Eosinophilic Fasciitis (Shulman Syndrome)

Since the first description of Shulman syndrome in the literature, the condition has appeared in more than 100 articles, mostly of Anglo-Saxon origin. These articles have generated much debate regarding the condition's relation to scleroderma or its existence as an autonomous entity.¹¹ The condition should be set apart from all other sclerodermiform states. Scleredema adultorum (Buschke disease) is related to respiratory infection or to diabetes mellitus of long evolution. Scleredema adultorum presents a centrifugal evolution, beginning at the cervical region and root of the upper limbs, with half of the cases occurring during childhood or adolescence, and is twice as frequent in women.⁸ Mucin is generally evident at the beginning of the disease.¹² Myalgia-eosinophilia syndrome presents respiratory and neurologic symptoms associated with myalgia of sudden onset and is accompanied by a temporary cutaneous eruption that varies from maculopapular to urticarial. Myalgia-eosinophilia syndrome may present intense itching, and is related to the ingestion of the amino acid L-tryptophan.^13-16 Systemic scleroderma presents a universal induration of the skin accompanied by vasospastic phenomena in several organs with a variety of symptoms. Patients with acroesclerosis, a form of systemic scleroderma, present with calcinosis, Raynaud phenomenon, esophageal alterations, sclerodactyly, and telangiectasis (also known as CREST syndrome) on the face and upper trunk. The circumscribed forms are characterized by several types of cutaneous lesions, which are localized and seldom accompanied by other alterations.^17-20

Peripheral eosinophilia is a common finding in patients with eosinophilic fasciitis, presenting in more than 80% of cases; it also may occur in different forms in systemic sclerosis (7%) and in localized scleroderma (31%).^17,21 In nonmedicated patients with eosinophilic fasciitis, peripheral eosinophilia was a consistent finding, even in those who had the disease for more than 30 months. Most authors define eosinophilia as an eosinophil count of more than 600 cells/cm³; however, others have defined it as 400 or even 300 cells/cm³, with existence of a relationship between the peripheral and tissue count of eosinophils in most cases. In one study, eosinophilia above 1000 cells/mm³ was found in 61% of patients, but only 1% had systemic sclerosis and 8% had the localized form, indicating that peripheral eosinophilia is not only more frequent and intense but also guides the diagnosis.^17,21 Tissue eosinophilia is more variable than peripheral eosinophilia.

In some cases, the peripheral eosinophils are present in numbers within reference range in a certain blood sample, showing that the finding may be temporary. It is important to make the differential diagnosis between systemic sclerosis, morphea, and eosinophilic fasciitis because they not only have different prognoses but also have different treatments.^21,22 Eosinophilic fasciitis responds well to systemic corticoid therapy; in scleroderma, steroids are not always useful, and morphea can remit spontaneously. Absence of Raynaud phenomenon and induration of the limbs after intense and unusual exercise help to establish the diagnosis of eosinophilic fasciitis. Fascia damage can be found, though rarely, in the late phases of systemic sclerosis. Proliferative fasciitis represents a pseudosarcomatous reaction involving the muscular fascia and the subcutaneous fibrous septum; in addition, despite not presenting eosinophilia, proliferative fasciitis affects the extremities of adults and may have trauma as an etiological factor.¹⁹

The diagnosis of eosinophilic fasciitis may be confirmed by histopathologic examination, results of which show an inflammatory infiltrate with eosinophils extending to the muscular fascia.¹⁹ Tissue eosinophilia is defined as the presence of 3 or more cells in the microscopic field.¹⁶ The eosinophils are present in the entire damaged area from disease onset; however, this must not be confused with disseminated eosinophilic collagenosis, in which the cells infiltrate several organs, leading to focal necrosis and necrotizing endarteritis. When the biopsy result does not reach a diagnosis of hypodermis and fascia, the sclerotic aspect prevails, which is not characteristic of eosinophilic fasciitis. In the initial phases of eosinophilic fasciitis, there are no significant epidermal and dermal alterations; in scleroderma, there are variable degrees of edema and sclerosis, and the inflammatory process, when present, occurs in the dermis and in the upper portion of the subcutaneous cell tissue.^8,19,20