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Hyperimmunoglobulinaemia D syndrome (HIDS) is an autosomal recessive auto-inflammatory syndrome caused by mutation in the mevalonate kinase (MVK) gene.1 It presents with febrile episodes starting in infancy with increased serum immunoglobulin (Ig)D levels.2 It is mostly described in people of Dutch or North European descent.3 Isolated cases have been reported from Turkey,4 Japan5 and Qatar.6 No case of HIDS has been reported from South Asia.
A 15-year-old boy from Kerala, India, presented with history of febrile episodes lasting 3–7 days since the age of 3 months. The episodes of fever were variably associated with polyarthritis, abdominal pain, headache, vomiting, diarrhoea, pleuritic chest pain and erythematous papular rash. The episodes occurred at an interval of 1–6 months. He underwent a laparotomy at the age of 8 years, when …