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Eosinophilic Fasciitis (Shulman Syndrome)

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We present a case of eosinophilic fasciitis, or Shulman syndrome, in a 35-year-old man and discuss its clinical and histopathologic aspects, as well as its relationship to scleroderma. Although controversial, the tendency is to set Shulman syndrome apart from all other sclerodermiform states.


 

References

Case Report

A 35-year-old white man visited our Sector of Dermatology complaining about a feeling of increased weight and volume in his arms and legs that had started 2 months previously. The symptoms had begun 48 hours after a vigorous and unusual physical effort—carrying 30- to 40-kg boxes during a more than 2.5-hour period. When the patient sought medical assistance, his problem was diagnosed as rheumatism. He was prescribed a nonsteroidal anti-inflammatory drug, with partial improvement. A few days later, his condition evolved to hand and wrist joint induration lasting more than 2 hours that was associated with a discrete disturbance to his normal way of walking.

The patient did not exercise regularly and denied the use of medication containing L-tryptophan. He did not report any systemic signs and symptoms such as fever, reduced weight, hyporexia, dyspnea, difficulty swallowing solids and liquids, previous upper respiratory tract infections, recent history of hepatitis, diabetes mellitus, or similar cases in the family.

Results of a physical examination showed loss of weight and skin blushing. The patient was hydrated, acyanotic, anicteric, feverless, and eupneic. His arterial pressure was 120x80 mm Hg, with ample peripheral pulses. The patient showed atypical facies without edema, with normal wrinkling for his gender and age and a mouth opening of 4.5 cm. His thorax and abdomen were without abnormalities. His upper and lower limbs had increased volume with infiltration varying from discrete to moderate, without sustained depressions at finger pressure except on the extremities. There was absence of Raynaud phenomenon. Results of an examination of articulations showed absence of edema or synovitis of the knees, ankles, wrists, and hands; mobility was free and painless.

Results of a dermatologic examination revealed visible sclerosis of limb skin, which was more evident at the extremities and diminished in intensity in the direction of the proximal region; in addition, the affected area had a discrete yellowish color, scarcity of hair, and telangiectasia (Figures 1 and 2).

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Results of laboratory tests showed intense eosinophilia (28%: a blood count of 2500 eosinophils in 8900 leukocytes); an increased erythrocyte sedimentation rate; and hypergammaglobulinemia (35.7%). His immunologic profile was evaluated for antinuclear factor, anti-DNA, antitopoisomerase, and anticentromere antibodies; all test results were negative. Antibodies for human immunodeficiency virus and indicators for hepatitis also were negative. Urine sediment and parasitologic stool examinations, as well as an x-ray of the thorax, did not reveal alterations. An incisional biopsy down to the muscular fascia was performed with a scalpel on the anterior surface of the right forearm.

Results of a histopathologic examination with hematoxylin-eosin stain revealed an inflammatory infiltrate of lymphocytes, plasma cells, and eosinophils around dermal vessels, mostly at the muscular fascia and adjacent adipose tissue (Figures 3 and 4). Results of direct immunofluorescence showed immunoreactivity to fibrinogen in some vessels of the superficial dermis; however, the results were negative for IgG, IgM, IgA, and C3, configuring an unspecific pattern of vascular reaction.

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The patient was diagnosed with eosinophilic fasciitis (Shulman syndrome), prescribed a therapeutic regimen of prednisone 20 mg/d (0.3 mg/kg) and diclofenac potassium 100 mg/d, and advised to avoid exercise and to rest. The patient's condition progressively improved, as did the cutaneous sclerosis, which improved faster in the upper limbs. After 6 months of prednisone with gradual withdrawal, examination results showed only a discrete sclerosis of the arms, forearms, thighs, and legs.

Comment

Shulman syndrome, first described in 1974 by Shulman,1 is characterized by a sudden onset of a symmetrical edema with induration at the extremities. In 1975, Rodnan et al2 proposed the name eosinophilic fasciitis after evaluating a laboratorial aspect of the disease. At that time, more than 200 cases were reported, some in nonhuman primates such as the rhesus monkey.3

Eosinophilic fasciitis is an inflammatory disease of unknown etiology that affects the muscular fascia and is characterized by a considerable increase of serous and tissue eosinophils, with hypergammaglobulinemia.4-6 This sclerodermiform syndrome presents clinical and histopathologic characteristics that allow a clinical distinction from scleroderma, despite that in some cases such differentiation may be difficult. Eosinophilic fasciitis mostly affects white men aged 30 to 70 years, frequently after the performance of vigorous and unusual physical effort. Patients complain about pain and edema with sudden onset and centripetal evolution starting at the extremities, evolving to induration that leads to limitation of hand and feet mobility.7,8 Occasionally, the face and abdomen also may be involved.9 An important dermatologic sign for the diagnosis is called the valley signal, which can be observed during extension and abduction of the arms, and corresponds to the linear depression following the vascular path of the area involved. The description of 6 cases in the presence of hematologic neoplasia has led some authors to believe that eosinophilic fasciitis is a manifestation of a paraneoplastic syndrome.10

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