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Background: Severe cutaneous adverse reactions (SCARs) are associated with morbidity and mortality. Objective: The aim was to determine the different types of SCARs, their morphology, common offending drugs, interventions, and outcomes. Methods: A retrospective cohort study was conducted of all patients admitted to the dermatology service at the University Hospital of the West Indies with Stevens-Johnson syndrome (SJS), SJS/toxic epidermal necrolysis overlap (TEN), TEN, drug reaction with eosinophilia and systemic symptoms and acute generalized exanthematous pustulosis between January 1, 2012 to June 1, 2022. Results: Fifty-one cases (51) met the inclusion criteria for SCAR. SJS, SJS/TEN overlap and TEN together accounted for 71.2% of cases. SCARs were most frequent in the fourth, fifth and 6th decades of life and there was a female preponderance. Antibiotics (31%) and anticonvulsants (29%) were the most common causative agents for SCARs. Most patients had at least 1 complication. The liver was the most common extracutaneous organ affected. Mortality was 7.8%. The main cause of death was sepsis. Limitations: Results were not generalizable. There were missing data and loss to follow-up. Conclusion: Judicious use of antimicrobials and corticosteroids may be beneficial in treatment of severe cutaneous drug reactions.

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BACKGROUND: Acute generalized exanthematous pustulosis is a rare disease. Although it is usually related to drug intake, it is occasionally associated with infections, especially in the pediatric age. It is characterized by the sudden onset of sterile non-follicular pustules on an erythematous fundus, fever, and leukocytosis, with frequent and prompt spontaneous resolution. It mainly affects adults and is uncommon in childhood. Complications have been reported in approximately 20% of cases. CASE REPORT: We report the case of a 10-year-old female patient with a 5-day history of fever and dermatosis characterized by countless non-follicular pustules, predominantly on the trunk, inguinal folds, and proximal thighs but not involving palms, soles, and mucous membranes. The patient reported an incident of upper respiratory tract infection that occurred 7 days earlier. Histopathological examination confirmed the diagnosis of acute generalized exanthematous pustulosis. Spontaneous resolution occurred within 2 weeks. CONCLUSIONS: This disease is one of the severe cutaneous adverse reactions that usually have a self-limited and benign course within a few weeks. We propose that a previous respiratory infection triggered the acute generalized exanthematous pustulosis in this pediatric case. Knowledge of this pathology by the medical professionals, in general, and the pediatricians, in particular, will prevent an aggressive and inappropriate approach and management.

INTRODUCCIÓN: La pustulosis exantemática generalizada aguda es una enfermedad rara. Aunque usualmente se relaciona con el consumo de drogas, ocasionalmente se asocia con infecciones, sobre todo en edad pediátrica. Se caracteriza por el inicio súbito de pústulas no foliculares estériles sobre un fondo eritematoso, fiebre y leucocitosis, con frecuente y pronta resolución espontánea. Afecta principalmente a los adultos, y no es frecuente en la niñez. Se han reportado complicaciones en cerca del 20% de casos. CASO CLÍNICO: Se presenta el caso de una paciente de 10 años con fiebre e historia de dermatosis de 5 días de evolución caracterizada por incontables pústulas no foliculares de predominio en tronco, pliegues inguinales y parte proximal de muslos, respetando palmas, plantas y mucosas. Refirió antecedente de infección respiratoria alta 7 días antes. El examen histopatológico confirmó el diagnóstico de pustulosis exantemática generalizada aguda. Presentó resolución espontánea en el transcurso de 2 semanas. CONCLUSIONES: Esta enfermedad es una de las reacciones adversas cutáneas severas, que tiene un curso usualmente autolimitado y benigno en pocas semanas. Proponemos que la pustulosis exantemática generalizada aguda en este caso pediátrico fue desencadenada por la infección respiratoria previa. El conocimiento de esta patología por parte del gremio médico, en general, y del pediatra, en particular, evitará un abordaje y manejo agresivo e inapropiado.

