Generalized pustular psoriasis (GPP) or Deficiency of interleukin thirty-six–receptor antagonist (DITRA)

This is an extremely rare autosomal recessive disease. In 2011 it was found to be caused by mutations in a gene called IL36RN, which lead to production of an abnormal inflammatory protein called the IL-36 receptor antagonist. This abnormal protein leads to overactive inflammation through mechanisms which are not yet fully understood.

Symptoms may start any time form childhood to the sixth decade. Attacks may be life threatening and can be triggered by stress, pregnancy or drugs.

Symptoms include:

  • fever
  • rash all over the body, with pustules

As in all the inherited fever syndromes there are raised levels of inflammatory markers