Case Presentation: A 50-year-old man presented with one week of diarrhea. He reported ten voluminous watery bowel movements each day, nausea, and frequent non-bloody, non-bilious emesis. Additional symptoms included increasing right-sided abdominal pain, generalized pruritus, and diffuse weakness. Eating meat exacerbated both his abdominal discomfort and pruritus. His history included Ulcerative Colitis, Primary Sclerosing Cholangitis, Clostridium difficle treated via total colectomy with ileoanal pouch, and treated disseminated Mycobacterium avium complex. He had previously travelled to both rural Indonesia and Bermuda within the past five years. Despite normal vital signs, he appeared uncomfortable with diffuse abdominal tenderness elicited on palpation without peritoneal signs and a blanching erythematous rash overlying his sternum. Laboratory analyses revealed a white blood cell count of 17.7 with absolute neutrophil count of 11,830, and absolute eosinophil count of 3,510, a sodium of 132, and creatinine of 2.0, as well as normal lactate and inflammatory markers. Stool studies, including Clostridium difficle toxin, common bacteria and viruses associated with diarrhea, and ova and parasites, yielded negative results. CT scan of the abdomen and pelvis revealed no acute processes. Further labs revealed low IgG, normal tryptase, normal T- B- and Natural killer cell levels, negative IgE alpha gal, and negative Strongyloides IgG. At this time, his abdominal pain intensified, diarrhea became more frequent, rash spread, and absolute eosinophil count rose to 7,900. Colonoscopy found patchy erythematous mucosa in the mid-ileum, and enteroscopy discovered the same patchy erythematous mucosa throughout the visualized section of small bowel. Tissue biopsies of the duodenum, ileum, and rectal pouch each revealed prominent mucosal eosinophilia. Treatment commenced with intravenous steroids, and his abdominal pain, rash, and eosinophilia significantly improved.
Discussion: Hypereosinophilic syndrome, while uncommon, is a clinically significant and potentially lethal multi-system disorder. Each presentation is unique, dependent upon the organ systems affected and degree of severity. Most often, eosinophil-mediated damage affects the skin, heart, lungs, brain, and gastrointestinal system, including enteritis and colitis. While acute diarrhea is most often infectious in origin, this patient’s negative infectious workup and lack of clinical improvement with supportive care required a more comprehensive evaluation, including critical analysis of the white blood cell differential and unexplained rash. Given the severity of eosinophil mediated organ damage, a repeat blood draw one month later to prove hypereosinophilic syndrome is not practical. Rather, an aggressive and comprehensive workup of hypereosinophilia is required. Following the exclusion of alternative etiologies, one should pursue tissue biopsy of the affected organs. However, once pathology confirms the presence of eosinophils, it is imperative to ensure that Strongyloides has been excluded by ELISA testing before initiating treatment with steroids. If not, introduction of an immunosuppressed state would enable Strongyloides to cause a potentially life-threatening infection.
Conclusions: This case highlights the needs to critically evaluate the white blood cell differential, obtain tissue to confirm hypereosinophilic syndrome, and rule out Strongyloides via ELISA prior to treatment with intravenous steroids.