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Abstract Background: Acute generalized exanthematous pustulosis is a rare disease. Although it is usually related to drug intake, it is occasionally associated with infections, especially in the pediatric age. It is characterized by the sudden onset of sterile non-follicular pustules on an erythematous fundus, fever, and leukocytosis, with frequent and prompt spontaneous resolution. It mainly affects adults and is uncommon in childhood. Complications have been reported in approximately 20% of cases. Case report: We report the case of a 10-year-old female patient with a 5-day history of fever and dermatosis characterized by countless non-follicular pustules, predominantly on the trunk, inguinal folds, and proximal thighs but not involving palms, soles, and mucous membranes. The patient reported an incident of upper respiratory tract infection that occurred 7 days earlier. Histopathological examination confirmed the diagnosis of acute generalized exanthematous pustulosis. Spontaneous resolution occurred within 2 weeks. Conclusions: This disease is one of the severe cutaneous adverse reactions that usually have a self-limited and benign course within a few weeks. We propose that a previous respiratory infection triggered the acute generalized exanthematous pustulosis in this pediatric case. Knowledge of this pathology by the medical professionals, in general, and the pediatricians, in particular, will prevent an aggressive and inappropriate approach and management.

Resumen Introducción: La pustulosis exantemática generalizada aguda es una enfermedad rara. Aunque usualmente se relaciona con el consumo de drogas, ocasionalmente se asocia con infecciones, sobre todo en edad pediátrica. Se caracteriza por el inicio súbito de pústulas no foliculares estériles sobre un fondo eritematoso, fiebre y leucocitosis, con frecuente y pronta resolución espontánea. Afecta principalmente a los adultos, y no es frecuente en la niñez. Se han reportado complicaciones en cerca del 20% de casos. Caso clínico: Se presenta el caso de una paciente de 10 años con fiebre e historia de dermatosis de 5 días de evolución caracterizada por incontables pústulas no foliculares de predominio en tronco, pliegues inguinales y parte proximal de muslos, respetando palmas, plantas y mucosas. Refirió antecedente de infección respiratoria alta 7 días antes. El examen histopatológico confirmó el diagnóstico de pustulosis exantemática generalizada aguda. Presentó resolución espontánea en el transcurso de 2 semanas. Conclusiones: Esta enfermedad es una de las reacciones adversas cutáneas severas, que tiene un curso usualmente autolimitado y benigno en pocas semanas. Proponemos que la pustulosis exantemática generalizada aguda en este caso pediátrico fue desencadenada por la infección respiratoria previa. El conocimiento de esta patología por parte del gremio médico, en general, y del pediatra, en particular, evitará un abordaje y manejo agresivo e inapropiado.

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La pustulosis exantematosa generalizada aguda (PEGA) es una patología dermatológica poco frecuente, caracterizada por la aparición brusca y generalizada de múltiples pústulas puntiformes, estériles, no foliculares, sobre una base eritematosa y edematosa. Es desencadenada frecuentemente por fármacos, entre los cuales resaltan los antibióticos. Presentamos el caso de un paciente varón de 40 años, que ingresó a emergencia por síndrome doloroso abdominal a descartar patología tumoral intraabdominal, indicándole inicialmente ceftriaxona y metronidazol. Al día siguiente, reingresó a emergencia encontrando, tras la cirugía de emergencia, una apendicitis aguda complicada con perforación intestinal, por lo cual rotan la antibioticoterapia a imipenem-cilastatina + metronidazol. A las siguientes 48 horas, presentó eritema generalizado y algunas pústulas pequeñas y microvesículas en región torácica, extremidades superiores y cuello. Asimismo, se observó en los exámenes de laboratorio, leucocitosis neutrofílica e hipoalbuminemia. En la biopsia, se encontró pústulas neutrofílicas subcorneales e intraepidérmicas, con dermis papilar edematosa e infiltrado inflamatorio perivascular con presencia de neutrófilos y escasos eosinófilos. Con todo lo descrito anteriormente, llegamos a la conclusión de una PEGA, desencadenado por los antibióticos recibidos, ceftriaxona o imipenem-cilastatina. Tras 6 días de la suspensión de imipenem-cilastatina, paciente mostró mejoría de lesiones dérmicas, con leve eritema y escasa descamación fina.

