General |
High IgD (>14 mg/dL), measured on 2 occasions, at least 1 month apart |
Elevated IgA (>260 mg/dL) |
Recurrent attacks |
Mutations in MVK gene (classic HIDS) |
Decreased MVK activity (classic HIDS) |
During Attacks |
Elevated inflammatory markers (ESR, CRP, SAA, and leukocytosis) |
Abrupt onset of fever (>38.5°C or 101.3°F) |
Lymphadenopathy (predominantly cervical) |
Abdominal distress (vomiting, diarrhea, pain) |
Skin manifestations (varying erythematous macules, papules, petichiae, and purpura) |
Arthralgias/arthritis |
Splenomegaly |
Mildly elevated urinary mevalonic acid concentration (classic HIDS) |