What is fasciitis and how many types of it?
Nov 12, 2015 · Eosinophilic fasciitis is a very rare condition in which muscle tissue underneath the skin, called fascia, becomes swollen and thick. Rapid swelling can occur in the hands, arms, legs, and feet. People with this condition have a buildup of eosinophils, a type of white blood cell, in the affected fascia and muscles.
What it is like, living with eosinophilic esophagitis?
Apr 30, 2015 · Eosinophilic fasciitis is a rare sclerodermiform syndrome of unknown etiology. It is characterized by the thickening of the muscular fascia and subcutaneous tissue, with a variable infiltration of eosinophils. Peripheral eosinophilia, poly or monoclonal hypergammaglobulinemia and increased erythrocyte sedimentation rate can be seen.
What causes Eosinophilic folliculitis?
Is eosinophilic esophagitis contagious?
What causes eosinophilic fasciitis?
Causes. The exact cause of eosinophilic fasciitis is unknown (idiopathic). Researchers believe that the disorder results due to a nonspecific triggering event that causes an abnormal immune system response, specifically an abnormal allergic or inflammatory reaction.
What is the treatment for eosinophilic fasciitis?
Treatment of eosinophilic fasciitis is directed at eliminating the tissue inflammation and includes aspirin, other anti-inflammatory drugs (NSAIDs), and cortisone. Many patients will improve spontaneously. Others can be afflicted with persistent tissue and joint pain, in addition to thickening of the involved tissues.
What does eosinophilic fasciitis do to the body?
The usual initial symptoms of eosinophilic fasciitis are pain, swelling, and inflammation of the skin, particularly over the inside of the arms and the front of the legs. The skin of the face, chest, and abdomen may occasionally be affected. Typically, the fingers and toes are not affected.
Is eosinophilic fasciitis an autoimmune disease?
What is the outcome for eosinophilic fasciitis? Eosinophilic fasciitis can be associated with or progress to morphoea or other autoimmune connective tissue disorders in 20–30% of cases. Associated haematological disorders such as aplastic anaemia and lymphoma have been reported.
Is eosinophilic fasciitis curable?
In most cases, the condition goes away within 1 to 3 years. However, symptoms may last longer or come back.Jan 31, 2021
What causes fasciitis in the body?
Fascia-Related Muscle Pain and Stiffness Factors that cause fascia to become gummy and crinkle up (called adhesion) include: A lifestyle of limited physical activity (too little movement day after day) Repetitive movement that overworks one part of the body. Trauma such as surgery or injury.
How many cases of eosinophilic fasciitis are there?
Since 1974, over 300 patients with eosinophilic fasciitis have been reported. Despite this, the current understanding of the disease relies on a relatively few large case series and multiple case reports. Therefore, the understanding of key aspects of the disease continues to evolve.Dec 24, 2020
How do you diagnose scleroderma?
To diagnose scleroderma, a doctor usually begins by asking you about your symptoms, health, and medical history. The doctor will also examine your skin for signs of hardening and thickening. If you have hard, thickened skin, a dermatologist may perform a skin biopsy to help diagnose you.
What are the symptoms of eosinophilic gastroenteritis?
Eosinophilic gastroenteritis may affect any part of the gastrointestinal tract from the esophagus to the rectum. Symptoms include dysphagia (sometimes presenting as food impaction), heartburn, abdominal pain, nausea, vomiting, diarrhea, weight loss, and bloating (ascites is possible).
Is eosinophilic fasciitis rare?
Summary. Eosinophilic fasciitis is a very rare condition in which muscle tissue underneath the skin, called fascia, becomes swollen and thick. Rapid swelling can occur in the hands, arms, legs, and feet.
What drugs can cause eosinophilia?
Asymptomatic eosinophilia has been associated most often with quinine, penicillins, cephalosporins, or quinolones. Pulmonary infiltrates with peripheral eosinophilia have been particularly associated with NSAIDs, sulfas, and nitrofurantoin.
Does eosinophilia cause fatigue?
