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Pansclerotic Morphea

MedGen UID:
857586
•Concept ID:
C3897992
•
Disease or Syndrome

Definition

A rare, aggressive form of morphea characterized by sclerosis of the dermis, fascia, and muscle over large parts of the body, resulting in contractures and immobility. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPansclerotic Morphea

Professional guidelines

PubMed

Treatment in Juvenile Scleroderma.
Zulian F, Tirelli F
Curr Rheumatol Rep 2020 Jun 26;22(8):45. doi: 10.1007/s11926-020-00910-x. PMID: 32591919
Evaluation of the Effectiveness and Tolerability of Mycophenolate Mofetil and Mycophenolic Acid for the Treatment of Morphea.
Arthur M, Fett NM, Latour E, Jacobe H, Kunzler E, Florez-Pollack S, Houser J, Sharma S, Prasad S, Femia A, Stern MJ, Pappas-Taffer LK, Gaffney R, Fernandez AP, Knabel D, Cardones AR, Leung N, Laumann A, Yu JM, Zhao J, Vleugels RA, Tkachenko E, Lo K
JAMA Dermatol 2020 May 1;156(5):521-528. doi: 10.1001/jamadermatol.2020.0035. PMID: 32236497Free 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

Recent clinical studies

Etiology

An Evaluation of the Performance of Current Morphea Subtype Classifications.
Prasad S, Zhu JL, Schollaert-Fitch K, Torok KS, Jacobe HT
JAMA Dermatol 2021 Apr 1;157(4):1-8. doi: 10.1001/jamadermatol.2020.5809. PMID: 33595618Free PMC Article
Mycophenolate mofetil for methotrexate-resistant juvenile localized scleroderma.
Martini G, Saggioro L, Culpo R, Vittadello F, Meneghel A, Zulian F
Rheumatology (Oxford) 2021 Mar 2;60(3):1387-1391. doi: 10.1093/rheumatology/keaa392. PMID: 32978631Free PMC Article
Treatment in Juvenile Scleroderma.
Zulian F, Tirelli F
Curr Rheumatol Rep 2020 Jun 26;22(8):45. doi: 10.1007/s11926-020-00910-x. PMID: 32591919
Disabling pansclerotic morphea of childhood poses a high risk of chronic ulceration of the skin and squamous cell carcinoma.
Wollina U, Buslau M, Heinig B, Petrov I, Unger E, Kyriopoulou E, Koch A, Köstler E, Schönlebe J, Haroske G, Doede T, Pramatarov K
Int J Low Extrem Wounds 2007 Dec;6(4):291-8. doi: 10.1177/1534734607308731. PMID: 18048875
Deep morphea.
Bielsa I, Ariza A
Semin Cutan Med Surg 2007 Jun;26(2):90-5. doi: 10.1016/j.sder.2007.02.005. PMID: 17544960

Diagnosis

Case Report: Pansclerotic Morphea-Clinical Features, Differential Diagnoses and Modern Treatment Concepts.
Ventéjou S, Schwieger-Briel A, Nicolai R, Christen-Zaech S, Schnider C, Hofer M, Bogiatzi S, Hohl D, De Benedetti F, Morren MA
Front Immunol 2021;12:656407. Epub 2021 Mar 9 doi: 10.3389/fimmu.2021.656407. PMID: 33767715Free PMC Article
An Evaluation of the Performance of Current Morphea Subtype Classifications.
Prasad S, Zhu JL, Schollaert-Fitch K, Torok KS, Jacobe HT
JAMA Dermatol 2021 Apr 1;157(4):1-8. doi: 10.1001/jamadermatol.2020.5809. PMID: 33595618Free PMC Article
Challenges in the diagnosis and treatment of disabling pansclerotic morphea of childhood: case-based review.
Soh HJ, Samuel C, Heaton V, Renton WD, Cox A, Munro J
Rheumatol Int 2019 May;39(5):933-941. Epub 2019 Mar 5 doi: 10.1007/s00296-019-04269-w. PMID: 30838436
Deep morphea.
Bielsa I, Ariza A
Semin Cutan Med Surg 2007 Jun;26(2):90-5. doi: 10.1016/j.sder.2007.02.005. PMID: 17544960
Eosinophilic fasciitis.
Doyle JA, Ginsburg WW
Med Clin North Am 1989 Sep;73(5):1157-66. doi: 10.1016/s0025-7125(16)30625-3. PMID: 2671538

