Epub 2015 May 20 doi: 10.1016/j.rdc.2015.04.004. PMID: 26210126
Treatment of complications associated with systemic sclerosis.
Moore SC, Desantis ER
Am J Health Syst Pharm 2008 Feb 15;65(4):315-21. doi: 10.2146/ajhp070024. PMID: 18238768
Treatment of cutaneous calcinosis in limited systemic sclerosis with minocycline.
Robertson LP, Marshall RW, Hickling P
Ann Rheum Dis 2003 Mar;62(3):267-9. doi: 10.1136/ard.62.3.267. PMID: 12594118Free PMC Article

Recent clinical studies

Etiology

Systemic sclerosis and primary biliary cholangitis share an antibody population with identical specificity.
Favoino E, Grapsi E, Barbuti G, Liakouli V, Ruscitti P, Foti C, Giacomelli R, Perosa F
Clin Exp Immunol 2023 Apr 7;212(1):32-38. doi: 10.1093/cei/uxad012. PMID: 36715304Free PMC Article
Therapeutic Approaches to Systemic Sclerosis: Recent Approvals and Future Candidate Therapies.
Lescoat A, Roofeh D, Kuwana M, Lafyatis R, Allanore Y, Khanna D
Clin Rev Allergy Immunol 2023 Jun;64(3):239-261. Epub 2021 Sep 1 doi: 10.1007/s12016-021-08891-0. PMID: 34468946Free PMC Article
Symptom experience of limited cutaneous systemic sclerosis from the Patients' perspective: A qualitative study(✰,✰✰,★,★★).
Lescoat A, Murphy SL, Chen YT, Vann N, Galdo FD, Cella D, Buch MH, Khanna D
Semin Arthritis Rheum 2022 Feb;52:151926. Epub 2021 Nov 7 doi: 10.1016/j.semarthrit.2021.11.003. PMID: 34785028Free PMC Article
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
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

Cutaneous Features, Autoantibody Profile, and Nailfold Capillaroscopy of Systemic Sclerosis: A Study of 60 Cases.
Dave J, Mahajan S, Khadilkar P, Pradhan V
J Assoc Physicians India 2022 Nov;70(11):11-12. doi: 10.5005/japi-11001-0136. PMID: 37355940
Symptom experience of limited cutaneous systemic sclerosis from the Patients' perspective: A qualitative study(✰,✰✰,★,★★).
Lescoat A, Murphy SL, Chen YT, Vann N, Galdo FD, Cella D, Buch MH, Khanna D
Semin Arthritis Rheum 2022 Feb;52:151926. Epub 2021 Nov 7 doi: 10.1016/j.semarthrit.2021.11.003. PMID: 34785028Free PMC Article
The burden of systemic sclerosis in Switzerland - the Swiss systemic sclerosis EUSTAR cohort.
Hernández J, Jordan S, Dobrota R, Iudici M, Hasler P, Ribi C, Villiger P, Vlachoyiannopoulos P, Vacca A, Garzanova L, Giollo A, Rosato E, Kötter I, Carreira PE, Doria A, Henes J, Müller-Ladner U, Smith V, Distler J, Gabrielli A, Hoffman-Vold AM, Walker U, Distler O; The Eustar Collaborators
Swiss Med Wkly 2021 Jul 5;151:w20528. Epub 2021 Jul 14 doi: 10.4414/smw.2021.20528. PMID: 34283895
Systemic Sclerosis and Pulmonary Disease.
Ngo K
Adv Exp Med Biol 2021;1303:173-182. doi: 10.1007/978-3-030-63046-1_10. PMID: 33788193
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

