Eosinophilic fasciitis 4.45/5 (11)

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Definition

Eosinophilic fasciitis is a rare inflammatory disease that without a known cause attacks the subcutaneous tissue and the fascia surrounding the muscles. The skin on the legs and/or forearms, often on both sides, becomes warm, red and tender. Eventually the skin becomes tight and you can see indentations with an uneven surface (orange peel skin). The symptoms are distinguished from systemic sclerosis (scleroderma) and similar conditions. Eosinophilic fasciitis was described by the American Lawrence Shulman (1919-2009) in 1974 (Shulman LE, 1974). Some cases are associated with underlying blood and/or cancer.

Occurrence

Eosinophilic fasciitis, the Masson A, 2013, Medicine (Baltimore). Open-in

Adult women are attacked slightly more often than men. The average age at onset is 47–57 years (Wright NA, 2016). The disease rarely occurs in children (Grisanti MW, 1989). Eosinophilic fasciitis is defined as a rare disease.

Symptoms

Eosinophilic fasciitis usually starts quite suddenly and can only be felt after physical exertion. Night sweats and a slight fever may occur, but otherwise the general condition need not be particularly affected.

One notices swelling, stiffness and sees some redness over one or more muscles or tendons in the arms or legs. Swelling around the ankles (oedema) occurs when the legs are affected. Painful tightening in affected skin areas is common. In 95%, both legs or both arms are attacked symmetrically (Wright NA, 2016).

Arthritis (arthritis) seen in up to 40%, but are not as obvious as in arthritis (rheumatoid arthritis) or similar joint diseases.

Muscle pain can also be part of the disease picture.

Carpal tunnel syndrome / canalis carpi syndrome with numbness and pain in the hands is not uncommon and is due to compression of median nerve at the wrist.

Skin- the symptoms are typical. Increased heat, redness, swelling and pain in both legs or forearms is seen. After weeks or months, the skin becomes less swollen, but more tight, hard and with indentations. Most often the changes are limited, but in some cases the face, chest and stomach area can also be attacked, so that all movement is significantly restricted. Unlike systemic sclerosis/scleroderma, the hands and feet are not affected Raynaud's phenomenon ("corpse fingers") is unusual. Internal organs are not attacked.

Examinations

MR at Eosinophilic Fasciitis, Lee HS, Chang SJ, Allergy Asthma Immunol Res (2013). CC BY-NC 3.0

Medical history maps symptoms (see above).

Clinical a general status is made with an assessment of the skin, joints and internal organs. Skin changes that are detected can be increased heat and redness, pseudocellulite or Orange peel (orange peel). "Groove sign" is an indentation/indentation that follows the course of veins and is particularly visible when the affected arm or leg is lifted up. The reason is increased connective tissue (fibrosis) in the underlying tissue (Fruchter R, 2017). Signs of concurrent blood disease and/or cancer are assessed in each case.

Blood tests. Increased blood lowering reaction (SR), CRP. Characteristically, an increased number of eosinophilic white blood cells is seen in more than 80% of cases. These are measured by differential counting in a regular blood test. After possible treatment with prednisolone or other cortisone, the samples usually quickly return to normal. Some people may have slightly elevated CK with simultaneous muscle manifestation. No one special Antibodies available.

Imaging. MRI examination of the affected area is expected to show signs of inflammation in the connective tissue around the muscles (fasciitis) (reference: Moulton SJ, 2005).

Tissue sample (biopsy) shows eosinophilia in tissue. Biopsies are taken surgically so that all skin layers and muscle fascia are included. Eosinophilic white blood cells, lymphocytes and plasma cells are expected on tissue culture. Sometimes inflammation of blood vessels (vasculitis) is also seen in the small vessels of the fascia. Deposition of immunoglobulins can occasionally also be detected (reference: Hironobu I, 2019).

-Bone marrow. About 10% with eosinophilic fasciitis also have a blood disease (various types) which also includes malignant cases. If a blood disorder is suspected, further investigation with a bone marrow examination is appropriate.

