U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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.
 
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.

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.

Professional guidelines

PubMed

Pach J, Leventhal JS
Crit Rev Immunol 2022;42(4):1-20. doi: 10.1615/CritRevImmunol.2023046895. PMID: 37022356
Marzano AV, Genovese G
Am J Clin Dermatol 2020 Aug;21(4):525-539. doi: 10.1007/s40257-020-00520-4. PMID: 32394361
Spry CJ
Allergy 1982 Nov;37(8):539-51. doi: 10.1111/j.1398-9995.1982.tb02339.x. PMID: 6758627

Recent clinical studies

Etiology

Marzano AV, Genovese G
Am J Clin Dermatol 2020 Aug;21(4):525-539. doi: 10.1007/s40257-020-00520-4. PMID: 32394361
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
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
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
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

Marzano AV, Genovese G
Am J Clin Dermatol 2020 Aug;21(4):525-539. doi: 10.1007/s40257-020-00520-4. PMID: 32394361
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
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
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
Bielsa I, Ariza A
Semin Cutan Med Surg 2007 Jun;26(2):90-5. doi: 10.1016/j.sder.2007.02.005. PMID: 17544960

Therapy

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
Marzano AV, Genovese G
Am J Clin Dermatol 2020 Aug;21(4):525-539. doi: 10.1007/s40257-020-00520-4. PMID: 32394361
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
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
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

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
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
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
Jimenez SA, Varga J
Curr Opin Rheumatol 1991 Dec;3(6):986-94. doi: 10.1097/00002281-199112000-00015. PMID: 1772754
Krauser RE, Tuthill RJ
Arch Dermatol 1977 Aug;113(8):1092-3. PMID: 889336

Clinical prediction guides

Boussaa H, Kamoun M, Miladi S, Makhlouf Y, Abdelghani KB, Fazaa A, Laatar A
Mod Rheumatol Case Rep 2023 Dec 29;8(1):224-228. doi: 10.1093/mrcr/rxad063. PMID: 37902576
Bilewicz-Stebel M, Bergler-Czop B, Stebel R, Weryńska-Kalemba M, Matuszewska A
Acta Dermatovenerol Croat 2020 Dec;28(3):190-192. PMID: 33422175
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
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
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

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...