NDT Advance Access originally published online on September 5, 2006
Nephrology Dialysis Transplantation 2006 21(11):3301-3303; doi:10.1093/ndt/gfl388
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Association of DRESS Syndrome with chylous ascites
1Department of Pediatrics, Children's Hospital, Changhua Christian Hospital, Changhua, 2The Divisions of Nephrology, Chang Gung Children Hospital and 3The Divisions of Nephrology, Chang Gung Memorial Hospital, Lin-Kou Medical Center, Chang Gung University College of Medicine, Taoyuan, Taiwan
Correspondence and offprint requests to: Dr Fan Lee, MD, PhD, Divisions of Nephrology, Chang Gung Memorial Hospital, Lin-Kou Medical Center, Taoyuan, Taiwan. Email: fanlee007{at}yahoo.com
Keywords: chylous ascites; DRESS syndrome; eosinophilia; peritoneal dialysis
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Introduction |
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Chylous ascites is a rare manifestation that is attributable to the presence of thoracic or intestinal lymph in the abdominal cavity. It is well-known that inflammatory disease can produce chyloperitoneum [1]. Drug rash with eosinophilia and systemic symptoms (DRESS syndrome) is manifested by skin rash, fever, lymphadenopathy, visceral involvement and haematological abnormalities, including eosinophilia and/or atypical lymphocytosis [2]. We report an unusual case of DRESS syndrome associated with chylous ascites in a patient on peritoneal dialysis (PD).
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Case |
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A 13-year-old girl with a history of congenital defect with chromosomal translocation [t(10, 15), 46 XX], complicated with dysgenesis of the corpus callosum, mental retardation, intermittent central sleep apnoea, seizure episodes, cleft palate and atrial septal defect (ASD), had been on night-time continuous cyclic peritoneal dialysis (CCPD) for 1 year. A peritoneal catheter was inserted for PD 1 year earlier. She was admitted to the hospital because of hypotension, fever (39.1°C), leucocytosis and seizure episodes. On physical examination, the patient was febrile, frail and appeared chronically ill. The white blood cell (WBC) count was 19 900/ml (1% eosinophils, 81% segmented forms, 5% band forms, 6% monocytes and 5% lymphocytes), the haematocrit was 28.2%, and the platelet count was 314 000/ml. She was given broad-spectrum antibiotics intravenously for probable sepsis. During hospitalization, 2.5% dextrose dialysate for PD was used throughout her stay. On day 7, sputum culture revealed Gram-negative glucose non-fermenting bacilli growth. Blood culture was negative. Bilateral maxillary sinusitis was found by a computerized tomographic (CT) scan of the brain. On the 11th hospital day, she was endotracheally intubated due to aspiration pneumonia. On day 29, she was extubated, however, stridors were noted at times; bronchoscopy revealed bilateral vocal cord paresis and laryngomalacia. A bi-level positive airway pressure respirator was used when necessary. Nevertheless, leucocytosis, hypotension episodes and intermittent fever were still present. Antibiotics given intravenously were continued, and dopamine was prescribed intermittently. On day 42, three days after I.V. Augmentin was administered, eosinophilia had increased from 693/ml (WBC count 19 800/ml, 3.5% eosinophils, 78.5% segmented forms, 7.5% monocytes, 1% lymphocytes) to 3833/ml (WBC count 15 600/ml, 24.5% eosinophils, 51.5% segmented forms, 9.5% monocytes and 10% lymphocytes). Augmentin was stopped 5 days later because of worsening eosinophilia, leucocytosis and persistent fever. On day 50, biochemistry data showed the following: Ca 9.4 mg/dl, P 8.3 mg/dl, Na 127 mEq/l, K 3.8 mEq/l and Cl 93 mEq/l. The WBC count was 17 600/ml (51% eosinophils, 28.5% segmented forms, 3.5% monocytes and 14% lymphocytes), haematocrit 24.5% and platelets 232 000/ml. On hospital day 52, itching erythematous skin rashes appeared and after 1 week became generalized maculopapular skin rashes. The skin biopsy pathological report showed ‘lichenoid dermatitis with atypical cell and fat necrosis’. Inguinal lymphadenopathy was also found. On day 55, liver dysfunction was noted with a glutamic-oxaloacetic transaminase (GOT) value of 71 IU/l (on day 36, the GOT value was 40 IU/l) and chest radiography revealed ‘mild increased perihilar infiltration on both sides’. On day 56, renal function examination showed a creatinine (Cr) level of 4.1 mg/dl and a blood urea nitrogen (BUN) level of 57 mg/dl. A CT scan of the abdomen demonstrated no fat-fluid level, pancreatitis, abdominal mass or thrombi in the vena cava or iliac veins. A leukemoid reaction was noted on day 56 and it persisted for 8 days. The eosinophilia increased progressively, reaching the peak level of 22 639/ml on day 58, the same day as milky peritoneal fluid appeared. The WBC count was 55 900/ml (40.5% eosinophils, 43.5% segmented forms, 5.5% monocytes and 6.5% lymphocytes). The triglyceride content of the peritoneal dialysate was elevated at 183 mg/dl. Serum Cr level was 4.1 mg/dl, albumin 3.8 g/dl and haematocrit 25.6%. Physical examination revealed no evidence of peritoneal inflammation, and dialysis fluid contained <100 WBCs/ml. Peritoneal dialysate cytology showed ‘increased leucocyte count with eosinophil predominance in peritoneal effluent’. Peritoneal fluid cultures were negative for bacteria, fungi and acid-fast bacilli (AFB). A low-fat diet with medium chain triglycerides was started. Peritoneal effluent gradually cleared. On day 65, the peritoneal fluid returned to normal appearance; skin rashes faded during the same period of time, but chronic skin exfoliation was persistent. The WBC count was decreased to 29 500/ml (9.5% eosinophils, 73% segmented forms, 2.5% monocytes and 6.5% lymphocytes). The serum albumin 3.0 g/dl, total serum cholesterol 110 mg/dl and triglycerides 137 mg/dl. By day 70, the WBC count was 22 100/ml (6% eosinophils, 75% segmented forms, 8% monocytes and 9% lymphocytes). On day 72, the patient died from sudden onset of apnoea because of milk aspiration. Lots of milk was found in her trachea during endotracheal intubation.
