Symptoms of HIDS
- • Onset in infancy (90% of cases with onset at <1 year of age; episodes become more frequent in childhood).
- • Recurrent episodes of fever/chills, abdominal pain, nausea/vomiting, erythematous fixed rash (typically macular, can be papular or morbilliform), arthritis, headache, adenopathy, orogenital ulcers (40%), splenomegaly.
- • Duration of attacks: 3 to 7 days; recurrence of attacks: every 4 to 6 weeks.
- • Can be associated with palpable purpura or erythema elevatum diutinum as well.
- • Amyloidosis is rare.
- • HIDS is on the less-severe end of the spectrum of MVK deficiency; complete enzyme deficiency results in mevalonic aciduria, with developmental delay, dysmorphism, organomegaly, and sometimes periodic inflammatory crises.
