A 16 year-old patient of Hawaiian ancestry was admitted for confusion in April 2018. He had no relevant past medical history except he went to the dentist few months ago for patchy darkish pigmented macules of the oral mucosa (Fig. 1A). His mother reported that he complained of pharyngitis and diarrhoea since one week. Blood pressure, body temperature and heart rates at admission were 80/50 mm Hg, 38.3°C and 163 beats per min respectively. Oxygen saturation and glycemia were estimated to 100% room air and 3.2 mmol/L using pulse oximeter and glucometer respectively.