Orthopedic Considerations with Eosinophilic Fasciitis: A Case Report and Literature Review

Abstract

Eosinophilic fasciitis (EF) or Shulman's disease is a very rare condition first described in 1974 by Dr. Shulman in patients with diffuse fasciitis and eosinophilia. Fewer than 300 cases have been reported worldwide in the past 35 years. The current understanding of the disease in the medical community relies only on a few large case series and multiple case reports. The proposed etiology, pathological mechanisms, and consensus for therapy are obscure or lacking. The presentation of the disease is variable, but certain signs and symptoms have been associated with EF. The extreme rarity of the disease, the large constellation of signs and symptoms, as well as the lack of knowledge about eosinophilic fasciitis and make this disease difficult to recognize and treat. Through the review of the literature, there is only one other case by Yamanishi where recurrent asthma has been seen to be associated with eosinophilic fasciitis. To the knowledge of the authors of this paper this patient represents the second recorded incident. The case described by the authors of this paper demonstrated an initial biopsy of mixed cell fasciitis including eosinophils, compared to the eosinophil-rich sample taken at a later date. This could be a unique aspect to the pathology of the disease not previously discovered. Similar scenarios were not noted in a review of the literature. A change in the pathological findings as shown in this case from non-eosinophil-rich sample to one rich in eosinophils is unique in a patient actively suffering from EF. The authors of this paper propose that an allergic reaction (at the patient's puncture site) occurred, which initially caused the left hand symptoms that led to the patient's first presentation to the hospital. This is a unique causative agent, not found in the review of the literature. Through a review of the literature and the presentation of this patient, the authors propose an underlying dysregulation of the immune system, leading to the initiation or synergistic perpetuation of EF. This is a unique outlook on the disease pathology, not explained much in the medical literature.