Fasciitis eosinophil M35.4

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Diffuse fasciitis with eosinophilia; eosinophilic fasciitis; Eosinophilic fasciitis; Fasciitis diffuse with eosinophilia; Shulman Syndrome

History
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Shulman, 1974

Definition
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Rare, acute scleroderma-like (or associated) disease with doughy, edematous, red swelling and indurations of the extremities in connection with pronounced blood eosinophilia, elevated CRP and hypergammaglobulinemia of unknown origin (probably a clinical picture belonging to the circumscipt scleroderma group).

There is great clinical similarity with the " Eosinophilic-Myalgie-Syndrome" first described in 1989, which occurs after taking tryptophane, and the " Toxic-Oil-Syndrome" after consumption of contaminated cooking oil.

Etiopathogenesis
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Unknown. Probably a defect in the immune system. Questionable connection with previous physical exertion or trauma. Possible association with thrombocytopenia, aplastic or haemolytic anaemia. Occurrence in the context of Lyme borreliosis is possible.

Manifestation
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Mostly occurring in middle adulthood (2nd-6th decade of life). In childhood mostly occurring in the female sex (75%), in later life balanced ratio. Occurs frequently after previous physical exertion or trauma.

Localization
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Occurs symmetrically on the extremities, especially on the forearms. Rarely localized on the trunk and face.

Clinical features
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  • Sudden appearance of initially doughy, painful, red swellings with rapidly following, wood-like indurations of deep tissue parts. The veins appear sunken (negative vein pattern).
  • Fascial indurations are at the forefront of the changes, whereby the sclerosing inflammation can also spread to the subcutaneous fatty tissue and the lower parts of the dermis. Clinically typical for the disease (also in distinction to systemic scleroderma) is the easy displacement of the skin over the deep, wood-like solid sclerosing zones. Fingers and toes remain unaffected.
  • Frequent formation of joint contractures and carpal tunnel syndrome. Occasional accompanying arthralgia, myositis, paresthesia of the fingers. No involvement of internal organs, no Raynaud's phenomenon.
  • In recent years other associated diseases have been described: Hashimoto's thyroiditis, involvement of the lungs, esophagus, myocardium, kidney, colon and CNS. Associations with breast or prostate carcinoma.

Laboratory
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In over 90% blood eosinophilia up to 50% (500-200 eosinophiles/µl); CRP elevation; BSG acceleration; hypergammaglobulinemia; slight CK elevation. ANA and rheumatoid factor are usually negative.

Histology
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Note: For histological confirmation a deep and sufficiently large excision biopsy is necessary.

Marked thickening and sclerosing of the fascia; in some cases fibrotic strands protrude into the overlying subcutaneous fatty tissue. Lymphoplasmocellular infiltrate of varying degrees, possibly with infiltration of eosinophils of varying density. Muscle involvement with degeneration of the muscle fibres is possible.

Direct Immunofluorescence
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Immunoglobulins andC3 in the epidermal basement membrane zone and perivascularly or diffusely in the connective tissue of the fascia. They are diagnostically irrelevant.

Diagnosis
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Clinical, blood eosinophilia, histoeosinophilia, no organ involvement or Raynaud's symptoms, hypergammaglobulinemia. For histological examination: biopsy en bloc with dermis, subcutis, fascia and muscle tissue.

Differential diagnosis
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Clinical:

External therapy
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Lymph drainage (see lymphedema), careful massage.

Radiation therapy
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Success under therapy with UVA1 irradiation has been observed in case reports of eosinophilic fasciitis.

Internal therapy
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  • Agents of choice are glucocorticoids at medium dosages such as prednisolone 1 mg/kg bw/day. Rapid regression of the signs of inflammation is expected, indurations persist longer. In case of progression of the symptoms, increase the dosage of glucocorticoids up to 100 mg/day. According to the clinical findings, slow reduction to the lowest possible maintenance dose.
  • If necessary, combination with azathioprine (e.g. Imurek) 1-2 mg/kg bw/day (saving of glucocorticoids!). Therapy failures as well as spontaneous remissions are known.
  • Other immunomodulatory systemic therapeutics can be used in addition: cyclophosphamide, methotrexate, chloroquine. Positive individual results were described under extracorporeal photopheresis and interferon alpha.

Progression/forecast
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Chronic, intermittent course; prompt response to systemic glucocorticoids.

Literature
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  1. Antic M (2006) Eosinophilic fasciitis 30 years after - what do we really know Dermatology 213: 93-101
  2. Dziadzio L (2003) Cytokine abnormalities in a patient with eosinophilic fasciitis. Ann Allergy Asthma Immunol 90: 452-455
  3. Hintner H et al (1981) Fasciitis with eosinophilia - The Shulman syndrome. Dermatologist 32: 75-79
  4. Jinnin M (2003) Circulating soluble CD40 ligand in patients with eosinophilic fasciitis. Ann Rheum Dis 62: 190-191
  5. Mosconi S et al (2002) Eosinophilic fasciitis (Shulman syndrome). Dermatology 205: 204-206
  6. Romano C et al (2003) Extracorporeal photochemotherapy in the treatment of eosinophilic fasciitis. J Eur Acad Dermatol Venereol 17: 10-13
  7. Shulman LE (1974) Diffuse fasciitis with hyperglobulinemia and eosinophilia: A new syndrome? J Rheumatology (Toronto) 1: S46
  8. Shulman LE (1975) Diffuse fasciitis with eosinophilia: a new syndrome? Trans Assoc Am Physicians 88: 70-86
  9. by Gizycki et al (1991) Eosinophilia-Myalgie Syndrome and L-tryptophan ingestion. dermatologist 42: 179-182
  10. Weber HO et al (2008) Eosinophilic fasciitis and combined UVA1--retinoid--corticosteroid treatment: two case reports. Acta Derm Venereol 88: 304-306

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Last updated on: 29.10.2020