Common symptoms include muscle pain (myalgia), muscle weakness, cramping, skin rashes, difficulty breathing (dyspnea) and fatigue. Affected individuals have elevated levels of certain white blood cells known as eosinophils in the various tissues of the body, a condition known as eosinophilia.
What is eosinophilic fasciitis?
Eosinophilic fasciitis, also called Shulman syndrome, is a rare variant of scleroderma or deep form of morphoea involving the subcutis and fascia of the skin characterised by fascial thickening with an eosinophilic tissue infiltrate and peripheral eosinophilia.
Who gets eosinophilic fasciitis?
Eosinophilic fasciitis usually affects adults aged 20–60 years, although there are reports in children and the elderly. Both sexes can be affected, with a slight male predominance (1.5:1). Occurrence in siblings has been reported but it is not regarded as an inherited condition.
What causes eosinophilic fasciitis?
Eosinophilic fasciitis is possibly the result of an autoimmune mechanism targeting damaged fascia. Autoantibodies including rheumatoid factor (RF) and antinuclear antibodies (ANA) are sometimes detected.
What are the clinical features of eosinophilic fasciitis?
Eosinophilic fasciitis typically develops suddenly 1–2 weeks after strenuous exercise or work; less commonly the onset is gradual.
How is eosinophilic fasciitis diagnosed?
Eosinophilic fasciitis should be considered on the typical history and examination, and confirmed on deep skin biopsy including subcutis, fascia, and muscle [see Eosinophilic fasciitis pathology ].
What is the outcome for eosinophilic fasciitis?
Eosinophilic fasciitis can be associated with or progress to morphoea or other autoimmune connective tissue disorders in 20–30% of cases. Associated haematological disorders such as aplastic anaemia and lymphoma have been reported.
Prognosis
- Although researchers have been able to establish a clear syndrome with characteristic or core symptoms, much about eosinophilic fasciitis is not fully understood. Several factors including the small number of identified cases, the lack of large clinical studies, and the possibility of other unknown influencing factors prevent physicians from developing an accurate picture of associat…
Symptoms
- The onset of eosinophilic fasciitis is often sudden (acute), developing over a few days or weeks. In these cases, the disorder may progress rapidly. Less often, a less severe form of disorder that progresses more slowly can develop. In many cases, an episode of eosinophilic fasciitis follows strenuous physical exercise or activity. Specific symptoms and severity can vary greatly from on…
Signs and symptoms
- Initial symptoms associated with eosinophilic fasciitis include pain and swelling and inflammation of the skin, especially of the arms and legs. The arms and forearms are affected more often than the legs and thighs. The hands and feet are usually unaffected. Affected areas may initially become tender. Affected individuals may develop characteristic shallow grooves or furrows in t…
Pathophysiology
- Some scientists believe that eosinophilic fasciitis may be a variant of scleroderma or morphea (localized scleroderma). These rare disorders are characterized by the hardening and thickening of skin and surrounding tissue, often due to the malfunction of the immune system.
Clinical significance
- Some cases of eosinophilic fasciitis have been related to the ingestion of a dietary supplement known as L-tryptophan. Tryptophan is an essential amino acid found in numerous foods including poultry and was used as a sleep aid or to treat depression. In 1989 L-tryptophan ingestion was linked to an epidemic of a disorder known as eosinophilia-myalgia syndrome. (For more informa…
Classification
- Some researchers believe eosinophilic fasciitis is one of several disorders that should be grouped under the designation fasciitis-panniculitis syndrome (FPS). Disorders under this designation are characterized by hardening and thickening of the skin due to inflammation and fibrosis.
Causes
- In some cases, eosinophilic fasciitis can be associated with another disorder such as acquired aplastic anemia, hemolytic anemia, myelodysplastic syndromes, myeloproliferative disorders, lymphoma, leukemia, thyroid disorders, and primary biliary cirrhosis. The relationship between these disorders and eosinophilic fasciitis, if any, is not completely understood. (For more inform…
Epidemiology
- Eosinophilic fasciitis affects both sexes. Some reports suggest that women are affected with greater frequency than men. The disorder can occur at any age (with age at diagnosis ranging from 1-88), but most often occurs in individuals between 30-60 years of age. It occurs with greater frequency in Caucasians. Approximately 300 cases have been reported in the medical literature.
