History
The case definition of EMS is useful to identify patients with suspected EMS; however, to ensure a more accurate diagnosis, a more stringent set of criteria must be applied. One set of proposed classification criteria includes 4 axes: (1) the presence of a distinct acute episode with the typical signs and symptoms; (2) major physical findings, including typical involvement of organs, such as the skin, the muscles, the lungs, and the nerves; (3) characteristic laboratory values, including an eosinophil count greater than 1.0 X 109 cells/L; and (4) characteristic histopathologic features.
Acute episode
The acute episode is characterized by shortness of breath; cough; fever; debilitating fatigue; arthralgias; paresthesias; severe weakness; muscle cramps; periorbital and peripheral edema; skin hypersensitivity; and a generalized erythematous, maculopapular, or blotchy erythematous rash.
After this acute episode, most patients have more chronic symptoms that involve several organ systems.
Cutaneous involvement
Cutaneous involvement occurs in 60% of patients with EMS. After the initial symptoms, this is the most prominent feature of the disease.
In the acute phase of the disease, patients often have a diffuse eruption with pruritus and swelling.
Later in the disease, patients may experience skin tightening, which reflects sclerodermoid changes. However, unlike scleroderma, the fingers and the toes are almost always spared, and the Raynaud phenomenon is usually absent.
Patients may note an inability to tolerate even light touch. This finding tends to be more pronounced in the lower extremities than elsewhere.
Alopecia is also noted in more than one quarter of patients.
Muscular involvement
By definition, patients must have myalgia. This condition usually begins in the proximal muscle groups, such as those in the shoulders, the buttocks, and the thighs; then, myalgia becomes incapacitating.
Patients may have stiffness and aches in the affected muscles, as well as muscle cramps, particularly during exercise.
Patients also complain of weakness and muscle wasting that limit their ability to walk or lift heavy objects.
Myalgia in the jaw can lead to pain in the facial muscle or the jaw.
Nervous system involvement
Central nervous system and peripheral nervous system involvement is seen in 27% of patients, in whom disorders of these systems are the presenting features.
Patients may have decreased sensation, particularly in the hands, or hyperesthesia in the back and the extremities.
Patients may present with weakness, cognitive deficits, or bladder dysfunction.
Pulmonary involvement [25, 26]
Pulmonary symptoms are the major presenting complaints in the acute phase of EMS.
Patients report rapidly progressive shortness of breath associated with a nonproductive cough and other symptoms related to upper respiratory tract infections.
Patients may have chest tightness, pleuritic chest pain, or dyspnea on exertion.
Cardiac involvement
Most patients do not have heart problems; however, pericarditis, myocarditis, and cardiac arrhythmias are known complications of the disease.
Patients with arrhythmias may have palpitations.
Gastrointestinal involvement [27, 28]
Gastrointestinal problems are not common problems in this disease.
Some patients experience abdominal pain, nausea, vomiting, diarrhea, and weight loss.
Rheumatologic involvement
About 73% of patients have joint pain.
This pain can be located in the wrist, the knees, the ankles, the shoulders, the hips, the spine, or the interphalangeal and metacarpophalangeal joints.
Physical Examination
Cutaneous manifestations [29, 30, 31]
A diffuse eruption consisting of erythematous macules and papules often develops over the trunk and the extremities. The skin may have a mottled appearance and occasionally appears ecchymotic. No palpable purpura is evident.
Four weeks to 4 months after the onset of myalgias, a progressive peau d'orange–type induration may develop (see the image below). This process tends to start in the distal part of the lower and upper extremities and gradually moves proximally. The digits are characteristically spared. The skin classically appears firm, shiny, and hide-bound.
Indurated edematous plaques on a patient with hypereosinophilic syndrome.
Sometimes, venous furrowing of the uplifted arm is observed.
Neuromuscular manifestations [32]
Neuromuscular examination of patients with EMS reveals weakness and paresthesias. Some patients have cutaneous hyperesthesia (the inability to tolerate touch). This finding tends to be more pronounced in the lower extremities.
Muscles are often tender to palpation.
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Indurated edematous plaques on a patient with hypereosinophilic syndrome.
