Abstract Eosinophilic cellulitis (Well's syndrome) is a rare relapsing inflammatory disorder with ∼150 cases reported worldwide, characterized by eosinophilic dermal infiltration. HLH is a life-threatening syndrome of immune overactivation, often triggered by infection, malignancy, or autoimmune disease. We present a case of hemophagocytic lymphohistiocytosis (HLH) developing in a patient with a history of Well’s syndrome. This case highlights the need for early HLH recognition and its potentially fatal consequences when diagnosis is delayed. A 50-year-old female with eosinophilic cellulitis, endometrial hyperplasia, GERD, and LCIS presented with fever, dysuria, abdominal pain, and foul-smelling urine on post-op day 2 following elective hysterectomy and salpingectomy. CT imaging showed postoperative changes and left gonadal vein thrombosis. Despite antibiotics, she developed pancytopenia with ANC 500, DIC, ferritin 100,000, and hypoxic respiratory failure requiring intubation and ICU admission. She developed acute liver and kidney failure and severe hypoglycemia. Infectious workup was negative except for enterovirus. Peripheral smear revealed pancytopenia with bandemia and thrombocytopenia. She received platelet transfusions, cryoprecipitate, and Granix. Bone marrow biopsy revealed hemophagocytosis without evidence of leukemia or lymphoma. HLH-94 protocol (dexamethasone, IVIG, etoposide) was initiated but she passed away shortly thereafter. Well’s syndrome is an eosinophilic dermatosis possibly triggered by infections, malignancy, or vaccines. Though its etiology remains unclear, the immune dysregulation may overlap with HLH. This patient’s deterioration was initially attributed to infection. However, with worsening cytopenias, multiorgan failure, and lab markers (fever, ferritin, triglycerides, hypofibrinogenemia, pancytopenia), HLH was suspected. Her HScore was 210, indicating ∼90% probability of HLH. While Well’s syndrome has not been previously associated with HLH, both may share impaired NK cell activity, leading to unchecked eosinophil and macrophage activation. This case underscores the importance of considering HLH in patients with rare autoimmune backgrounds and unexplained systemic deterioration, as delays in recognition can be fatal. This abstract is funded by: None
Daoud et al. (Fri,) studied this question.