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​DITRA – Deficiency of interleukin-36 receptor antagonist

DITRA is an autosomal recessive (hereditary) autoinflammatory disease caused by mutations in the IL36RN gene. It is a rare, life-threatening auto-inflammatory disease characterized by repeated episodes of high fever and the sudden onset of generalized pustular psoriasis GPP). In most cases, symptoms develop during childhood. However, the age of onset can vary considerably. Neonatal onset has also been reported. Differential diagnosis (distinguishing a particular disease or condition from others that present similar clinical features) includes other causes of GPP, mainly DIRA, in cases where symptoms appear during the first weeks of life.​

Characteristics of Deficiency of interleukin-36 receptor antagonist


Patients suffering from this syndrome often present the following symptoms:

  • flares of acute generalised skin pustulation (raised bumps filled with pus)

  • fever

  • systemic inflammation

  • general malaise

  • marked leukocytosis (increased number of white cells) during flares

  • elevated CRP (C-reactive protein) levels


It can also cause:

  • geographic tongue

  • nail dystrophy

  • failure to thrive


Other phenotypes of the IL36RN mutation may include:

  • related pustular disorders

  • palmoplantar pustulosis (of palms and soles)

  • acrodermatitis continua of Hallopeau (abbr. ACH, a rare inflammatory disease characterised by pustular eruptions beginning in the tips of fingers and toes)

  • acute generalized exanthematous pustulosis (a sudden skin eruption, also known as pustular drug eruption).


There is no established treatment. Topical and oral steroids, vitamin D3, acitretin and anti-TNF-α are effective.

These other treatments have been successful depending on the case:

  • intravenous methylprednisolone

  • Cefotaxime and co-trimoxazole

  • Anakinra

  • Adalimumab and methotrexate

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