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Fasciitis eosinophilM35.4

Author:Prof. Dr. med. Peter Altmeyer

Last updated on: 14.05.2021

Synonym(s)

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

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HistoryThis section has been translated automatically.

Shulman, 1974

DefinitionThis section has been translated automatically.

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.

EtiopathogenesisThis section has been translated automatically.

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.

ManifestationThis section has been translated automatically.

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.

LocalizationThis section has been translated automatically.

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

Clinical featuresThis section has been translated automatically.

Sudden onset of initially doughy, painful, red swellings followed rapidly by woody indurations of deep tissues. The veins appear sunken (negative vein drawing).

The fascial indurations are in the foreground of the changes, whereby the sclerosing inflammation can also spread to the subcutaneous adipose tissue and the lower parts of the dermis. Clinically typical for the disease (also in differentiation to systemic scleroderma) is the easy displaceability of the skin over the deep, wood-like firm sclerosing zones. Fingers and toes remain unaffected.

Frequent development of joint contractures and carpal tunnel syndrome. Occasional accompanying arthralgias, myositis, paresthesias of the fingers. No involvement of internal organs, no Raynaud's phenomenon.

In recent years, other associated diseases have been described: Hashimoto's thyroiditis, co-involvement of lung, esophagus, myocardium, kidney, colon and CNS. Associations with carcinoma of the breast or prostate.

LaboratoryThis section has been translated automatically.

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.

HistologyThis section has been translated automatically.

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 ImmunofluorescenceThis section has been translated automatically.

Immunoglobulins and_C3 in the epidermal basement membrane zone and perivascularly or diffusely in the connective tissue of the fascia. They are diagnostically irrelevant.

DiagnosisThis section has been translated automatically.

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 diagnosisThis section has been translated automatically.

Clinical:

Systemic scleroderma: Raynaud's phenomenon, acrosclerosis, no blood eosinophilia.
Dermatomyositis: heliotropic exanthema, no deep sclerosis
Nephrogenic systemic fibrosis: mostly in renal insufficient patients with after application of gadolinium containing contrast media.
Dermatoliposclerosis: only lower leg involvement, eminently chronic, always CVI.

External therapyThis section has been translated automatically.

Lymph drainage (see lymphedema), careful massage.

Radiation therapyThis section has been translated automatically.

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

Internal therapyThis section has been translated automatically.

Glucocorticoids in medium dosage such as prednisolone 1 mg/kg bw/day are the treatment of choice. Prompt regression of the inflammatory signs is expected, indurations persist longer. If the symptoms progress, increase the dose 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 glucocorticoids!). Therapy failures as well as spontaneous remissions are known.

Other immunomodulatory system therapeutics can be used in addition: cyclophosphamide, methotrexate, chloroquine. Positive individual results have been described with extracorporeal photopheresis and interferon alfa.

Progression/forecastThis section has been translated automatically.

Chronic, intermittent course; prompt response to systemic glucocorticoids.

LiteratureThis section has been translated automatically.

Antic M (2006) Eosinophilic fasciitis 30 years after - what do we really know? Dermatology 213: 93-101.
Dziadzio L (2003) Cytokine abnormalities in a patient with eosinophilic fasciitis. Ann Allergy Asthma Immunol 90: 452-455
Hintner H et al (1981) Fasciitis with eosinophilia - The Shulman syndrome. Dermatologist 32: 75-79
Ihn H (2019) Eosinophilic fasciitis: From pathophysiology to treatment. Allergol Int 68:437-439.
Jinnin M (2003) Circulating soluble CD40 ligand in patients with eosinophilic fasciitis. Ann Rheum Dis 62: 190-191
Mosconi S et al (2002) Eosinophilic fasciitis (Shulman syndrome). Dermatology 205: 204-206
Romano C et al (2003) Extracorporeal photochemotherapy in the treatment of eosinophilic fasciitis. J Eur Acad Dermatol Venereol 17: 10-13
Shulman LE (1974) Diffuse fasciitis with hyperglobulinemia and eosinophilia: A new syndrome? J Rheumatology (Toronto) 1: S46
Shulman LE (1975) Diffuse fasciitis with eosinophilia: a new syndrome? Trans Assoc Am Physicians 88: 70-86
Von Gizycki et al (1991) Eosinophilia-myalgia syndrome and L-tryptophan intake. Dermatologist 42: 179-182
Weber HO et al (2008) Eosinophilic fasciitis and combined UVA1--retinoid--corticosteroid treatment: two case reports. Acta Derm Venereol 88: 304-306

Authors

Last updated on: 14.05.2021

Author: Prof. Dr. med. Peter Altmeyer

Editors: Dr. med. Conrad Hempel Dr. Kathrin Hillmann