Haemophagocytic Lymphohistiocytosis as a Complication of Israeli Spotted Fever

Rickettsia spp. human infection is endemic in Portugal in the form of Mediterranean spotted fever caused by R. conorii subsp. conorii and Israeli spotted fever (ISF) caused by R. conorii subsp. israelensis. We describe a rare case of haemophagocytic lymphohistiocytosis (HLH) due to ISF, with atypical manifestations. We highlight the need for clinical suspicion for this diagnosis and the importance of timely intervention and support. LEARNING POINTS: Haemophagocytic lymphohistiocytosis is a rare complication of rickettsiosis. An exuberant inflammatory reaction with multisystemic dysfunction should raise suspicion. Cutaneous and neurological manifestations can be serious and extensive.