Severe cutaneous allergic reactions in Brazil: new risk alleles to be identified in our population?

Results Twenty-three cases of SCAR were identified: 12 DRESS/ DIHS, 1 overlap DRESS/AGEP and 10 SJS/TEN. Sixteen patients (70%) were female, the median age was 41 years (IQR=26-50). The aromatic anticonvulsivants were implicated in the etiology in 73% of cases, followed by antibiotics (30%). All patients with DRESS/DIHS exhibited cutaneous, systemic and laboratory characteristic changes of this syndrome. Patients with SJS/TEN had fever and mucosal involvement, 20% had neurological abnormalities and no one organ involvement or ocular complications. Eight (66%) patients with DRESS had late reactivation of disease. There was 1 death due to refractory cardiac insufficiency. During the 1year of outpatient follow-up, we found autoimmune changes in 21% of patients. Reactions to others drugs following diagnosis occurred in 2 patients with DRESS/DIHS, 1 patient with SJS/TEN had solar urticaria and another one with DRESS/DIHS developed dermographism. All patients with DRESS/DIHS were treated with corticosteroids, with an average of 108 days (9-180) of treatment. Eight out of 10 patients with SJS/TEN used corticosteroids with good response. IGIV was used in 1 patient with SJS/TEN and associated with steroids in 1 patient with DRESS/DIHS. We identified the known relationship between carbamazepine (CBZ) and HLAA*31:01 in 2 patients with DRESS and allopurinol and HLA-B*58:01 in 2 patients (one SJS/TEN and one overlap DRESS/AGEP). Interestingly, we found the same alleles in 3 patients with DRESS caused by phenytoin (HLA-A*23 and HLA-B*53) and in 3 (DRESS) with carbamazepine (HLA-A*74 and HLA-B*15). None of them were family related.