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Morphea

MedGen UID:
315951
Concept ID:
C1527383
Disease or Syndrome
Synonym: Morpheas
SNOMED CT: Morphea (201049004)
 
HPO: HP:0012344

Definition

Isolated patches of hardened skin (scleroderma). [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMorphea

Conditions with this feature

Disabling pansclerotic morphea of childhood
MedGen UID:
858243
Concept ID:
C3898649
Disease or Syndrome
Disabling pansclerotic morphea of childhood is the most severe subtype of deep morphea within the spectrum of juvenile localized scleroderma. Patients affected by this systemic inflammatory disorder experience poor wound healing with rapidly progressive deep fibrosis involving the mucous membranes, dermis, subcutaneous fat, fascia, muscles, and bone, leading to contractures, musculoskeletal atrophy, and articular ankylosis. Systemic manifestations include cytopenias and hypogammaglobulinemia, but scleroderma-associated autoantibodies are usually not present. The disorder is associated with high morbidity and mortality due to squamous cell carcinoma, restrictive pulmonary disease, sepsis, and gangrene (Baghdassarian et al., 2023).

Professional guidelines

PubMed

McGaugh S, Kallis P, De Benedetto A, Thomas RM
Dermatol Ther 2022 Jun;35(6):e15437. Epub 2022 Mar 22 doi: 10.1111/dth.15437. PMID: 35278019
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
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

Zulian F, Tirelli F
Curr Rheumatol Rep 2020 Jun 26;22(8):45. doi: 10.1007/s11926-020-00910-x. PMID: 32591919
Li SC
Pediatr Clin North Am 2018 Aug;65(4):757-781. doi: 10.1016/j.pcl.2018.04.002. PMID: 30031497
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
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: 28303481Free PMC Article
Nashel J, Steen V
Curr Rheumatol Rep 2012 Feb;14(1):39-46. doi: 10.1007/s11926-011-0220-8. PMID: 22131103

Diagnosis

Rodríguez-Salgado P, García-Romero MT
Gac Med Mex 2019;155(5):522-531. doi: 10.24875/GMM.18004288. PMID: 31695234
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
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: 28303481Free PMC Article
Wick MR
Semin Diagn Pathol 2017 May;34(3):261-272. Epub 2016 Dec 27 doi: 10.1053/j.semdp.2016.12.004. PMID: 28129926
Careta MF, Romiti R
An Bras Dermatol 2015 Jan-Feb;90(1):62-73. doi: 10.1590/abd1806-4841.20152890. PMID: 25672301Free PMC Article

Therapy

Bagherani N, Smoller BR, Tavoosidana G, Ghanadan A, Wollina U, Lotti T
J Cosmet Dermatol 2023 Sep;22(9):2399-2407. Epub 2023 Mar 31 doi: 10.1111/jocd.15741. PMID: 36999460
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
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: 34272836Free PMC Article
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: 28303481Free 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

Prognosis

Foeldvari I, Marrani E
Expert Rev Clin Immunol 2023 Jul-Dec;19(10):1225-1238. Epub 2023 Jul 26 doi: 10.1080/1744666X.2023.2237685. PMID: 37462119
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: 34272836Free PMC Article
Li SC
Pediatr Clin North Am 2018 Aug;65(4):757-781. doi: 10.1016/j.pcl.2018.04.002. PMID: 30031497
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
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

Clinical prediction guides

Knobler R, Geroldinger-Simić M, Kreuter A, Hunzelmann N, Moinzadeh P, Rongioletti F, Denton CP, Mouthon L, Cutolo M, Smith V, Gabrielli A, Bagot M, Olesen AB, Foeldvari I, Jalili A, Kähäri V, Kárpáti S, Kofoed K, Olszewska M, Panelius J, Quaglino P, Seneschal J, Sticherling M, Sunderkötter C, Tanew A, Wolf P, Worm M, Skrok A, Rudnicka L, Krieg T
J Eur Acad Dermatol Venereol 2024 Jul;38(7):1251-1280. Epub 2024 Mar 8 doi: 10.1111/jdv.19912. PMID: 38456584
Speeckaert R, Lambert J, Bulat V, Belpaire A, Speeckaert M, van Geel N
Front Immunol 2020;11:568447. Epub 2020 Oct 27 doi: 10.3389/fimmu.2020.568447. PMID: 33193342Free PMC Article
Zulian F, Tirelli F
Curr Rheumatol Rep 2020 Jun 26;22(8):45. doi: 10.1007/s11926-020-00910-x. PMID: 32591919
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
Kreuter A
Dermatol Ther 2012 Mar-Apr;25(2):135-47. doi: 10.1111/j.1529-8019.2012.01479.x. PMID: 22741933

Recent systematic reviews

Sanchez-Espino LF, Luca N, Pope E, Laxer RM, Knight AM, Sibbald C
Arthritis Care Res (Hoboken) 2024 Mar;76(3):340-349. Epub 2024 Jan 7 doi: 10.1002/acr.25243. PMID: 37750181
Sanchez I, Ibraheim MK, Lee BA, Kraus CN, Elsensohn A
Am J Dermatopathol 2023 Dec 1;45(12):820-821. doi: 10.1097/DAD.0000000000002550. PMID: 37883982
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
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: 31309547Free PMC Article
Zwischenberger BA, Jacobe HT
J Am Acad Dermatol 2011 Nov;65(5):925-41. Epub 2011 Jun 8 doi: 10.1016/j.jaad.2010.09.006. PMID: 21645943

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