Eosinophilic fasciitis
Encyclopedia
Eosinophilic fasciitis also known as "Shulman's syndrome",
is a form of fasciitis
. It is distinguished from scleroderma
primarily because the affected area is the fascia
, not the dermis
as in scleroderma
. Also, unlike scleroderma, Raynaud's phenomenon
and telangiectasia
are not observed.
It was first characterized in 1974, and it is not yet known whether it is actually a distinct condition or just a different presentation. However, it remains used for diagnostic purposes.
Several cases have been reported after strenuous exercise.
less common features are joint pain and carpal tunnel syndrome.
s such as prednisone
, though other medications such as hydroxychloroquine
have also been used.
The pronostic is usually good in the case of an early treatment if there is no visceral involvement.
is a form of fasciitis
Fasciitis
In medicine, fasciitis refers to an inflammation of the fascia. In particular, it often refers to one of the following diseases:* Necrotizing fasciitis* Plantar fasciitis* Eosinophilic fasciitis* Paraneoplastic fasciitis...
. It is distinguished from scleroderma
Scleroderma
Systemic sclerosis or systemic scleroderma is a systemic autoimmune disease or systemic connective tissue disease that is a subtype of scleroderma.-Skin symptoms:...
primarily because the affected area is the fascia
Fascia
A fascia is a layer of fibrous tissue that permeates the human body. A fascia is a connective tissue that surrounds muscles, groups of muscles, blood vessels, and nerves, binding those structures together in much the same manner as plastic wrap can be used to hold the contents of sandwiches...
, not the dermis
Dermis
The dermis is a layer of skin between the epidermis and subcutaneous tissues, and is composed of two layers, the papillary and reticular dermis...
as in scleroderma
Scleroderma
Systemic sclerosis or systemic scleroderma is a systemic autoimmune disease or systemic connective tissue disease that is a subtype of scleroderma.-Skin symptoms:...
. Also, unlike scleroderma, Raynaud's phenomenon
Raynaud's phenomenon
In medicine, Raynaud's phenomenon is a vasospastic disorder causing discoloration of the fingers, toes, and occasionally other areas. This condition can also cause nails to become brittle with longitudinal ridges. Named for French physician Maurice Raynaud , the phenomenon is believed to be the...
and telangiectasia
Telangiectasia
Telangiectasias /tɛ.læn.dʒiː'ɛk.teɪ.ʃi:ə/ are small dilated blood vessels near the surface of the skin or mucous membranes, measuring between 0.5 and 1 millimeter in diameter. They can develop anywhere on the body but are commonly seen on the face around the nose, cheeks, and chin...
are not observed.
It was first characterized in 1974, and it is not yet known whether it is actually a distinct condition or just a different presentation. However, it remains used for diagnostic purposes.
Several cases have been reported after strenuous exercise.
Symptoms
As it is a rare disease, a clear set of symptoms is difficult to define. Usually, patients show severe pain and swelling are reported but clinical presentations vary. It can have a 'orange peel' like appearance.less common features are joint pain and carpal tunnel syndrome.
Treatment
Common treatments include corticosteroidCorticosteroid
Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex. Corticosteroids are involved in a wide range of physiologic systems such as stress response, immune response and regulation of inflammation, carbohydrate metabolism, protein catabolism, blood electrolyte...
s such as prednisone
Prednisone
Prednisone is a synthetic corticosteroid drug that is particularly effective as an immunosuppressant drug. It is used to treat certain inflammatory diseases and some types of cancer, but has significant adverse effects...
, though other medications such as hydroxychloroquine
Hydroxychloroquine
Hydroxychloroquine is an antimalarial drug, sold under the trade names Plaquenil,Axemal, Dolquine, and Quensyl, also used to reduce inflammation in the treatment of rheumatoid arthritis and lupus...
have also been used.
The pronostic is usually good in the case of an early treatment if there is no visceral involvement.