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Fasciitis with eosinophilia syndrome(EF)

MedGen UID:
82673
•Concept ID:
C0264005
•
Disease or Syndrome
Synonyms: EF; Eosinophilic fasciitis; Shulman syndrome
SNOMED CT: Fasciitis with eosinophilia syndrome (24129002); Shulman's syndrome (24129002); Eosinophilic fasciitis (24129002)
Modes of inheritance:
Unknown inheritance
MedGen UID:
989040
•Concept ID:
CN307042
•
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
See: This record
Unknown inheritance (Orphanet)
 
HPO: HP:0045029
Monarch Initiative: MONDO:0009175
OMIM®: 226350
Orphanet: ORPHA3165

Definition

Inflammation and thickening (localized fibrosis) of the fascia, the tissue under the skin and over the muscle, typically associated with a build up of eosinophils in the muscles and tissues. [from HPO]

Clinical features

From HPO
Fasciitis with eosinophilia syndrome
MedGen UID:
82673
•Concept ID:
C0264005
•
Disease or Syndrome
Inflammation and thickening (localized fibrosis) of the fascia, the tissue under the skin and over the muscle, typically associated with a build up of eosinophils in the muscles and tissues.
See: Feature record | Search on this feature

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFasciitis with eosinophilia syndrome
Follow this link to review classifications for Fasciitis with eosinophilia syndrome in Orphanet.

Conditions with this feature

Fasciitis with eosinophilia syndrome
MedGen UID:
82673
•Concept ID:
C0264005
•
Disease or Syndrome
Inflammation and thickening (localized fibrosis) of the fascia, the tissue under the skin and over the muscle, typically associated with a build up of eosinophils in the muscles and tissues.
See: Condition Record
Fasciitis with eosinophilia syndrome

Professional guidelines

PubMed

Cutaneous Immune-Related Adverse Events Secondary to Immune Checkpoint Inhibitors and Their Management.
Pach J, Leventhal JS
Crit Rev Immunol 2022;42(4):1-20. doi: 10.1615/CritRevImmunol.2023046895. PMID: 37022356
Eosinophilic Dermatoses: Recognition and Management.
Marzano AV, Genovese G
Am J Clin Dermatol 2020 Aug;21(4):525-539. doi: 10.1007/s40257-020-00520-4. PMID: 32394361
The eosinophilia-myalgia syndrome: status of 205 patients and results of treatment 2 years after onset.
Hertzman PA, Clauw DJ, Kaufman LD, Varga J, Silver RM, Thacker HL, Mease P, Espinoza LR, Pincus T
Ann Intern Med 1995 Jun 1;122(11):851-5. doi: 10.7326/0003-4819-122-11-199506010-00008. PMID: 7741371

Recent clinical studies

Etiology

Eosinophilic Dermatoses: Recognition and Management.
Marzano AV, Genovese G
Am J Clin Dermatol 2020 Aug;21(4):525-539. doi: 10.1007/s40257-020-00520-4. PMID: 32394361
Eosinophilic dermatoses.
Peckruhn M, Elsner P, Tittelbach J
J Dtsch Dermatol Ges 2019 Oct;17(10):1039-1051. Epub 2019 Sep 27 doi: 10.1111/ddg.13943. PMID: 31562692
Morphea and Eosinophilic Fasciitis: An Update.
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
Eosinophilic Skin Diseases: A Comprehensive Review.
Long H, Zhang G, Wang L, Lu Q
Clin Rev Allergy Immunol 2016 Apr;50(2):189-213. doi: 10.1007/s12016-015-8485-8. PMID: 25876839
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

Not just eosinophilic fasciitis.
Chirila R, Cristea ER, Purcarea MR, Tribus LC
J Med Life 2021 Jan-Mar;14(1):121-124. doi: 10.25122/jml-2021-0015. PMID: 33767797Free PMC Article
Eosinophilic Dermatoses: Recognition and Management.
Marzano AV, Genovese G
Am J Clin Dermatol 2020 Aug;21(4):525-539. doi: 10.1007/s40257-020-00520-4. PMID: 32394361
Morphea and Eosinophilic Fasciitis: An Update.
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
Eosinophilic fasciitis.
Lamback EB, Resende FS, Lenzi TC
An Bras Dermatol 2016 Sep-Oct;91(5 suppl 1):57-59. doi: 10.1590/abd1806-4841.20164683. PMID: 28300895Free PMC Article
Eosinophilic Skin Diseases: A Comprehensive Review.
Long H, Zhang G, Wang L, Lu Q
Clin Rev Allergy Immunol 2016 Apr;50(2):189-213. doi: 10.1007/s12016-015-8485-8. PMID: 25876839

