PP47 CLINICAL SIGNIFICANCE OF HERPESVIRUS 6 (HHV6) IN PEDIATRIC TRANSPLANTATION

Abstract

Case report: We report the case of an 80-day-old infant referred to our department with refractory severe dermatitis, resembling Acrodermatitis Enteropathica (AE), associated with moderate failure to thrive. She had been hospitalized in a different clinic when she was 50 days old. During that time she was treated with oral steroids for suspected atopic dermatitis with no lasting benefits. The baby had been exclusively breast fed. At admission, the patient was well below the 3rd percentile for weight and at the 25th percentile for length, while the respective percentiles at birth were both at the 50th. Dermatological examination revealed erythematous, scaly plaques with crusts and ulcerations, predominantly in the diaper area, perioral area and extremities. Mucous membranes and nails were not involved. Laboratory tests revealed anemia, hypoalbuminemia and zinc deficiency. Laboratory abnormalities included the following: albumin minimum values of 16.2 and 19.2 g/dl (38–54 g/dl), haemoglobin minimum values of 5.5 and 6.7 g/dl (10–13 g/dl). Two albumin infusions and two blood transfusions were required. A sweat test was done but the amount of sweat collected was insufficient for an accurate result, influenced by the skin condition. However, stools were positive for fat analysis; steatocrit was 28% ( 5 U/g). With the suspect of CF pancreatic enzyme supplementation was started and was associated with clinical and laboratory findings improvement. The dermatitis showed a gradual resolution, preceded by an extensive desquamation. Finally, mutation analysis for CF showed homozygous mutation 508. After three months of therapy, the patient’s weight increased from the 3rd to the 25th percentile, the dermatitis had completely resolved and laboratory findings had improved. Conclusions: Skin manifestations of CF are under-estimated; however, dermatitis can be the presenting sign of the disease, prior to pulmonary and gastrointestinal symptoms. AE-like dermatitis may concur and allows a correct and early diagnosis in atypical presentations of CF, particularly in association with other symptoms such as failure to thrive or laboratory findings such as anemia and hypoalbuminemia. Even in cases of negative neonatal screening, further investigations such as the sweat test and/or genetic analysis may be required if there are symptoms suggestive of CF. It is important to offer genetic counselling to parents with a family history of CF so that they are aware of the possibility of performing CF carrier screening and prenatal diagnosis.