Discussion

To our knowledge, this is the first case of interleukin-36 receptor antagonist deficiency (DITRA) reported in South America. The genetic cause and the pathogenesis for acute generalized pustular psoriasis were unknown until 2011, when Marrakchi et al. described nine families affected by the disease with an autosomic recessive pattern and identified mutations of interleukin-36 receptor antagonist as a gene associated with the disease [1]. GPP develops as a primary condition or in association with preexisting psoriasis. However, patients, who debuted with generalized pustular psoriasis the de novo, are more likely to have IL36RN mutations [2] even more when its presentation is at early ages. In our case, symptoms began