Acute generalized exanthematic pustulosis (PEGA) is a rare dermatological pathology characterized by the sudden and generalized appearance of multiple, sterile, non-follicular, punctate pustules on an erythematous and edematous base. It is frequently triggered by drugs, among which antibiotics stand out. We present the case of a 40-year-old male patient who was admitted to the emergency room due to abdominal pain síndrome, to rule out intra-abdominal tumor pathology, initially indicating ceftriaxone and metronidazole. The next day, he was re-admitted to the emergency room, finding, after emergency surgery, acute appendicitis complicated with intestinal perforation, for which they rotated the antibiotic therapy to imipenem-cilastatin + metronidazole. At the next 48 hours, he presents generalized erythema and some small pustules and microvesicles in the thoracic region, upper extremities and neck. Likewise, it is shown in laboratory tests, neutrophilic leukocytosis and hypoalbuminemia. In the biopsy, subcorneal and intraepidermal neutrophilic pustules are found, with edematous papillary dermis and perivascular inflammatory infiltrate with the presence of neutrophils and few eosinophils. With everything described above, we reached the conclusion of a PEGA, triggered by received antibiotics, ceftriaxone or imipenemcilastatin. After 6 days of the suspension of imipenem-cilastatin, the patient shows improvement of dermal lesions, with mild erythema and scant fine scaling.

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Introdução: As reações cutâneas graves a medicamentos (RCGM) compreendem um grupo de doenças caracterizadas por hipersensibilidade tardia a um ou vários tipos de fármacos. Por ser uma doença potencialmente fatal, o diagnóstico precoce, bem como o início do tratamento, são de suma importância. Objetivo: Analisar a evolução das RCGM em pacientes pediátricos acompanhados em dois hospitais da cidade de São Paulo, SP. Método: Trata-se de um estudo retrospectivo baseado na análise de prontuários de pacientes atendidos no período de 2002 a 2018 em dois hospitais da capital paulista. Resultados: Não houve diferença entre os sexos, prevaleceu a faixa etária dos adolescentes, e os medicamentos mais implicados com o desenvolvimento das lesões cutâneas foram os anticonvulsivantes, sendo os principais a carbamazepina e fenitoína, sem diferença entre eles, seguidos dos antibióticos betalactâmicos. No tratamento, todos os pacientes fizeram uso de corticoides sistêmicos e anti-histamínicos, sendo que oito pacientes também receberam imunoglobulina intravenosa e um recebeu ciclosporina. A taxa de mortalidade foi baixa e, em relação às complicações e sequelas, a autoimunidade foi a mais encontrada. Conclusão: Os casos de RCGM são eventos raros na faixa etária pediátrica, todavia de alta morbimortalidade e risco de sequelas. O diagnóstico e tratamento precoces contribuem para um melhor prognóstico, sendo de suma importância a identificação da medicação associada, bem como a retirada da mesma.

Background: Severe cutaneous adverse reactions (SCARs) comprise a group of diseases characterized by late hypersensitivity to one or more types of drugs. Because they are potentially fatal, early diagnosis and initiation of treatment are of paramount importance. Objective: To analyze the evolution of SCARs in pediatric patients followed up in two hospitals in São Paulo, SP, Brazil. Methods: This is a retrospective study based on the analysis of medical records of patients treated between 2002 and 2018 in two hospitals in the state capital. Results: There was no difference between sexes, and the age group of adolescents prevailed. Anticonvulsants were the drugs most implicated in the development of skin lesions, especially carbamazepine and phenytoin, with no difference between them, followed by betalactam antibiotics. During treatment, all patients used systemic corticosteroids and antihistamines; eight patients also received intravenous immunoglobulin and one received cyclosporine. The mortality rate was low, and regarding complications and sequelae, autoimmunity was the most commonly found. Conclusion: Cases of SCAR are rare events in the pediatric age group, but morbidity, mortality, and risk of sequelae are high. Early diagnosis and treatment contribute to a better prognosis, and identification of the associated medication as well as its withdrawal are extremely important.

