Eosinophilic fasciitis

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The eosinophilic fasciitis (synonym: Shulman's syndrome ) is a rare disease of the connective tissue, which is inflammation of the fascia with swelling and hardening of the skin, an increased incidence of eosinophil granulocytes in the blood ( eosinophilia ) and a hypergammaglobulinemia comes. Several hundred cases have been described worldwide since it was first described in 1974. The disease usually occurs between the ages of 20 and 30 and between the ages of 45 and 60, although cases in children and very old patients have also been described. The therapy of choice is the administration of high-dose corticosteroids such as cortisone .

Disease emergence

The causes of the disease are unknown. Some cases were preceded by taking certain medications (such as statins ), or by infection or strenuous exercise. Most of the time, however, the symptoms appear without a recognizable trigger. A stimulus - of whatever kind - probably leads to a misdirected immune response, through which eosinophilic granulocytes and lymphocytes attach to the fascia. As part of the inflammatory reaction, the connective tissue of the fasciae increases. Exactly which mechanisms play a role in this has not yet been clarified.

Symptoms

The disease usually begins with symmetrical reddening and hardening of the subcutaneous tissue, followed by orange peel and edema on the arms and legs. This often leads to a restriction in joint mobility. A typical sign are skin retractions that follow the course of the subcutaneous veins. The signs sometimes show up on the neck or trunk, but the internal organs are usually not affected.

diagnosis

Laboratory findings

Blood tests often reveal an increase in eosinophil granulocytes ( eosinophilia ), an increase in immunoglobulins (hypergammaglobulinemia) and C-reactive protein, and an accelerated rate of sedimentation .

Imaging

Magnetic resonance tomography (MRI) is best suited for imaging diagnostics. Here the affected fascia appear thickened and enriched with water. The use of ultrasound is still in the experimental stage, but appears to be suitable for monitoring the progress during therapy.

pathology

The presence of eosinophilic fasciitis can be proven by a deep biopsy, which includes the skin and muscles, and a subsequent microscopic examination of the tissue . Here, the fasciae appear thickened and penetrated by eosinophilic granulocytes and T killer cells . Also, macrophages and plasma cells may be encountered. In some cases, myositis (inflammation of the muscles) is also detected.

Differential diagnoses

Eosinophilic fasciitis must be differentiated from the following diseases in the differential diagnosis:

Web links

Individual evidence

  1. ↑ Milos Antic, Stephan Lautenschlager, Peter H. Itin: Eosinophilic Fasciitis 30 Years after - What Do We Really Know? In: Dermatology . tape 213 , no. 2 , 2006, ISSN  1018-8665 , p. 93-101 , doi : 10.1159 / 000093847 ( karger.com [accessed March 30, 2020]).
  2. ↑ a b c d e David Lebeaux, Damien Sène: Eosinophilic fasciitis (Shulman disease) . In: Best Practice & Research Clinical Rheumatology . tape 26 , no. 4 , August 2012, p. 449–458 , doi : 10.1016 / j.berh.2012.08.001 ( elsevier.com [accessed March 30, 2020]).
  3. ↑ Sabrina Poradosú, Esther Vander Linden, Alex Michotte, Hendrik De Raeve, Sabine D. Allard: Eosinophilic fasciitis: an Atypical Presentation of a Rare Disease . In: European Journal of Case Reports in Internal Medicine 2015. Link (English)