Background Generalized pustular psoriasis (GPP) is a rare and severe phenotype of psoriasis characterized by sudden outbreak of widespread coalescent sterile pustules associated with a spectrum of systemic symptoms. Objective We aimed to describe the epidemiology and treatment of GPP in Brazil from the public health care system perspective. Methods This was a retrospective public claims database study, using outpatient and inpatient databases, with information from January 2018 to August 2020, based on records of health resource utilization by patients with GPP. Outpatient treatment regimens and fatal inpatient outcomes were described. Results In total, 1458 outpatients of all ages were identified, of whom 53% were women. We estimated the GPP prevalence in Brazil to be between 0.7 and 0.9 per 100,000. Acitretin was the most commonly dispensed drug. Of all the outpatients, 769 outpatients could be tracked in the inpatient database, and 151 had hospital admissions during the study period. Of them, 5.3% had a fatal outcome during hospitalization. A primary skin condition or an infection was the most frequent hospitalization cause. Limitation The International Classification of Diseases codes for GPP and psoriasis have not been previously validated in this context. Conclusion GPP is a rare disease in Brazil and affects individuals of all ages and both sexes. Hospitalizations and disease-related deaths highlight the need for its prompt diagnosis, close medical follow-up, and effective treatment.

Atualmente, os histiócitos não são mais compreendidos como células únicas, mas como um grupo heterogêneo de células com o mesmo aspecto histológico, mas com características e funções distintas entre si. Várias doenças proliferativas de histiócitos, conhecidas como histiocitoses, são descritas. Tais doenças são raras, e seu estudo costuma ser difícil. Este artigo objetiva simplificar o entendimento desse grupo de doenças, adequando-o a esse novo paradigma da heterogeneidade dos histiócitos.

The authors present part I of a review of multicentric reticulohistiocytosis, a rare systemic proliferative disease of histiocytes of unknown cause. It is clinically characterized by cutaneous and mucosal nodules and by osteoarticular lesions. The disease occurs in outbreaks that progress in severity, with spontaneous regression, but usually leaving incapacitating arthritis and disfiguring facial lesions. The authors discuss the historical, epidemiologic, and clinical aspects of this disease.