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BACKGROUND AND OBJECTIVE: Severe cutaneous adverse reactions to drugs (SCARs) are associated with high morbidity and mortality and with sequelae. Objective: To characterize patients with SCARs in 8 health care institutions in Latin America. METHODS: We performed a cross-sectional, descriptive, multicenter study of patients diagnosed with SCARs in Latin America between January 2009 and December 2018. The analysis was carried out using a database in BD Clinic. RESULTS: We collected 70 patients, of whom 42 (60%) were women. Mean age was 38.7 years. Forty-two patients (60%) had DRESS-DIHS, 12 (17.1%) TEN, 5 (7.1%) SJS, 6 (8.5%) AGEP, 4 (5.7%) other reactions not classified as SCARs, and 1 (1.4%) overlapping SJS-TEN. The main causative drugs were aromatic anticonvulsants in 31 cases (44.3%), ß-lactam antibiotics in 11 cases (15.7%), and non-ß-lactam antibiotics in 6 cases (8.6%). In all of the cases, the suspected drug was withdrawn at the first sign of a SCAR. Sixty-six patients (94.2%) received anti-inflammatory treatment, mostly systemic corticosteroids. Complications occurred in 53 cases (75.7%), and 3 patients died (4.3%). Thirteen patients (18.6%) had sequelae. CONCLUSIONS: This is the first multicenter report on SCARs in Latin America. DRESS-DIHS was the most frequently reported clinical entity, and anticonvulsants were the main triggers. Most of the patients received systemic corticosteroids. Complications were frequent, and 3 patients died.

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Presentamos el caso de una paciente mujer de 91 años, hospitalizada por infección de herida operatoria secundaria a cirugía de cadera. Se administró tratamiento antibiótico con meropenem, presentando a los 4 días: fiebre, astenia y una erupción eritematosa diseminada con pústulas no foliculares a predominio de tronco, extremidades y áreas de pliegues; además, se observó leucocitosis con neutrofilia. Al estudio histopatológico de biopsia de piel, se evidenciaron pústulas subcorneales con exocitosis de neutrófilos. Se suspendió antibiótico, con mejoría clínica a los 5 días posteriores de la suspensión de meropenem. Existen pocos reportes de casos de pustulosis exantemática generalizada aguda producido por meropenem.

We present the case of a 91-year-old female, patient hospitalized for operative wound infection secondary to hip surgery. Antibiotic treatment with meropenem was administered, presenting at 4 days: fever, asthenia and disseminated erythematous rash with non-follicular pustules predominantly of the trunk, extremities and fold areas; In addition, leukocytosis is found with neutrophilia. Histopathological study of skin biopsy showed subcorneal pustules with exocytosis of neutrophils. Antibiotic was discontinued, with clinical improvement 5 days after the suspension of meropenem. There are few reports of cases of acute generalized exanthematous pustulosis caused by meropenem.

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Abstract Acute generalized exanthematous pustulosis is a rare drug-induced eruption that is characterized by acute, nonfollicular sterile pustules on an erythematous and edematous base. The most frequently implicated drugs are beta-lactam antibiotics. Hydroxychloroquine has been widely used to treat dermatologic and rheumatologic diseases and has been reported as a rare cause of acute generalized exanthematous pustulosis. A 42-year-old female presented with pustular lesions on the skin surface with erythema, facial edema, and occasional atypical target-like lesions after 21 days of treatment with 200mg/day hydroxychloroquine for rheumatoid arthritis, diagnosed one month previously. We report a case with acute generalized exanthematous pustulosis induced by hydroxychloroquine and treated with dapsone and systemic corticosteroid.

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Abstract Acute localized exanthematous pustulosis is a localized variant of acute generalized exanthematous pustulosis, which is characterized by the eruption of multiple scattered pustules following drug administration. A 72-year-old woman presented with multiple erythematous pustules on her face, which had appeared two days after using cefoperazone and sodium sulbactam. Histopathological findings showed subcorneal pustules and mixed inflammatory cell infiltration in the dermis. The pustules resolved within about two weeks after the patient discontinued the antibiotics. This report discusses the case of a woman with a cutaneous drug reaction consistent with acute localized exanthematous pustulosis that occurred after cefoperazone and sodium sulbactam were administered.

