ABSTRACT
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.
ABSTRACT
La pustulosis exantemática generalizada aguda (AGEP, en inglés) es una enfermedad poco frecuente, causada por drogas, caracterizada por aparición aguda de numerosas pústulas estériles en una base eritematosa, asociada en ocasiones a fiebre y leucocitosis, en algunos casos puede causar un compromiso de piel severo pero que resuelve rápidamente con la suspensión del fármaco sin tratamiento especial requerido. Puede confundirse con otras entidades como la psoriasis pustular e inusualmente produce compromiso sistémico. Presentamos un caso de AGEP secundario a terapia anti-TNF con compromiso sistémico.
The acute generalized exanthematous pustulosis (AGEP, in english)is a rare disease caused by drugs, characterized by acute onsetof numerous sterile pustules on an erythematous base, sometimesassociated with fever and leukocytosis, in some cases may causea severe skin involvement reaction but solved quickly with the drugsuspension without special treatment required. It can be confusedwith other entities such as pustular psoriasis and unusually producesystemic involvement. We present a case of secondary to anti-TNFtherapy AGEP systemic involvement.
Subject(s)
Humans , Acute Generalized Exanthematous Pustulosis , Psoriasis , Skin DiseasesABSTRACT
La acrodermatitis continua de Hallopeau (ACH) es una enfermedad inflamatoria crónica que afecta a los dedos de las manos y/o pies, se caracteriza por una placa eritematodescamativa con erupciones pustulosas estériles. Es una patología rara o quizá subdiagnosticada y más frecuente en mujeres de edad mediana, según los pocos casos reportados. Se presenta el siguiente caso clínico de una adolescente de 15 años de edad, que presentó una placa eritematodescamativa con pústulas estériles, en tres oportunidades a lo largo de dos años. El diagnóstico de ACH se realizó por la clínica y la histología. Se indicó tratamiento con acitretina vía oral y se tiene mejoría clínica importante.
The acrodermatitis continua of Hallopeau (ACH) is a chronic inflammatory disease that affects the fingers and toes, is characterized by erythematous scaly plaque with sterile pustular eruptions. It is a rare condition or perhaps underdiagnosed, according the few reported cases is more common in middle aged women. We report the case of a 15 year-old woman which presented an erythematous plaque with sterile pustules on three occasions along two years, the diagnosis of ACH was made by clinical and histology. Acitretin therapy is indicated orally and has significant clinical improvement.