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Discussion |
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DRESS syndrome complicated by chylous ascites has not been reported before. Our patient was a case of chromosomal translocation [t(10, 15), 46 XX], and had been on night-time CCPD for 1 year. She was diagnosed as having DRESS syndrome during hospitalization because of progressive maculopapular skin eruption with persistent exfoliative dermatitis, inguinal lymphoadenopathy, liver dysfunction and severe eosinophilia. The appearance of eosinophilia was closely related to Augmentin given intravenously, and generalized itching skin rashes were noted 13 days after Augmentin was administered. The temporal relationship of the worsening eosinophilia combined with progressive skin rashes and the appearance of milky ascites was noticed. When milky ascites appeared, infectious peritonitis was noted in the beginning, but it was not found later as there was a lack of both peritoneal inflammation manifestation and repeated peritoneal fluid routine, and bacterial, fungal and AFB cultures remained sterile. Subsequently, chylous ascites was diagnosed due to a high triglycerides level of 183 mg/dl in the effluent.
Leakage of chyle into the peritoneal cavity because of inflammation, trauma or obstruction of the lymphatic system leading to chyloperitoneum and chyloperitoneum in PD has been defined as triglyceride values over 110 mg/dl in the dialysate [3]. There are numerous causes of chylous ascites including abdominal malignancy, cirrhosis, infectious aetiologies, congenital, inflammatory, post-operative and traumatic causes, pelvic irradiation, spontaneous bacterial peritonitis, surgery and miscellaneous disorders. There are many inflammatory causes of chylous ascites including tuberculosis, acute or chronic pancreatitis, adhesions with intestinal obstruction, radiation, pulmonary fibrosis, constrictive pericarditis, idiopathic retroperitoneal fibrosis and sarcoidsois [1].
Resolution of the chyle leak occurred within 7 days in this patient and a whole body CT scan showed no evidence of malignancy, pancreatitis, gastrointestinal malformation, intra-abdominal or intrathoracic masses, thrombi in the vena cava or iliac veins, or retroperitoneal adenopathies. Cardiac ultrasound indicated ASD, but with intact systolic function and no pericardial effusion. Sonographic examination of the abdomen did not reveal any visceral abnormality. This patient was not taking the type of calcium channel blocker reported to be related to chyloperitoneum [4]. The abdominal CT did not demonstrate the presence of fat-fluid level of ascites, effluent AFB stain and culture were negative, and the patient had been on PD for only 1 year. All of these findings make the diagnosis of pseudochylous ascites unlikely [5]. Her albumin level was 3.8 g/dl, which is a sufficient level to avoid hypoalbuminaemia inducing oedema in the small intestinal mucosa, which could result in exudation of chylomicrons into the peritoneal fluid.
Since the PD catheter was placed 1 year previously, significant trauma to lymphatic vessels and extravasation of lymph following surgery was not a consideration. It is always possible that repeated microtrauma associated with catheter movement might have induced the elevation of triglyceride values; however, this possibility does not explain the temporal correlation of onset and resolution of the patient's milky ascites and her DRESS syndrome. Besides, her PD catheter was always in the right position when evaluated by radiology of the abdomen. Moreover, the milky dialysate subsided almost immediately upon reduction of both eosinophilia and skin rashes.
Amoxcillin, a hapten-like drug, is capable of binding to soluble or cell-bound proteins leading to modification of major histocompatibilty complex (MHC) molecules or their associated peptides. The conformational change of antigenic determinants leads to T-cell and B-cell reactions. The activation pattern provides information to the pathophysiological mechanism ongoing in both the skin and the visceral target organs [6]. Although Th2 cells induce the hypersensitivity type IVb response responsible for the maculopapular exanthem with eosinophilia, it is probably the CD8+ T-cells that result in organ damage [7,8]. It has also been proposed that uncontrolled eosinophilia is toxic to the target organ directly and amplifies the hypersensitivity response in drug-induced maculopapular exanthems [9]. We did not notice atypical lymphocytosis in the patient's plasma, but the amount of lymphocytes in the circulation does not indicate the number of activated T-cells accumulated in the homing or target organs. The patient's substantial eosinophilia in plasma demonstrates that there were activated T-cells existing somewhere in her immune network. We assume one of the homing or target organs in our case was the internal or external environment of cisterna, abdominal or thoracic ducts in the lymphatic vessels. This patient did have inguinal lymphadenopathy. Lymph nodes are structurally and functionally closely associated with the lymphatic system, as has been suggested in the case of sarcoidosis-related chyloperitoneum [10].
In conclusion, this is the first report in the literature of DRESS syndrome associated with chylous ascites in a patient undergoing PD. We suggest that DRESS syndrome should be considered in the differential diagnosis of inflammation causing chylous ascites. Immediate cessation of the responsible medication and a low-fat diet with medium chain triglycerides led to resolution of both DRESS syndrome and chylous ascites in the present case.
Conflict of interest statement. None declared.
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References |
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Accepted in revised form: 1. 6.06
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