Eosinophilic fasciitis (EF) (EE-oh-SIN-oh-FIL-ik fashi-EYE-tis): This disease involves the fascia (FA-shuh), the thin connective tissue around the muscles, particularly those of the forearms, arms, legs, and trunk. EF causes the muscles to become encased in collagen, the fibrous protein that makes up tissue such as the skin and tendons. Permanent shortening of the muscles and tendons, called contractures, may develop, sometimes causing disfigurement and problems with joint motion and function. EF may begin after hard physical exertion. The disease usually fades away after several years, but people sometimes have relapses. Although the upper layers of the skin are not thickened in EF, the thickened fascia may cause the skin to look somewhat like the tight, hard skin of scleroderma. A skin biopsy easily distinguishes between the two.

Skin thickening on the fingers and hands: This also appears with diabetes, mycosis fungoides, amyloidosis, and adult celiac disease. It can also result from hand trauma.

Generalized scleroderma-like skin thickening: This may occur with scleromyxedema, graft-versus-host disease, porphyria cutanea tarda, and human adjuvant disease.

Internal organ damage: Similar to that seen in systemic sclerosis, this may instead be related to primary pulmonary hypertension, idiopathic pulmonary fibrosis, or collagenous colitis.

Raynaud's phenomenon: This condition also appears with atherosclerosis or systemic lupus erythematosus or in the absence of underlying disease.

An explanation of most of these other diseases is beyond the scope of this booklet. What's important to understand, however, is that diagnosing scleroderma isn't always easy, and it may take time for you and your doctor to do this. While having a definite diagnosis may be helpful, you do not need to know the precise form of your disease to receive proper treatment.

How Is Scleroderma Treated?

As we have discussed that many cases of scleroderma have infections so we need to get rid of the infection to help these patients. A test for CMV, EBV and Herpes antibodies should be done on all patients and if a infection is found it should be treated and . that a course of IVIg can be given for a month at least.

All patients should follow the cidpusa foundation diet guideline described in the diet chapter.

Use of a hulda clark zapper is very useful please see the electronic section for full details. Vibramycine 100 mg daily for two months is very useful.

Raynaud's Phenomenon: More than 90 percent of people with scleroderma have this condition, in which the fingers and sometimes other extremities change color in response to cold temperature or anxiety. For many, Raynaud's phenomenon precedes other manifestations of the disease. In other people, however, Raynaud's phenomenon is unrelated to scleroderma, but may signal damage to the blood vessels supplying the hands arising from occupational injuries (from using jackhammers, for example), trauma, excessive smoking, circulatory problems, and drug use or exposure to toxic substances. For some people, cold fingers and toes are the extent of the problem and are little more than a nuisance. For others, the condition can worsen and lead to puffy fingers, finger ulcers, and other complications that require aggressive treatment.

If you have Raynaud's phenomenon, the following measures may make you more comfortable and help prevent problems:

Don't smoke! Smoking narrows the blood vessels even more and makes Raynaud's phenomenon worse.
Dress warmly, with special attention to hands and feet. Dress in layers and try to stay indoors during cold weather.
Use biofeedback, which governs various body processes that are not normally thought of as being under conscious control, and relaxation exercises.
For severe cases, speak to your doctor about prescribing drugs called calcium channel blockers, such as nifedipine (Procardia^*), which can open up small blood vessels and improve circulation. Other drugs are in development and may become available.
If Raynaud's leads to skin sores or ulcers, increasing your dose of calcium channel blockers (under the direction of your doctor ONLY) may help. You can also protect skin ulcers from further injury or infection by applying nitroglycerine paste or antibiotic cream. Severe ulcerations on the fingertips can be treated with bioengineered skin.

*Brand names included in this booklet are provided as examples only, and their inclusion does not mean that these products are endorsed by the National Institutes of Health or any other Government agency. Also, if a particular brand name is not mentioned, this does not mean or imply that the product is unsatisfactory.

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