What Is Eosinophilic Fasciitis (Shulman's Syndrome)?

• Eosinophils are a particular type of white blood cells, usually representing a small percentage (less than 8% of the total white blood cell population) that are easily stained by eosin and other dyes; they have a characteristic double-lobed nucleus.
• The number of these cells (eosinophil count) increases in certain illnesses, includingallergies,asthma, Addison's disease,sarcoidosis, parasite infections, drug reactions, and connective tissue diseases (such asrheumatoid arthritisandscleroderma).
• Eosinophilic fasciitis is sometimes referred to as Shulman's syndrome.

• 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."

• Although the cause seems related to an inflammatory response, the agent(s) that trigger the response are not yet identified.
• In the 1980s, there was a toxic product in some lots ofL-tryptophan, anover-the-countersleepaide that was available at the time, which caused illness similar to eosinophilic fasciitis.

• 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, andpainof the involved area. Occasionally, there is discoloration of the skin over the tissues affected and the skin can appear thicker than normal. Joint contractures occur in 50%-75% of patients.
• The muscle of the involved area can become weakened. Muscle enzyme blood levels can be found to be elevated in the blood, particularly the enzymes aldolase and creatine phosphokinase (CPK).

• The diagnosis of eosinophilic fasciitis is made with askin biopsyof a full thickness of involved deep skin tissue.
• The biopsy site is usually small, and the doctor numbs the area before the tissue is removed for study by a pathologist, dermatologist, or trained technician. In addition, the thickened fascia can be detected byMRI.

• Treatment of eosinophilic fasciitis is directed at eliminating the tissue inflammation and includesaspirin, other anti-inflammatorydrugs(NSAIDs), and cortisone. Many patients will improve spontaneously. Others can be afflicted with persistent tissue andjoint pain, in addition to thickening of the involved tissues.
• For aggressive eosinophilic fasciitis, cortisone medications (such asprednisoneandprednisolone) are sometimes initially administered intravenously. Also considered are immune-suppression medications (such asmethotrexate[Rheumatrex,Trexall],cyclophosphamide[Cytoxan], andpenicillamine[Depen,Cuprimine]). More recently,mycophenolate mofetil(Cellcept) andrituximab(Rituxan) are being studied as potential therapies.
• Medical research has shown that immune-suppression drugs, such as methotrexate, can reduce both the immune inflammation and the need for continued cortisone medications.

• 嗜酸性筋膜炎是由pediatr治疗icians, internists, dermatologists, andrheumatologists; occasionally, surgeons are consulted for deep biopsies and joint contractures.

• The outlook for eosinophilic fasciitis is generally good, particularly if treated aggressively and early.
• Along with medications, physical therapy can be required for optimal rehabilitation.
• Poor function, however, is not uncommon, especially in children.
• Those witharthritis, atrophy of muscle, scarring limiting the joint range of motion (contracture), and shortening of limb length tend to do worse.

Because we do not yet know the cause of eosinophilic fasciitis, it cannot be prevented.