Diagnosis
- Symptoms of the following disorders can be similar to those of eosinophilic fasciitis. Comparisons may be useful for a differential diagnosis. Eosinophilia-myalgia syndrome occurred as an epidemic in 1989 associated with the ingestion of contaminated L-tryptophan, a dietary supplement widely sold at that time. The contaminant remains unknown. This syndrome abruptl…
Toxicity
- The Spanish toxic oil syndrome due to ingestion of contaminated rapeseed oil occurred as an epidemic in 1981 and caused skin involvement similar to eosinophilic fasciitis. The epidemic abruptly ceased once the contaminated rapeseed oil was no longed consumed.
Terminology
- Eosinophilic disorder is a general term for any disorder characterized by infiltration of the skin and tissue by a certain type of white blood cell called eosinophils, including disease resulting from arthropod bites, infections, and drug reactions. Churg-Strauss syndrome, hypereosinophilic syndrome and eosinophilic cellulitis are examples of disorders characterized by elevated levels …
Treatment
- Treatment The treatment of eosinophilic fasciitis is directed toward preventing and alleviating tissue inflammation. In some cases, affected individuals improve without treatment (spontaneous remission). Many individuals respond favorably to corticosteroid therapy, and the drug prednisone is often prescribed. Prednisone therapy may be required for two months or longer. In many case…
Resources
- Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov . All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
Literature
- Li JY. Eosinophilic Fasciitis. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:8.
Selected publications
- De Masson A, Bouaziz JD, Peffault de Latour R, et al. Severe aplastic anemia associated with eosinophilic fasciitis: report of 4 cases and review of the literature. Medicine (Baltimore). 2013;92:69-81. http://www.ncbi.nlm.nih.gov/pubmed/23429351 Lebeaux D, Frances C, Barete S, et al. Eosinophilic fasciitis (Shulman disease): new insights into the therapeutic management fro…
Other sources
- Samona J. Orthopedic considerations with eosinophilic fasciitis: a case report and literature review. Case Report Orthop. 2012;2012:865360. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3504250/
Publication
- Antic M, Lautenschlager S, Itin PH. Eosinophilic fasciitis 30 years after what do we really know? Report of 11 patients and review of the literature. Dermatology. 2005;213:93-101. http://www.ncbi.nlm.nih.gov/pubmed/16902285
Classification
Cause
- The cause of eosinophilic fasciitis is unknown but it may have something to do with abnormal immune responses as hypergammaglobulinaemia and antinuclear antibodies are present. In addition, toxic, environmental, or drug exposures have been implicated.
Epidemiology
- It affects females and males, children and adults, with most cases occurring between the ages of 30 and 60 years.
Signs and symptoms
- Patients usually present suddenly with painful, tender, swollen and red extremities. Within weeks to months patients develop stiffness and affected skin becomes indurated, creating a characteristic orange-peel appearance over the surfaces of the extremities. In severely affected areas, the skin and the subcutaneous tissue are bound tightly to the underlying muscle. This cre…
Diagnosis
- Laboratory studies in early active disease show eosinophilia, elevated ESR and polyclonal immunoglobulin (Ig)G. However, a full-thickness skin biopsy that includes the dermis, subcutaneous fat and fascia is necessary to confirm a diagnosis of eosinophilic fasciitis, which has specific pathological features.
Treatment
- Treatment of eosinophilic fasciitis is directed at preventing tissue inflammation. Oral corticosteroids are the mainstay of treatment, with most patients responding well to moderate-to-high doses of corticosteroids particularly if started early in the course of the disease. Continued low doses may be required for 2-5 years. In some cases, the disease may resolve spontaneously…
Management
- A physiotherapist is also important in the overall management of eosinophilic fasciitis to prevent and treat joint contractures.