what is shulmans syndrome

What is the pathophysiology of Shulman syndrome?

Aug 04, 2021 · Eosinophilic fasciitis is sometimes referred to as Shulman's syndrome. What is fascia? The fascia is a sheet or band of fibrous connective tissue under the skin that covers a surface of underlying tissues. Fascia surrounds each of the muscles that move the skeleton. When the fascia is inflamed, the condition is referred to as "fasciitis."

What is eosinophilic fasciitis (Shulman syndrome)?

[Shulman's syndrome (eosinophilic fasciitis)] Eosinophilic fasciitis is a rare disease characterized by edema, painful indurations, and progressive muscle weakness. Mainly the extremities are involved. We report on a 22-year-old woman with eosinophilic fasciitis presenting with progressive muscle weakness of both hands and feet and a reduced ge …

Can prednisone and diclofenac be used to treat Shulman syndrome?

Nov 15, 2021 · Eosinophilic fasciitis (/ˌiːəˌsɪnəˈfɪlɪk ˌfæʃiˈaɪtɪs, ˌiːoʊ-, -ˌfæsi-/), also known as Shulman’s syndrome, is an inflammatory disease that affects the fascia, other connective tissues, surrounding muscles, blood vessels and nerves. What does eosinophilic fasciitis do to …

How is scleromyxedema differentiated from Churg-Strauss syndrome?

Eosinophilic fasciitis (EF) or Shulman syndrome, is a scleroderma-like syndrome with an early phase characterized by erythema and edema distributed over the limbs or trunk and a later phase, dominated by collagenous thickening of the subcutaneous fascia. Eosinophilic fasciitis associated with factor V Leiden: a case report More results

What are the signs and symptoms of eosinophilic fasciitis?

SymptomsTenderness and swelling of the skin on the arms, legs, or sometimes the joints (most often on both sides of the body)Arthritis.Carpal tunnel syndrome.Muscle pain.Thickened skin that looks puckered.Jan 31, 2021

What triggers 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.

Can eosinophils cause joint pain?

Additional symptoms that often occur during this earlier phase of eosinophilia-myalgia syndrome include cough, fever, fatigue, joint pain (arthralgia), swelling due to the abnormal accumulation of fluid (edema), and a sensation of numbness or tingling, most often in the hands, feet, arms or legs.

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.

How is eosinophilic fasciitis diagnosed?

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].

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

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.

What causes induration?

Skin induration is a deep thickening of the skin that can result from edema, inflammation, or infiltration, including by cancer. Diagnosis of skin induration is made by palpation (feeling the area) and assessing whether the raised area has a hard, resistant feeling.Nov 24, 2021

What does eosinophilic fasciitis do to the body?

Eosinophilic fasciitis causes inflammation of the tissues beneath the skin as well as sometimes in the skin. This leads to symptoms of swelling, stiffness, warmth, and pain of the involved area. Occasionally, there is discoloration of the skin over the tissues affected and the skin can appear thicker than normal.

How long does eosinophilic fasciitis last?

In most cases, the condition goes away within 1 to 3 years. However, symptoms may last longer or come back.

Is eosinophilic fasciitis an autoimmune disease?

Eosinophilic fasciitis is a rare autoimmune rheumatic disorder. Many autoimmune disorders affect connective tissue and a variety of organs. Connective tissue read more in which the skin and tissue that lies beneath the skin become painfully inflamed and swollen and gradually harden in the arms and legs.

Is eosinophilic fasciitis painful?

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.

How can I reduce my eosinophil count?

Glucocorticoids are the most effective current therapy used to reduce eosinophil numbers in the blood and tissue (Table 1), but the pleiotropic effects of corticosteroids can result in potentially harmful side effects and limit their therapeutic use.

What is eosinophilic dermatitis?

Eosinophilic dermatoses are a heterogeneous group of diseases, characterized by an eosinophil-rich infiltrate and/or degranulation of eosinophils. Blood eosinophilia may be an associated feature.

Is scleroderma always fatal?

It is the most fatal of all the rheumatologic diseases. Systemic scleroderma is very unpredictable although most cases can be classified into one of four different general patterns of disease (see Classification).

What are the symptoms of carpal tunnel syndrome?

Signs and symptoms. Because the disease is rare and clinical presentations vary, a clear set of symptoms is difficult to define. Severe pain and swelling are often reported, and skin can resemble orange peel in appearance. Less common features include joint pain and carpal tunnel syndrome.

What is the name of the disease that affects the fascia?

Rheumatology. Eosinophilic fasciitis ( / ˌiːəˌsɪnəˈfɪlɪk ˌfæʃiˈaɪtɪs, ˌiːoʊ -, - ˌfæsi -/ ), also known as Shulman's syndrome, is an inflammatory disease that affects the fascia, other connective tissues, surrounding muscles, blood vessels and nerves.

When was scleroderma first diagnosed?

The condition was first characterized in 1974, but it is not yet known whether it is actually a distinct condition or merely a variant presentation of another syndrome. The presentation is similar to that of scleroderma or systemic sclerosis.

What is the name of the disease that is characterized by hardening of the skin?

Eosinophilic fasciitis, also known as Shulman syndrome, is named after the physician who, in 1974, was the first to report on the disorder in the medical literature. Some researchers believe that eosinophilic fasciitis is a variant of scleroderma (systemic sclerosis), an autoimmune connective tissue disorder characterized by hardening of the skin.

Can scleroderma cause fatigue?

In some cases, localized areas of scleroderma (morphea) may develop. Some affected individuals may develop nonspecific symptoms including fatigue, weight loss, fever, and a general feeling of ill health (malaise) or a general lack of strength (asthenia).

When was Shulman syndrome first described?

Shulman syndrome, first described in 1974 by Shulman, 1 is characterized by a sudden onset of a symmetrical edema with induration at the extremities. In 1975, Rodnan et al 2 proposed the name eosinophilic fasciitis after evaluating a laboratorial aspect of the disease.

How long did it take for a white man to feel a swollen arm?

A 35-year-old white man visited our Sector of Dermatology complaining about a feeling of increased weight and volume in his arms and legs that had started 2 months previously. The symptoms had begun 48 hours after a vigorous and unusual physical effort—carrying 30- to 40-kg boxes during a more than 2.5-hour period. When the patient sought medical assistance, his problem was diagnosed as rheumatism. He was prescribed a nonsteroidal anti-inflammatory drug, with partial improvement. A few days later, his condition evolved to hand and wrist joint induration lasting more than 2 hours that was associated with a discrete disturbance to his normal way of walking.

Overview

Eosinophilic fasciitis , also known as Shulman's syndrome, is an inflammatory disease that affects the fascia, other connective tissues, surrounding muscles, blood vessels and nerves. Unlike other forms of fasciitis, eosinophilic fasciitis is typically self-limited and confined to the arms and legs, although it can require treatment with corticosteroids, and some cases are associated with aplastic anemia.

Signs and symptoms

Cause

Diagnosis

Treatment

Epidemiology

See also

Further reading