Eosinophilic Fasciitis - Causes, Symptoms, Diagnosis, and Treatment

Introduction

Eosinophilic fasciitis is also known as Shulman syndrome, which was named after the Shulman physician in 1974. It is a rare autoimmune skin disorder characterized by tough fibrous tissue beneath the skin. Eosinophilic fasciitis is considered to be a variant of systemic sclerosis. Eosinophilic fasciitis most commonly affects the legs and arms. The onset of this disorder is acute, and the severity will vary from one person to another. The exact etiology of eosinophilic fasciitis is not clear, but it was found that the incidence rate of this disorder has a history of trauma and strenuous physical exercise.

What Is Eosinophilic Fasciitis?

Eosinophilic fasciitis, also referred to as Shulman syndrome, is characterized by the inflammation of the fascia called fasciitis and with variable infiltration of eosinophils. Symptoms are very acute with local edema, pain, symmetrical limb stiffening, and progress to fibrosis, causing limited joint movements. A confirmed diagnosis can be made with a skin biopsy. The face, abdomen, chest, and buttocks are other areas affected rarely with eosinophilic fasciitis. Eosinophilic fasciitis most commonly affects individuals between 40-50 years of age.

What Are the Clinical Features of Eosinophilic Fasciitis?

Eosinophilic fasciitis is an acute inflammatory condition characterized by erythema (redness), localized edema, and pain. Symptoms of eosinophilic fasciitis are usually symmetrical but sometimes unilateral. Initial symptoms of eosinophilic fasciitis are seen mainly on the legs and arms with inflammatory swelling and pain, whereas the hands and feet are usually unaffected. Symptoms occur within a week or two after trauma or strenuous exercise.

The standard features of eosinophilic fasciitis are mentioned below:

• Pain.

• Redness.

• Pitting edema.

• Symmetrical, sometimes unilateral symptoms.

• Skin elasticity is lost.

• Orange peel-like appearance of skin due to skin fibrosis.

• Prominent hair follicles.

• Disease progression in the skin causes a woody appearance.

• Hyperpigmentation.

• Decreased contractures and flexibility.

• A groove sign called linear depression in the path of a superficial blood vessel is present.

• Symptoms may affect the thighs and upper arms.

• The absence of Raynaud's phenomenon (decreased blood flow to the fingers) and sclerodactyly (skin tightening) is the distinguishing feature of eosinophilic fasciitis from scleroderma.

What Are the Causes of Eosinophilic Fasciitis?

The exact etiology of eosinophilic fasciitis is unknown, but it is found to be a result of an autoimmune disorder. It is evident with the detection of autoantibodies like rheumatoid factor or RF and antinuclear antibodies or ANA. The cause of fibrosis is the increased activity of fibroblasts. Increased activity of fibroblasts is due to the eosinophils. However, the exact etiology of the overproduction and the accumulation of eosinophils (a type of white blood cell) is not known. Eosinophils release the transforming growth factor or TGF resulting in increased production of type 1 collagen, fibronectin, and TMIP-1 or tissue inhibitor metalloproteinase- 1. Specific triggers also cause eosinophilic fasciitis.

Such causative triggers of eosinophilic fasciitis are given below:

• Insect bite.

• Effect of radiation.

• Infections such as Mycoplasma arginine, Borrelia burgdorferi.

• Medications include Atorvastatin, Carbidopa, Pembrolizumab, and Nivolumab.

• Paraneoplastic condition, usually leukemia and myeloproliferative disorder.

• Graft versus host disease.

What Are the Symptoms of Eosinophilic Fasciitis?

The onset of symptoms in eosinophilic fasciitis is sudden and varies from person to person. Acute inflammatory symptoms of eosinophilic fasciitis include pain, swelling, and inflammation. Eosinophilic fasciitis affects the arms and legs more often than the thigh and the lower extremities. Venous grooving is seen, and the affected area is erythematous, which is red. Blood abnormalities can lead to anemia and thrombocytopenia (decreased platelet count).

The nonspecific symptoms of eosinophilic fasciitis include the following mentioned below:

• Fatigue.

• Weight loss.

• Fever.

• Malaise.

• Weakness.

• Myalgia or muscle pain.