Therapy

Case Report: Pansclerotic Morphea-Clinical Features, Differential Diagnoses and Modern Treatment Concepts.
Ventéjou S, Schwieger-Briel A, Nicolai R, Christen-Zaech S, Schnider C, Hofer M, Bogiatzi S, Hohl D, De Benedetti F, Morren MA
Front Immunol 2021;12:656407. Epub 2021 Mar 9 doi: 10.3389/fimmu.2021.656407. PMID: 33767715Free PMC Article
Treatment in Juvenile Scleroderma.
Zulian F, Tirelli F
Curr Rheumatol Rep 2020 Jun 26;22(8):45. doi: 10.1007/s11926-020-00910-x. PMID: 32591919
Challenges in the diagnosis and treatment of disabling pansclerotic morphea of childhood: case-based review.
Soh HJ, Samuel C, Heaton V, Renton WD, Cox A, Munro J
Rheumatol Int 2019 May;39(5):933-941. Epub 2019 Mar 5 doi: 10.1007/s00296-019-04269-w. PMID: 30838436
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
New developments in localized scleroderma.
Zulian F
Curr Opin Rheumatol 2008 Sep;20(5):601-7. doi: 10.1097/BOR.0b013e328309a5eb. PMID: 18698185

Prognosis

Mycophenolate mofetil for methotrexate-resistant juvenile localized scleroderma.
Martini G, Saggioro L, Culpo R, Vittadello F, Meneghel A, Zulian F
Rheumatology (Oxford) 2021 Mar 2;60(3):1387-1391. doi: 10.1093/rheumatology/keaa392. PMID: 32978631Free PMC Article
Challenges in the diagnosis and treatment of disabling pansclerotic morphea of childhood: case-based review.
Soh HJ, Samuel C, Heaton V, Renton WD, Cox A, Munro J
Rheumatol Int 2019 May;39(5):933-941. Epub 2019 Mar 5 doi: 10.1007/s00296-019-04269-w. PMID: 30838436
Clinical features of patients with morphea and the pansclerotic subtype: a cross-sectional study from the morphea in adults and children cohort.
Kim A, Marinkovich N, Vasquez R, Jacobe HT
J Rheumatol 2014 Jan;41(1):106-12. Epub 2013 Dec 1 doi: 10.3899/jrheum.130029. PMID: 24293577Free PMC Article
Disabling pansclerotic morphea of childhood--unusual case and management challenges.
Forsea AM, Cretu AN, Ionescu R, Giurcaneanu C
J Med Life 2008 Jul-Sep;1(3):348-54. PMID: 20108512Free PMC Article
Disabling pansclerotic morphea of childhood poses a high risk of chronic ulceration of the skin and squamous cell carcinoma.
Wollina U, Buslau M, Heinig B, Petrov I, Unger E, Kyriopoulou E, Koch A, Köstler E, Schönlebe J, Haroske G, Doede T, Pramatarov K
Int J Low Extrem Wounds 2007 Dec;6(4):291-8. doi: 10.1177/1534734607308731. PMID: 18048875

Clinical prediction guides

An Evaluation of the Performance of Current Morphea Subtype Classifications.
Prasad S, Zhu JL, Schollaert-Fitch K, Torok KS, Jacobe HT
JAMA Dermatol 2021 Apr 1;157(4):1-8. doi: 10.1001/jamadermatol.2020.5809. PMID: 33595618Free PMC Article
Mycophenolate mofetil for methotrexate-resistant juvenile localized scleroderma.
Martini G, Saggioro L, Culpo R, Vittadello F, Meneghel A, Zulian F
Rheumatology (Oxford) 2021 Mar 2;60(3):1387-1391. doi: 10.1093/rheumatology/keaa392. PMID: 32978631Free PMC Article
Treatment in Juvenile Scleroderma.
Zulian F, Tirelli F
Curr Rheumatol Rep 2020 Jun 26;22(8):45. doi: 10.1007/s11926-020-00910-x. PMID: 32591919
Clinical features of patients with morphea and the pansclerotic subtype: a cross-sectional study from the morphea in adults and children cohort.
Kim A, Marinkovich N, Vasquez R, Jacobe HT
J Rheumatol 2014 Jan;41(1):106-12. Epub 2013 Dec 1 doi: 10.3899/jrheum.130029. PMID: 24293577Free PMC Article
Disabling pansclerotic morphea of children.
Diaz-Perez JL, Connolly SM, Winkelmann RK
Arch Dermatol 1980 Feb;116(2):169-73. PMID: 7356347

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