Therapy

Effects of nintedanib in patients with limited cutaneous systemic sclerosis and interstitial lung disease.
Allanore Y, Khanna D, Smith V, Aringer M, Hoffmann-Vold AM, Kuwana M, Merkel PA, Stock C, Sambevski S, Denton CP; SENSCIS Trial Investigators
Rheumatology (Oxford) 2024 Mar 1;63(3):639-647. doi: 10.1093/rheumatology/kead280. PMID: 37294870Free PMC Article
Domains and outcome measures for the assessment of limited cutaneous systemic sclerosis: a scoping review protocol.
Lescoat A, Roofeh D, Townsend W, Hughes M, Sandler RD, Zimmermann F, Pauling JD, Buch MH, Khanna D
BMJ Open 2021 Mar 11;11(3):e044765. doi: 10.1136/bmjopen-2020-044765. PMID: 33707273Free PMC Article
Lymphedema secondary to limited cutaneous systemic sclerosis.
Moreira S, Crespo J, Santos L
BMJ Case Rep 2018 Jun 15;2018 doi: 10.1136/bcr-2017-224148. PMID: 29909388Free PMC Article
Management of Systemic Sclerosis-Related Skin Disease: A Review of Existing and Experimental Therapeutic Approaches.
Volkmann ER, Furst DE
Rheum Dis Clin North Am 2015 Aug;41(3):399-417. Epub 2015 May 20 doi: 10.1016/j.rdc.2015.04.004. PMID: 26210126
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

Prognosis

Effects of nintedanib in patients with limited cutaneous systemic sclerosis and interstitial lung disease.
Allanore Y, Khanna D, Smith V, Aringer M, Hoffmann-Vold AM, Kuwana M, Merkel PA, Stock C, Sambevski S, Denton CP; SENSCIS Trial Investigators
Rheumatology (Oxford) 2024 Mar 1;63(3):639-647. doi: 10.1093/rheumatology/kead280. PMID: 37294870Free PMC Article
Cutaneous Features, Autoantibody Profile, and Nailfold Capillaroscopy of Systemic Sclerosis: A Study of 60 Cases.
Dave J, Mahajan S, Khadilkar P, Pradhan V
J Assoc Physicians India 2022 Nov;70(11):11-12. doi: 10.5005/japi-11001-0136. PMID: 37355940
Systemic Sclerosis and Pulmonary Disease.
Ngo K
Adv Exp Med Biol 2021;1303:173-182. doi: 10.1007/978-3-030-63046-1_10. PMID: 33788193
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
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

Clinical prediction guides

Cutaneous Features, Autoantibody Profile, and Nailfold Capillaroscopy of Systemic Sclerosis: A Study of 60 Cases.
Dave J, Mahajan S, Khadilkar P, Pradhan V
J Assoc Physicians India 2022 Nov;70(11):11-12. doi: 10.5005/japi-11001-0136. PMID: 37355940
The burden of systemic sclerosis in Switzerland - the Swiss systemic sclerosis EUSTAR cohort.
Hernández J, Jordan S, Dobrota R, Iudici M, Hasler P, Ribi C, Villiger P, Vlachoyiannopoulos P, Vacca A, Garzanova L, Giollo A, Rosato E, Kötter I, Carreira PE, Doria A, Henes J, Müller-Ladner U, Smith V, Distler J, Gabrielli A, Hoffman-Vold AM, Walker U, Distler O; The Eustar Collaborators
Swiss Med Wkly 2021 Jul 5;151:w20528. Epub 2021 Jul 14 doi: 10.4414/smw.2021.20528. PMID: 34283895
Systemic Sclerosis and Pulmonary Disease.
Ngo K
Adv Exp Med Biol 2021;1303:173-182. doi: 10.1007/978-3-030-63046-1_10. PMID: 33788193
Outcomes of limited cutaneous systemic sclerosis patients: Results on more than 12,000 patients from the EUSTAR database.
Frantz C, Huscher D, Avouac J, Hachulla E, Balbir-Gurman A, Riemekasten G, Siegert E, Lazzaroni MG, Carreira PE, Vettori S, Zanatta E, Ullman S, Czirjàk L, Kowal-Bielecka O, Distler O, Matucci-Cerinic M, Allanore Y; EUSTAR co-authors
Autoimmun Rev 2020 Feb;19(2):102452. Epub 2019 Dec 12 doi: 10.1016/j.autrev.2019.102452. PMID: 31838157
Management of Systemic Sclerosis-Related Skin Disease: A Review of Existing and Experimental Therapeutic Approaches.
Volkmann ER, Furst DE
Rheum Dis Clin North Am 2015 Aug;41(3):399-417. Epub 2015 May 20 doi: 10.1016/j.rdc.2015.04.004. PMID: 26210126

Recent systematic reviews

Pauci-immune crescentic glomerulonephritis in limited cutaneous systemic sclerosis.
Chan PT, Mok CC
Clin Rheumatol 2012 Aug;31(8):1273-7. Epub 2012 May 30 doi: 10.1007/s10067-012-2010-8. PMID: 22644090

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