HistoryThis section has been translated automatically.
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.
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EtiopathogenesisThis section has been translated automatically.
ManifestationThis section has been translated automatically.
LocalizationThis section has been translated automatically.
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.
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.
DiagnosisThis section has been translated automatically.
Differential diagnosisThis section has been translated automatically.
- 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.
Radiation therapyThis section has been translated automatically.
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.
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
Incoming links (13)Dermatitis-arthritis syndromes; Eosinophilia skin changes; Eosinophilic fasciitis; Eosinophilic myalgie syndrome; Fasciitis diffuse with eosinophilia; Nephrogenic systemic fibrosis; Panniculitis nodularis nonsuppurativa; Polycythaemia vera; Pseudoscleroderma; Scleroderma circumscripts juvenile; ... Show all
Outgoing links (20)Azathioprine; Chloroquine; Chronic venous insufficiency (overview); Circumscripts of scleroderma (overview); Cyclophosphamide; Dermatoliposclerosis; Dermatomyositis (overview); Eosinophilic myalgie syndrome; Glucocorticosteroids systemic; Interferon alpha; ... Show all
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