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BACKGROUND: Between 62 and 90% of cases of acute generalized exanthematous pustulosis are caused by drugs. Its onset is rapid with generalized pustules, fever, and blood neutrophil count over 7000; pustules resolve spontaneously in less than 15 days. A case associated with piroxicam described. CASE REPORT: A 36-year-old with initial erythema of the thorax and abdomen, accompanied by burning, without fever, which later spread to his forearms, upper arms, and thighs, with face edema. A week earlier he had taken piroxicam for low back pain; at the time of hospitalization he received antihistamines, and topical and systemic steroids. Full blood count showed leukocytes at 8920, eosinophils at 600, neutrophils at 6600, total serum IgE at 188 UI, C-reactive protein at 2.9 mg/L, and no liver, kidney, or lung involvement. Treatment was initiated with intravenous antihistamines and ranitidine, saline, topical Vaseline plus topical mupirocin, and systemic steroids. On the second day of hospitalization neutrophils increased to 9000 and PCR to 3.3. The score to evaluate acute exanthematous pustulosis in the patient was 8, giving a definitive diagnosis. CONCLUSIONS: The differential diagnosis should be established primarily with pustular psoriasis. The prognosis is generally good, as reported.

Antecedentes: Entre 62 y 90% de los casos de pustulosis exantemática generalizada aguda son causados por medicamentos. Su inicio es rápido con pústulas generalizadas, fiebre, conteo de neutrófilos en sangre mayor de 7000; la resolución de las pústulas es espontánea en menos de 15 días. Se describe un caso asociado a piroxicam. Caso clínico: Hombre de 36 años de edad con eritema inicial en tórax y abdomen, acompañado de ardor, sin fiebre, que posteriormente se extendió a antebrazos, brazos y muslos; con edema de cara. Una semana antes había consumido piroxicam por dolor lumbar; al momento de su hospitalización recibía antihistamínicos, esteroides tópicos y sistémicos. El hemoleucograma mostró leucocitos de 8920, eosinófilos de 600, neutrófilos de 6600, IgE sérica total de 188 mg/L, proteína C reactiva de 2.9 mg/L, sin compromiso hepático, renal ni pulmonar. Se inició tratamiento con antihistamínicos y ranitidina intravenosos, solución salina, vaselina tópica más mupirocina tópica y esteroides sistémicos. Al segundo día de hospitalización, los neutrófilos aumentaron a 9000 y la proteína C reactiva a 3.3 mg/L. La puntuación para validar pustulosis exantemática aguda en el paciente fue de 8, indicativa de diagnóstico definitivo. Conclusiones: El diagnóstico diferencial debe establecerse principalmente con psoriasis pustulosa. El pronóstico en general es bueno, como sucedió con el caso informado.

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Abstract: Acute generalized exanthematous pustulosis (AGEP) is an acute febrile rash, usually induced by drugs, which recently has been linked to spider bite. We report a case of a male patient, 48 years old, with an erythematous rash accompanied by fever and small non-follicular pustules. He reported previous pain in the buttock with the onset of a necrotic plaque. The lesion was compatible with spider bite of the genus Loxosceles. According to the EuroSCAR group instrument, the patient scored +10 indicating definite diagnosis of AGEP. As the patient had a compatible lesion and had no other triggers of AGEP, in an Loxosceles endemic area, the AGEP would be associated with spider bite, as described in other publications.

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Acute generalized exanthematous pustulosis is an uncommon skin eruption, characterized by fever and the rapid onset of disseminated, non-follicular, sterile pustules, over an erythematous skin background. It is usually classified as a severe cutaneous adverse drug reaction, whose most relevant triggers are antibiotics and anticonvulsants. However, viral and bacterial infections have also rarely been associated with this dermatosis. We report the case of a patient, who developed lesions of acute generalized exanthematous pustulosis as an extrapulmonary manifestation of Mycoplasma pneumoniae infection.