Therapy

Not just eosinophilic fasciitis.
Chirila R, Cristea ER, Purcarea MR, Tribus LC
J Med Life 2021 Jan-Mar;14(1):121-124. doi: 10.25122/jml-2021-0015. PMID: 33767797Free PMC Article
Eosinophilic Dermatoses: Recognition and Management.
Marzano AV, Genovese G
Am J Clin Dermatol 2020 Aug;21(4):525-539. doi: 10.1007/s40257-020-00520-4. PMID: 32394361
Morphea and Eosinophilic Fasciitis: An Update.
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
Eosinophilic fasciitis.
Lamback EB, Resende FS, Lenzi TC
An Bras Dermatol 2016 Sep-Oct;91(5 suppl 1):57-59. doi: 10.1590/abd1806-4841.20164683. PMID: 28300895Free PMC Article
Eosinophilic fasciitis (Shulman disease).
Lebeaux D, Sène D
Best Pract Res Clin Rheumatol 2012 Aug;26(4):449-58. doi: 10.1016/j.berh.2012.08.001. PMID: 23040360

Prognosis

Eosinophilia Associated With CD3(-)CD4(+) T Cells: Characterization and Outcome of a Single-Center Cohort of 26 Patients.
Carpentier C, Verbanck S, Schandené L, Heimann P, Trépant AL, Cogan E, Roufosse F
Front Immunol 2020;11:1765. Epub 2020 Aug 11 doi: 10.3389/fimmu.2020.01765. PMID: 32849632Free PMC Article
Eosinophilic Skin Diseases: A Comprehensive Review.
Long H, Zhang G, Wang L, Lu Q
Clin Rev Allergy Immunol 2016 Apr;50(2):189-213. doi: 10.1007/s12016-015-8485-8. PMID: 25876839
The fasciitis-panniculitis syndrome: clinical spectrum and response to cimetidine.
Naschitz JE, Yeshurun D, Zuckerman E, Rosner I, Shajrawi I, Missellevitch I, Boss JH
Semin Arthritis Rheum 1992 Feb;21(4):211-20. doi: 10.1016/0049-0172(92)90051-e. PMID: 1570516
The eosinophilia-myalgia syndrome and eosinophilic fasciitis.
Jimenez SA, Varga J
Curr Opin Rheumatol 1991 Dec;3(6):986-94. doi: 10.1097/00002281-199112000-00015. PMID: 1772754
Eosinophilic fasciitis.
Krauser RE, Tuthill RJ
Arch Dermatol 1977 Aug;113(8):1092-3. PMID: 889336

Clinical prediction guides

Eosinophilic Fasciitis - Clinical Features and Therapeutic Management.
Bilewicz-Stebel M, Bergler-Czop B, Stebel R, Weryńska-Kalemba M, Matuszewska A
Acta Dermatovenerol Croat 2020 Dec;28(3):190-192. PMID: 33422175
Characteristics of Japanese patients with eosinophilic fasciitis: A brief multicenter study.
Yamamoto T, Ito T, Asano Y, Sato S, Motegi SI, Ishikawa O, Matsushita T, Takehara K, Makino T, Okiyama N, Fujimoto M, Jinnin M, Ihn H
J Dermatol 2020 Dec;47(12):1391-1394. Epub 2020 Aug 29 doi: 10.1111/1346-8138.15561. PMID: 32860239
Eosinophilia Associated With CD3(-)CD4(+) T Cells: Characterization and Outcome of a Single-Center Cohort of 26 Patients.
Carpentier C, Verbanck S, Schandené L, Heimann P, Trépant AL, Cogan E, Roufosse F
Front Immunol 2020;11:1765. Epub 2020 Aug 11 doi: 10.3389/fimmu.2020.01765. PMID: 32849632Free PMC Article
Eosinophilic fasciitis: clinical spectrum and therapeutic response in 52 cases.
Lakhanpal S, Ginsburg WW, Michet CJ, Doyle JA, Moore SB
Semin Arthritis Rheum 1988 May;17(4):221-31. doi: 10.1016/0049-0172(88)90008-x. PMID: 3232080
Toxic oil syndrome: a syndrome with features overlapping those of various forms of scleroderma.
Alonso-Ruiz A, Zea-Mendoza AC, Salazar-Vallinas JM, Rocamora-Ripoll A, Beltrán-Gutiérrez J
Semin Arthritis Rheum 1986 Feb;15(3):200-12. doi: 10.1016/0049-0172(86)90017-x. PMID: 3961509

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