Diagnosis

The diagnosis is based on medical history and examinations (see above). In this context, proposals have also been drawn up for diagnostic criteria that can be used (reference: Pinal-Fernandez I, 2014).

Similar conditions, differential diagnoses

Eosinophilic fasciitis with contractions of the skin (b). Systemic sclerosis with salt and pepper pigmentation (a). Ghosh S, 2013. CC BY NC SA 3.0

Borrelia/Lyme disease. Chronic Lyme disease with atrophic dermatitis can sometimes look similar, but is one-sided.

Dermatomyositis (reference: Chirila R, 2021)

Eosinophilia-myalgia syndrome. Eosinophilic fasciitis must not be confused with Eosinophilia-Myalgia syndrome which is caused by ingestion of L-tryptophan.

Fasciitis-panniculitis syndrome. Erythema, oedema, induration in extremities, arthritis, myalgia, fever. Previously thought to be a subgroup of eosinophilic fasciitis, but eosinophilia is often absent and the condition is more frequently associated with malignancy (reference: Nishikubo M, 2021). Tissue sample (histological) shows infiltration of inflammatory cells in adipose tissue (panniculitis) and muscle fascia compatible with septal and lobular panniculitis. The edema can be misinterpreted as deep vein thrombosis (reference: Morales M, 2006).

Graft vs. Host Disease (GVHD) (GVHD, rejection reaction). Most often related to transplantation.

Hypereosinophilic Syndrome lacks affection of the locomotor apparatus.

Linear scleroderma / morphea. Linear scleroderma is most common in children, but can start in adulthood. Mainly one side of the body is attacked. Eosinophilic fasciitis can also be confused with morphea, which is a scleroderma-like skin disease with a wide clinical spectrum. Some perceive eosinophilic fasciitis as an extreme form of morphea (Mertens JS, 2017).

Myositis. Fasciitis is also seen in myositis, especially in the early phase of the disease. Eosinophilic fasciitis can coexist with polycythemia vera.

Nephrogenic systemic fibrosis. After MRI contrast in renal failure

Skleredema (Buschke). Most common in diabetes. Upper body most often affected

Systemic sclerosis. The most important differential diagnosis is systemic sclerosis. Raynaud's phenomenon, fingers are attacked.

Toxic cooking oil syndrome. Occurred in Spain 1981 as a result of ingestion of too old, damaged cooking oil.

Treatment

Most people have a good effect from early treatment, while starting treatment later than six months from the onset of the disease increases the risk of a poorer effect (reference: Lebeaux D, 2012).

Corticosteroids (Prednisone). High doses to prevent scarring and persistent reduced mobility may be necessary. Initial dosage 40-60mg/d is suggested (reference: Lakhanpal S, 1988), but lower doses are also used. One expects a rapid response to signs of inflammation and a high number of eosinophilic white blood cells, but clinically a slow recovery is seen. Gradual tapering and termination of treatment over 2-5 years is not unusual. About half of people with eosinophilic fasciitis need other immunosuppressive treatment in addition (please see below).

DMARDS. Most will need a combination with steroid-sparing medication (DMARDs). The most common combination is included methotrexate which can increase the proportion with a good treatment effect (remission rate) from 30% to 64%. Other DMARDs such as mycophenolate (CellCept), ciclosporin (Sandimmun neoral), dapsone and azathioprine (Imurel) have less expected effect (reference: Wright NA, 2016).

Biological drugs is experimental medication, such as the IL-6 inhibitor tocilizumab, TNF inhibitors, rituximab and immunoglobulins. Efficacy has been reported in small studies or reports, but there are no randomized controlled trials (RCTs) (reference: Mufti A, 2021).

Prognosis

Gradual improvement after a few years. Many become almost completely symptom-free in the long term. The disease hardly affects life expectancy, except where serious associated blood disease and/or cancer is detected.

Literature


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