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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

Zulian F, Tirelli F
Curr Rheumatol Rep 2020 Jun 26;22(8):45. doi: 10.1007/s11926-020-00910-x. PMID: 32591919
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
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

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
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
Zulian F, Tirelli F
Curr Rheumatol Rep 2020 Jun 26;22(8):45. doi: 10.1007/s11926-020-00910-x. PMID: 32591919
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
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

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
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
Bielsa I, Ariza A
Semin Cutan Med Surg 2007 Jun;26(2):90-5. doi: 10.1016/j.sder.2007.02.005. PMID: 17544960
Doyle JA, Ginsburg WW
Med Clin North Am 1989 Sep;73(5):1157-66. doi: 10.1016/s0025-7125(16)30625-3. PMID: 2671538
Diaz-Perez JL, Connolly SM, Winkelmann RK
Arch Dermatol 1980 Feb;116(2):169-73. PMID: 7356347

Therapy

Zulian F, Tirelli F
Curr Rheumatol Rep 2020 Jun 26;22(8):45. doi: 10.1007/s11926-020-00910-x. PMID: 32591919
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
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
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
Zulian F
Curr Opin Rheumatol 2008 Sep;20(5):601-7. doi: 10.1097/BOR.0b013e328309a5eb. PMID: 18698185

Prognosis

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
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
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
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
Padmavathy L, Rao LL
Indian J Dermatol Venereol Leprol 2005 May-Jun;71(3):192-4. doi: 10.4103/0378-6323.16237. PMID: 16394411

Clinical prediction guides

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
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
Zulian F, Tirelli F
Curr Rheumatol Rep 2020 Jun 26;22(8):45. doi: 10.1007/s11926-020-00910-x. PMID: 32591919
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
Diaz-Perez JL, Connolly SM, Winkelmann RK
Arch Dermatol 1980 Feb;116(2):169-73. PMID: 7356347

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