• Carpal tunnel syndrome includes numbness, pain, tingling, and burning sensation in the hands due to the compression of peripheral nerves.

• Sometimes viscera, the internal organs, may be infected.

• Difficulty in grasping objects.

• Tiredness.

• Dizziness.

• Pale skin color.

• Bruising because of thrombocytopenia.

Who is at Risk of Eosinophilic Fasciitis?

Eosinophilic fasciitis is found in relation to the ingestion of L-tryptophan, a dietary supplement. Tryptophan is an essential fatty acid. Individuals with strenuous exercise will also be at risk for developing eosinophilic fasciitis.

Other risk factors include the following.

• Trauma.

• Extreme physical activity.

• Fasciitis-panniculitis syndrome, or FPS, is characterized by a subcutaneous induration.

• Acquired aplastic anemia.

• Hemolytic anemia.

• Myelodysplastic syndrome cancer is caused by immature blood cell production.

• Myeloproliferative disorders are a type of blood cancer due to abnormal bone marrow function.

• Lymphoma (aggressive cancer).

• Leukemia.

• Thyroid disorders.

What Are the Related Disorders of Eosinophilic Fasciitis?

Symptoms of eosinophilic fasciitis are similar in the following disorders. Therefore, it is helpful in the differential diagnosis.

Below mentioned are some of the related disorders of eosinophilic fasciitis.

• Scleroderma (skin becomes hard and tight).

• Morphea is also known as localized scleroderma.

• Eosinophilia-myalgia syndrome (causing subacute muscle pain and eosinophilia).

• The Spanish toxic oil syndrome includes intense myalgias, peripheral eosinophilia, and pulmonary infiltrates.

• Hypereosinophilic syndrome.

• Eosinophilic cellulitis.

What Is the Diagnosis of Eosinophilic Fasciitis?

Detailed patient history, proper clinical examination, and laboratory studies help diagnose eosinophilic fasciitis. Unique symptoms, like groove signs called venous grooving, the linear depression following the path of the superficial blood vessel, are noted. The blood investigations are evident with the increased number of eosinophils and immunoglobulins. In histopathological findings, inflammatory infiltration is observed in the epidermis and the perivascular area.

Some other diagnostic tools for diagnosing eosinophilic fasciitis are:

• Blood tests revealed increased eosinophils.

• Increases level of immunoglobulins.

• Elevated erythrocyte sedimentation rate or ESI.

• Magnetic resonance imaging or MRI for skin fibrosis.

• Increased C reactive protein or CRP.

• Elevated serum aldolase level.

• Hypergammaglobulinemia (increased immunoglobulin level in the blood).

• Skin biopsy.

Histopathological Findings

• Thick fascia.

• Focal or diffuse lesion.

• Perivascular infiltration of lymphocytes and eosinophils.

• Presence of collagen.

• Atrophy of epidermis.

• Small strips of adipose tissue in between the collagen bands.

What Is the Treatment for Eosinophilic Fasciitis?

Eosinophilic fasciitis treatment is done to prevent and alleviate skin tissue inflammation. Some patients are reported with spontaneous remissions without any treatment. Corticosteroid therapy has beneficial effects on eosinophilic fasciitis patients. In addition to drug therapy, supportive and symptomatic treatment can be given.

Various treatment measures done for eosinophilic fasciitis are given below.

• Corticosteroid therapy such as Prednisolone.

• Surgical decompression for carpal tunnel syndrome.

• Surgery for treating contractures.

• Nonsteroidal inflammatory drugs or NSAIDs.

• Physiotherapy.

Additional Drugs

• Benralizumab.

• Mepolizumab.

• Reslizumab.

• Cyclosporin.

• Cyclophosphamide.

• Azathioprine.

• Hydroxychloroquine.

• Rituximab.

• Dapsone.

• Immunoglobulins.

Conclusion

Eosinophilic fasciitis, also called Shulman syndrome, is a rare skin disorder affecting the skin, subcutaneous fascia, underlying muscle, and bone to cause swelling, inflammation, and thick skin. Eosinophilic fasciitis is considered an autoimmune disorder and is also associated with blood disorders such as aplastic anemia and lymphoma. Treatment of eosinophilic fasciitis is done for months to years. The prognosis for eosinophilic fasciitis is good.