A rare case of DRESS (Drug Reaction With Eosinophilia And Systemic Symptoms) with important involvement of heart, liver, central nervous system and bone marrow

摘要

A 46 year old woman from Sri Lanka, underwent bloodexamination after her return to Italy and found high titersof uric acid (8.2 mg/dl), thus her family physicianprescribed allopurinol 300 mg once daily. After 3 weeksshe presented to the ER with fever and esantematic rashon the thorax and limbs, therefore allopurinol was stoppedand began prednisone 25 mg/day. A week later shereturned to the ER for a worsening of the cutaneouseruption, high fever and marked weakness. The admittingphysician excluded an adverse drug reaction and wasadmitted to the Infectious diseases ward with altered liverfunction tests, high inflammation indices and eosinophilia.All the tests for infectious disease workup resulted negative,but her general condition worsened with high fever,anasarcatic status and neurological weakness. The totalbody CT scan showed lung and abdominal lymphadenopathyand pleural effusion. The bone marrow biopsyexcluded a lympho myeloproliferative disorder andrevealed two microgranulomas and 34% eosinophils. Thehepatic biopsy showed a diffuse acute hepatitis withmarked infiltrate of T-lymphocytes (CD3+) and eosinophils.Cardiac examination with EKG and echocardiographywas normal but the cardiac MNR dimostrated asevere pericardial inflammation with effusion. Only whenthe eosinophilic blood concentration raised to 52% thediagnosis of DRESS was made. The patient also resultedpositive for HLA-B*58:01. She began treatment with highglucocorticoid i.v. (1 mg/kg/day) and obtained rapidimprovement of the general condition of weakness, skinhealing by desquamation and normalization of eosinophiliaand hepatic function. She was discharged after one monthof hospital stay.ConclusionThis is a particular case of DRESS with extensive histological,blood, and radiological examination. It is important toemploy elevated levels of glucocorticoids in the treatmentof DRESS. The normal cardiac screening by EKG andechocardiography may sometimes prove inadequate foridentifying real cardiac injury. We believe that it’s importantto undergo cardiac MR imaging since appropriatetherapy may prevent progression of cardiac disease.