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The occurrence of acute generalized exanthematous pustulosis adverse reactions to medication administration is becoming more frequent. This article reports the case of a 78-year-old woman who attended the clinic with generalized papules and pustules on the scalp, trunk and limbs, with a concordant histology study and who was diagnosed with acute generalized exanthematous pustulosis (AGEP) associated with the use of phenytoin, a medication that may cause different skin reactions and that has been previously related to this disease. The patient was treated with systemic steroids and the disease had a satisfactory outcome.

La aparición de reacciones adversas a medicamentos del tipo pustulosis exantemática generalizada aguda es cada vez más frecuente. Se presenta el caso de una paciente de 78 años quien acude a consulta presentando unas pápulas y pústulas generalizadas en cuero cabelludo, tronco y extremidades, con estudio de histología compatible y a la que se le diagnostica pustulosis exantemática aguda generalizada (PEAG) asociada al uso de fenitoína, una medicación que puede provocar distintas reacciones cutáneas y que previamente se ha asociado a esta enfermedad. La paciente es tratada con esteroides sistémicos y la enfermedad llega a una resolución satisfactoria

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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.

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La pustulosis exantemática generalizada aguda (PEGA) es una erupción cutánea severa de escasa frecuencia, caracterizada por la aparición de pústulas estériles no foliculares sobre una base eritemato-edematosa, asociada a fiebre y neutrofilia. La hidroxicloroquina (HCQ), una droga antimalárica ampliamente utilizada en enfermedades dermatológicas, ha sido asociada como una causa poco frecuente de PEGA. Presentamos un paciente de sexo femenino de 20 años de edad, que desarrolló PEGA secundaria a la ingesta de hidroxicloroquina.

Acute generalized exanthematous pustulosis (AGEP) is an uncommon severe skin rash characterized by the appearance of non-follicular sterile pustules on an erythematous background, associated with fever and neutrophilia. It ́s characterized by an acute onset and spontaneous resolution within 2 weeks. Hydroxychloroquine (HCQ), an antimalarial drug widely used in dermatological diseases, has been associated as a rare cause of PEGA. We present a 20 years old woman who developed AGEP secondary to HCQ.

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La pustulosis exantemática aguda generalizada (PEAG) es una enfermedad poco frecuente, de patogenia desconocida, provocada generalmente por fármacos, entre los que se encuentran en primer lugar, los antibióticos del grupo de los betalactámicos. Se presenta el caso de una paciente con esta entidad, provocada por amoxicilina-clavulánico con gran extensión de las lesiones.(AU)

Acute generalized exanthematous pustulosis (AGEP) is a rare disease, with unknown pathogenesis, usually caused by drugs where we can find in the first place, antibiotics that are part of the betalactam group. A case of a patient with this entity, caused by amoxicillin-clavulanic with great extension of the lesions is reported.(AU)

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La pustulosis exantemática aguda generalizada (PEAG) es una enfermedad poco frecuente, de patogenia desconocida, provocada generalmente por fármacos, entre los que se encuentran en primer lugar, los antibióticos del grupo de los betalactámicos. Se presenta el caso de una paciente con esta entidad, provocada por amoxicilina-clavulánico con gran extensión de las lesiones.(AU)

Acute generalized exanthematous pustulosis (AGEP) is a rare disease, with unknown pathogenesis, usually caused by drugs where we can find in the first place, antibiotics that are part of the betalactam group. A case of a patient with this entity, caused by amoxicillin-clavulanic with great extension of the lesions is reported.(AU)

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La pustulosis exantemática aguda generalizada (PEAG) es una enfermedad poco frecuente, de patogenia desconocida, provocada generalmente por fármacos, entre los que se encuentran en primer lugar, los antibióticos del grupo de los betalactámicos. Se presenta el caso de una paciente con esta entidad, provocada por amoxicilina-clavulánico con gran extensión de las lesiones.

Acute generalized exanthematous pustulosis (AGEP) is a rare disease, with unknown pathogenesis, usually caused by drugs where we can find in the first place, antibiotics that are part of the betalactam group. A case of a patient with this entity, caused by amoxicillin-clavulanic with great extension of the lesions is reported.