Question A 10-year-old girl presented with a 1-year history of scaling and cracking of the lips, causing significant cosmetic concern. The clinical course was characterised by periods of exacerbation and partial remissions, and she had moderate to severe discomfort, especially during eating and speaking. She had been treated with emollient creams and low-potency topical steroids, with no effec- tive improvement. She was otherwise healthy and had a positive family history of psoriasis. Fig. 1 Fissuring and scalling of the lips, extending beyond vermillion border. Her examination revealed fissures and desquamation of the whole lips, extending beyond the vermillion border (Fig. 1). Oral mucosa and the surrounding skin were normal and there was no Dr Joana Correia1 involvement of other skin areas. Dr Susana Machado2 Patch testing with a standard series of common allergens and Dr Manuela Selores2 dental products were negative. Departments of 1Pediatrics and 2Dermatology What is your diagnosis? (Answer on page 315) Centro Hospitalar do Porto Porto Portugal
Prolonged fever in an 8-year-old boy post-Epstein–Barr virus infection
Question A 7-year-old boy presented to a tertiary paediatric Emergency Department with a 3-week history of fever, lethargy and loss of appetite. He had been reviewed by his family doctor on three sepa- rate occasions prior to presentation, and had completed a course of oral amoxicillin with no improvement. He had no significant past medical history, no sick contacts nor any history of recent travel. On examination, the patient was febrile (39.1 C) and tachyp- noeic. There were multiple small (<1 cm) non-tender lymph nodes palpable in the posterior cervical chain, left axilla and inguinal regions. The liver edge was palpable 4 cm below the right costal margin, with a tippable spleen. Epstein–Barr virus serology requested by the patient’s family doctor was consistent with acute Fig. 1 Wright–Giemsa stain of the patient’s bone marrow aspirate. infection (positive IgM and equivocal IgG to Epstein–Barr virus viral capsid antigen). A diagnosis of infectious mononucleosis was made. Professor Catherine Cole1,3,4,5 During admission, the patient continued to be febrile with progres- Dr Murray Princehorn6 sive anaemia and thrombocytopaenia, along with hyperferritinaemia Dr Christopher C Blyth1,2,7,8 (5480 μg/L) and hypertriglyceridaemia (3.6 mmol/L). His IgA, IgM 1 School of Paediatrics and Child Health and IgG levels were elevated (22.4, 5.3 and 13.3 g/L respectively). University of Western Australia Along with this, further questioning revealed an older brother who Princess Margaret Hospital passed away from overwhelming sepsis. A bone marrow aspirate Departments of 2Infectious Diseases, 4Haematology, and 6General was requested as part of the further investigative work-up (Fig. 1). Paediatrics What does it show? Given this appearance and the provided investi- PathWest Laboratory Medicine, Princess Margaret Hospital gation results, what is the likely diagnosis? (Answer on page 315) Departments of 3Haematology, 5Cancer Centre, 7Microbiology, and 8 Wesfarmers Centre for Vaccines and Infectious Diseases Mr Amirul H Ahmad Bazlee1,2 Telethon Kids Institute, University of Western Australia Dr Daniel Yeoh2 Perth, Western Australia Mr Jesper Jensen3 Australia
314 Journal of Paediatrics and Child Health 53 (2017) 314–315