History
Eosinophilic fasciitis patients present with the sudden onset of painful, tender, edematous, and erythematous extremities. The disorder progresses rapidly; within weeks to months, patients develop stiffness and sclerodermatous induration, resulting in characteristic flexion contractures and impaired mobility. The forearms, the upper arms, the lower legs, the thighs, and the trunk are involved (in order of decreasing frequency).
As many as 50% of patients report an episode of strenuous physical exercise or activity immediately preceding the onset of the illness.
Malaise, weakness, and fever are frequently present. A study in 2008 addressing the physical burden noted that fatigue was the most common symptom. Pain and itch were also frequent complaints. Of all patients, 62% noted fatigue, pain, and itch. [11]
Overt arthritis occurs in as many as 40% of patients. Symptoms of carpal tunnel syndrome have been reported. Visceral involvement and Raynaud phenomenon are rare.
Physical Examination
The clinical presentation of eosinophilic fasciitis evolves through 3 stages; the various stages present simultaneously in different areas of the body. The first stage presents with symmetric, diffuse, erythematous tenderness of the extremities, followed by an edematous phase that produces a coarsely dimpled appearance (cobblestoning) or a finely dimpled appearance (peau d'orange). The last phase involves rippling of the skin with areas of hypopigmentation, induration, and skin tightness.
Lower back part of the legs shows hypopigmentation, induration, biopsy site, and asymmetric involvement.
Close-up view of left posterior thigh 2 weeks later shows erythema, scaling, alopecia, and rippled induration.
Posterior part of the calf in the first week of illness shows erythema, edema, alopecia, scaling, and early induration. The right calf is relatively uninvolved with patchy erythema only.
Photograph of the posterior part of the calf at 3 weeks shows complete sclerosis and induration with patchy erythema.
In severely affected areas, both the skin and the subcutaneous tissues are bound-down and inseparable from the underlying muscle, and they have a woody-type appearance. With elevation of the involved extremities, furrows along the course of the superficial veins may be present; this finding is referred to as the groove sign. Although the extremities are preferentially involved (88%), the trunk may be involved. The hands, the feet, and the face are spared.
Joint contractures of the elbows, wrists, ankles, knees, and shoulders may be found in 55-75% of patients. Unilateral involvement has been reported. [12] Carpal tunnel syndrome is present in 20% of patients. Inflammatory arthritis is present in as many as 40% of patients.
Subclinical myositis is present in a minority of patients.
A concurrent localized lesion of morphea may be seen in 25% of patients.
In contrast to scleroderma, Raynaud phenomenon, abnormal nail fold capillaries, and sclerodactyly are not present. Visceral involvement is rare, with few reports of involvement of the lungs, the esophagus, and the myocardium.
Primary presentation as angioedema was also reported in a patient. [13]
Complications
Aplastic anemia and other forms of myelodysplasia may complicate eosinophilic fasciitis in as many as 10% of patients. Some authors advocate a bone marrow examination in all eosinophilic fasciitis patients. More recent case reports have shown associations of eosinophilic fasciitis with multiple myeloma, polycythemia vera, peripheral T-cell lymphoma, immunoglobulin A nephropathy, and idiopathic hypercalcemia.
An additional report in 2103 documented a patient with eosinophilic fasciitis who developed moderate aplastic pancytopenia during treatment. Late sequela 2 years later included onset of paroxysmal nocturnal hemoglobinuria and persistent stable pancytopenia. [14]
Associations have also been noted with systemic lupus erythematosus, [15] hyperthyroidism with thyroid adenoma, [16] and primary biliary cirrhosis. [17]
A case report in 2012 describes a 30-year-old woman with eosinophilic fasciitis who also presented with a painful sensory neuropathy in her legs, with resultant functional incapacity. It was thought the multiple mononeuritis was related to neurotoxicity from secreted eosinophil products. Treatment with corticosteroids and gabapentin improved the neuropathic symptoms. [18]
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Lower back part of the legs shows hypopigmentation, induration, biopsy site, and asymmetric involvement.
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Posterior thigh shows woody induration, sclerosis, and hypopigmentation.
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Close-up view of left posterior thigh 2 weeks later shows erythema, scaling, alopecia, and rippled induration.
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Posterior part of the calf in the first week of illness shows erythema, edema, alopecia, scaling, and early induration. The right calf is relatively uninvolved with patchy erythema only.
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Photograph of the posterior part of the calf at 3 weeks shows complete sclerosis and induration with patchy erythema.
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Note the marked thickening and replacement of the entire dermis with sclerotic collagen on this incisional biopsy sample from the left posterior part of the thigh.
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Photomicrograph of subcutaneous fat-fascia junction shows entrapment of subcutaneous fat by intersecting thick bands of fibrosis. Thickening and fibrosis of fascia and lymphoid aggregates are seen.
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Photomicrograph of fascia-skeletal muscle junction shows markedly thickened fascia with heavy inflammatory infiltration.
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High-power photomicrograph of fascia shows heavy inflammatory infiltration with numerous eosinophils, lymphocytes, and occasional plasma cells.
