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Fasciitis with eosinophilia syndrome Graphs for this disease are generated based on data from the Autoimmune Registry Inc.
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  • Names
    • Fasciitis with eosinophilia syndrome
    • Eosinophilic fasciitis
    • Pansclerotic morphea with deep fascial involvement
    • Shulman syndrome
    • Shulman's syndrome

    Category: Systemic
    Prevalence From 3,333 to 6,999 cases in the US (Spielmann 2018). Female/Male ratio: 2.3x.
    Age of Onset Onset typically occurs between ages 30 and 60.
    Description Eosinophilic fasciitis is a rare, scleroderma-like, autoimmune rheumatic disorder in which the skin and tissue (the fascia, fibrous tissue that separates different layers of tissues under the skin) that lies beneath the skin become painfully inflamed and swollen and gradually hardens. It affects the forearms, the upper arms, the lower legs, the thighs, and the trunk (in order of decreasing frequency). The disease is considered by some to be a deep variant of the skin condition morphea, and is also known as pansclerotic morphea with deep fascial involvement. Patients usually present suddenly with painful, tender, swollen and red extremities. Within weeks to months, patients develop stiffness and affected skin becomes hardened, creating a characteristic orange-peel appearance over the surfaces of the extremities. In severely affected areas, the skin and the subcutaneous tissue are bound tightly to the underlying muscle. This creates a woody-type appearance. The presentation of eosinophilic fasciitis is similar to scleroderma or systemic sclerosis. However unlike scleroderma it affects the fascia, not the skin (dermis). Unlike scleroderma and systemic sclerosis, effects such as Raynaud's phenomenon, which affects prominent small blood vessels in the extremities (telangiectasia), and visceral changes, such as swallowing problems, are absent. It was first characterized in 1974 and it is not yet known whether it is actually a distinct condition or just a different presentation of morphea.

    Not confirmed as an autoimmune or autoinflammatory disease.
    ICD-10 Code: M35.4     SNOMED Code: 24129002
    Patient Groups
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      Symptoms
      Resources Link to Clinical Trials
      Link to PubMed
      References Link to Cleveland Clinic
      Link to Healthline
      Profile by: Autoimmune Registry
    • Profile updated September 2024
    • Charts generated Nov 16, 2024 at 09:55 AM ET
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      Fasciitis with eosinophilia syndrome sex breakdown


      Fasciitis with eosinophilia syndrome age breakdown

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      Graphs for this disease are generated based on data from the Autoimmune Registry Inc.

    • Fasciitis with eosinophilia syndrome top comorbidities/symptoms
      Chart is omitted due to low report count.

      Fasciitis with eosinophilia syndrome top autoimmune comorbidities


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      * Disease is suspected as autoimmune, but unconfirmed.
      Data Source: Graphs on this page represent data from the Autoimmune Registry Inc. here. To protect participant anonymity, results derived from fewer than 5 participants are not graphed. The Autoimmune Registry Inc is a 501(c)(3) non-profit supported by donors like you.

    • Anti-inflammatory / specialty medications and supplements

      Fasciitis with eosinophilia syndrome top immunosuppressants / autoimmune therapies


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      * Biologic medications.
      Data Source: Graphs on this page represent data in electronic health records from the All of Us research database. As of July 2024, there are 451,000 sets of electronic health records collected from participants. A snapshot of the data can be found here. To protect participant anonymity, results derived from fewer than 20 participants are not graphed.
      The All of Us Research Program is supported by the National Institutes of Health, Office of the Director: Regional Medical Centers: 1 OT2 OD026549; 1 OT2 OD026554; 1 OT2 OD026557; 1 OT2 OD026556; 1 OT2 OD026550; 1 OT2 OD 026552; 1 OT2 OD026553; 1 OT2 OD026548; 1 OT2 OD026551; 1 OT2 OD026555; IAA #: AOD 16037; Federally Qualified Health Centers: HHSN 263201600085U; Data and Research Center: 5 U2C OD023196; Biobank: 1 U24 OD023121; The Participant Center: U24 OD023176; Participant Technology Systems Center: 1 U24 OD023163; Communications and Engagement: 3 OT2 OD023205; 3 OT2 OD023206; and Community Partners: 1 OT2 OD025277; 3 OT2 OD025315; 1 OT2 OD025337; 1 OT2 OD025276. In addition, the All of Us Research Program would not be possible without the partnership of its participants.

    • Quick links: 
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      Patient-reported 7-day average pain
      Chart is omitted due to low report count.


      Patient-reported 7-day average fatigue
      Chart is omitted due to low report count.


      General physical health
      Chart is omitted due to low report count.


      General mental health
      Chart is omitted due to low report count.


      Social and relationship satisfaction
      Chart is omitted due to low report count.


      General quality of life
      Chart is omitted due to low report count.


      Ability to carry out social roles and activities
      Chart is omitted due to low report count.


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      Data Source: Graphs on this page represent data in self-reported survey results from the All of Us research database. As of July 2024, there are 560,000 participants who have completed these surveys. This dataset, however, may not be representative of the US population. A snapshot of the data can be found here. To protect participant anonymity, results derived from fewer than 20 participants are not graphed.
      The All of Us Research Program is supported by the National Institutes of Health, Office of the Director: Regional Medical Centers: 1 OT2 OD026549; 1 OT2 OD026554; 1 OT2 OD026557; 1 OT2 OD026556; 1 OT2 OD026550; 1 OT2 OD 026552; 1 OT2 OD026553; 1 OT2 OD026548; 1 OT2 OD026551; 1 OT2 OD026555; IAA #: AOD 16037; Federally Qualified Health Centers: HHSN 263201600085U; Data and Research Center: 5 U2C OD023196; Biobank: 1 U24 OD023121; The Participant Center: U24 OD023176; Participant Technology Systems Center: 1 U24 OD023163; Communications and Engagement: 3 OT2 OD023205; 3 OT2 OD023206; and Community Partners: 1 OT2 OD025277; 3 OT2 OD025315; 1 OT2 OD025337; 1 OT2 OD025276. In addition, the All of Us Research Program would not be possible without the partnership of its participants.

    • Quick links: 
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      Concerns about ability to pay
      Chart is omitted due to low report count.


      Feeling unheard by healthcare providers
      Chart is omitted due to low report count.


      Patient employment status
      Chart is omitted due to low report count.


      Patient insurance coverage
      Chart is omitted due to low report count.


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      Graphs for this disease are generated based on data from the Autoimmune Registry Inc.