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  • MA, MKD-severe
    Mevalonate Aciduria (MA), Mevalonate Kinase Deficiency-severe
    Most present with symptoms at birth, or in early infancy. Most have facial features noted at birth.
  • MKD-mild/moderate (aka HIDS)
    Mevalonate Kinase Deficiency-mild/moderate, Hyperimmunoglobulinemia D with Periodic Fever Syndrome
    >90% present with symptoms in infancy.
  • Sweet's (SS)
    Sweet's Syndrome (SS); aka Acute Febrile Neutrophilic Dermatosis
    Adulthood, more frequently in middle-aged women, but also in men. It rarely presents in infants or children.
  • XLP2-MAS, XLP-2
    X-linked familial hemophagocytic lymphohistiocytosis; XIAP deficiency; X-linked lymphoproliferative syndrome type 2 (XLP-2)-MAS
    Infancy to early childhood. A few with school-age onset.

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