Fasciitis eosinophil M35.4

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.

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.

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.

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.

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.

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