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Generalized morphea

MedGen UID:
538045
•Concept ID:
C0263664
•
Disease or Syndrome
Synonyms: Circumscribed scleroderma, generalized type; Generalized Morphea; Generalized scleroderma
SNOMED CT: Generalized scleroderma (7513007); Circumscribed scleroderma, generalized type (7513007); Generalized morphea (7513007)

Definition

A type of morphea characterized by four or more plaques found in two or more anatomic locations. The plaques are indurated, generally well-delineated, and may include muscle atrophy in affected areas; there is no visceral involvement. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVGeneralized morphea

Professional guidelines

PubMed

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
Morphea in adults and children cohort II: patients with morphea experience delay in diagnosis and large variation in treatment.
Johnson W, Jacobe H
J Am Acad Dermatol 2012 Nov;67(5):881-9. Epub 2012 Feb 29 doi: 10.1016/j.jaad.2012.01.011. PMID: 22382198

Recent clinical studies

Etiology

Body site distribution of pediatric-onset morphea and association with extracutaneous manifestations.
Chiu YE, Shmuylovich L, Kiguradze T, Anderson K, Sibbald C, Tollefson M, Kunzler E, Tom WL, Bond K, Ahmad RC, Garcia-Romero MT, Irfan M, Kollman K, Hunt R, Stein SL, Arkin L, Wong V, Pope E, Jacobe H, Brandling-Bennett HA, Cordoro KM, Bercovitch L, Rangel SM, Liu X, Szabo A, Paller AS
J Am Acad Dermatol 2021 Jul;85(1):38-45. Epub 2021 Mar 6 doi: 10.1016/j.jaad.2021.03.017. PMID: 33689776
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
Comorbidity of localized scleroderma and primary biliary cholangitis.
Bali G, Szilvási A, Inotai D, Varga Á, Sárdy M, Kárpáti S, Medvecz M, Szegedi A, Hidvégi B
J Dtsch Dermatol Ges 2018 Nov;16(11):1323-1327. doi: 10.1111/ddg.13693. PMID: 30395408
Skin mapping for the classification of generalized morphea.
Teske N, Welser J, Jacobe H
J Am Acad Dermatol 2018 Feb;78(2):351-357. doi: 10.1016/j.jaad.2016.08.052. PMID: 29332706
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

Immunologic and nonimmunologic sclerodermal skin conditions - review.
Bobeica C, Niculet E, Craescu M, Parapiru EL, Corduneanu-Luca AM, Debita M, Pelin AM, Tiutiuca C, Vasile CI, Nicolescu AC, Miulescu M, Balan G, Tatu AL
Front Immunol 2023;14:1180221. Epub 2023 Jul 12 doi: 10.3389/fimmu.2023.1180221. PMID: 37600771Free PMC Article
RF - Generalized Morphea: Definition and Associations.
García-Vázquez A, Guillen-Climent S, Ramón Quiles MD
Actas Dermosifiliogr (Engl Ed) 2021 Apr;112(4):366-367. Epub 2020 Nov 20 doi: 10.1016/j.ad.2019.05.021. PMID: 33227275
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
Generalized morphea.
Gordon M, Lebwohl M
Mt Sinai J Med 1998 Sep;65(4):308. PMID: 9757755
Localized scleroderma.
Tuffanelli DL
Semin Cutan Med Surg 1998 Mar;17(1):27-33. doi: 10.1016/s1085-5629(98)80059-x. PMID: 9512104

Therapy

Generalized morphea treated with secukinumab.
Filoni A, Bonamonte D, Civino A, LA Torre F, Congedo M
Ital J Dermatol Venerol 2023 Aug;158(4):368-369. doi: 10.23736/S2784-8671.23.07502-3. PMID: 37539507
Tocilizumab for Corticosteroid-Refractory Immune Checkpoint Inhibitor-Induced Generalized Morphea.
Blaise M, Cardot-Leccia N, Seitz-Polski B, Picard-Gauci A, Bertold C, Passeron T, Montaudié H
JAMA Dermatol 2023 Jan 1;159(1):112-114. doi: 10.1001/jamadermatol.2022.5146. PMID: 36449277
A case of eosinophilic fasciitis and generalized morphea overlap.
Watanabe Y, Yamamoto M, Yamamoto T
Dermatol Online J 2020 Feb 15;26(2) PMID: 32239898
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
Localized and systemic scleroderma.
Hawk A, English JC 3rd
Semin Cutan Med Surg 2001 Mar;20(1):27-37. doi: 10.1053/sder.2001.23093. PMID: 11308134

Prognosis

Eosinophilic fasciitis associated with generalized morphea and IgA nephropathy.
Luo G, Wan Y, Wang X, Zhang J, Deng X
Dermatol Ther 2020 Jul;33(4):e13641. Epub 2020 Jun 20 doi: 10.1111/dth.13641. PMID: 32441406
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
Eosinophilic fasciitis/generalized morphea overlap.
Heidary N, Cheung W, Wang N, Kamino H, Franks AG Jr
Dermatol Online J 2009 Aug 15;15(8):2. PMID: 19891910
Localized and systemic scleroderma.
Hawk A, English JC 3rd
Semin Cutan Med Surg 2001 Mar;20(1):27-37. doi: 10.1053/sder.2001.23093. PMID: 11308134
Localized scleroderma.
Tuffanelli DL
Semin Cutan Med Surg 1998 Mar;17(1):27-33. doi: 10.1016/s1085-5629(98)80059-x. PMID: 9512104

Clinical prediction guides

Response of recalcitrant generalized morphea to intravenous immunoglobulins (IVIg): three cases and a review of the literature.
Kromer C, Mitterlechner L, Langer N, Schön MP, Mössner R
Eur J Dermatol 2021 Dec 1;31(6):822-829. doi: 10.1684/ejd.2021.4173. PMID: 35107073
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
A case of radiation-induced generalized morphea with prominent mucin deposition and tenderness.
Yanaba K, Umezawa Y, Nakagawa H
Am J Case Rep 2015 May 10;16:279-82. doi: 10.12659/AJCR.893481. PMID: 25958415Free PMC Article
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
Antinuclear and anti-single-stranded DNA antibodies in morphea and generalized morphea.
Falanga V, Medsger TA Jr, Reichlin M
Arch Dermatol 1987 Mar;123(3):350-3. PMID: 3545090

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