Aplasia pura de células rojas como forma de presentación de lupus eritematoso sistémico / Pure red cell aplasia as a presentig form of systemic lupus erythmatosus

La aplasia pura de células rojas (APCR) es un síndrome definido por anemia normocítica normocrómica, con reticulopenia severa y reducción importante o ausencia absoluta de precursores eritroides en la médula ósea. Ocasionalmente se desencadena en el curso de una colagenopatía o una enfermedad autoinmune. Presentamos el primer caso descripto en la literatura de un varón con APCR como forma de debut de lupus eritematoso sistémico (LES). Se trata de un hombre de 65 años que presentó anemia normocítica normocrómica, ANA 1/5120 y anti-Sm 2,61. Refería úlceras orales, poliartralgias, tumefacción de ambos tobillos y fotosensibilidad. Se realizó estudio de médula ósea con evidencia de hipoplasia de serie roja por paro madurativo a nivel de eritroblasto basófilo, ausencia casi completa de los elementos maduros y contenido muy elevado de proeritroblastos de gran tamaño. Con el diagnóstico de APCR como debut de LES, se lo trató con prednisona con buena respuesta. Podemos concluir que el despistaje de enfermedades sistémicas en pacientes con APCR es esencial para asegurar un correcto manejo y un mejor pronóstico.

Pure red cell aplasia (PRCA) is a syndrome defined by normocytic normochromic anemia with severe reticulocytopenia and marked reduction or absence of erythroid precursors from the bone marrow. Occasionally it is triggered in the course of collagen or autoimmune diseases. We present the first case reported in the literature of a man with PRCA as the onset form of systemic lupus erythematosus (SLE). A 65-year-old man, who presented normocytic normochromic anemia, ANA 1/5120 and anti-Sm 2,61. He reported oral ulcers, polyarthralgia, swelling of both ankles and photosensitivity. Bone marrow examination showed red cell line hypoplasia due to maturation arrest at the level of the basophilic erythroblast, almost absence of mature cells, and a very high content of large proerythroblasts. With the diagnosis of PRCA as the first manifestation of SLE, he was successfully treated with Prednisone. We can conclude that screening for systemic diseases in patients with PRCA is essential to ensure correct management and a better prognosis.

Increasing Incidence of mucormycosis in Spanish inpatients from 1997 to 2018.

BACKGROUND: Mucormycosis is a worldwide angio-invasive fungal infection that is associated with high morbidity and mortality. A few European studies have focused on the epidemiology. METHODOLOGY: A retrospective longitudinal descriptive study was performed with inpatients diagnosed with mucormycosis (ICD-9-CM, code 117.7, cases 1997-2015; and ICD-10, code B46, cases 2016-2018; along with length of hospital stay) in Spanish public hospitals between 1 January 1997 and 31 December 2018. Data were obtained from the Minimum Basic Data Set (CMBD in Spanish). PRINCIPAL FINDINGS: A total of 962 patients were recorded; 665 were men. The mean age (±SD) was 55 ± 18.8 years. The annual incidence rate increased from 0.74 to 1.24 cases per million person-years. The lethality rate was 31.3%. Renal failure (41.6%) and haematological malignancy (36.3%) were the main factors involved. CONCLUSIONS: Mucormycosis is a rare infectious disease in Spain, but it has had a significantly increased incidence in the last two decades. Being an adult male and having diabetes, neoplasm or renal failure are the main factors associated. High mortality is usually associated mainly with haematological malignancy and renal failure. CMBD studies could be an efficient tool for assessing changes in the epidemiology of mucormycosis.

Delusional parasitosis. A multicenter retrospective study in Spanish infectious disease services / Parasitosis delirante. Un estudio retrospectivo multicéntrico en los servicios españoles de enfermedades infecciosas

Introduction: Delusional parasitosis or Ekbom syndrome is a condition described mainly in the fields of psychiatry and dermatology, with a complex diagnostic and therapeutic approach. However, it is uncommon to assess patients with this disease in infectious disease units. The objective of this work is to describe the experience of three infectious diseases departments with respect to this entity. Methods: A retrospective descriptive study of 20 patients diagnosed with delusional parasitosis in three Infectious Diseases Services was performed between 2003 and 2017. Results: The median age of the patients was 54 years, with a female/male ratio of 1.5:1. In 9 patients, an endoparasitic delirium (mainly digestive) was described, in 5 an ectoparasitic form was described, and in the remaining 6, a mixed form was described. Fourteen patients presented some type of psychiatric disorder. Four patients had alcohol or drug abuse disorder. All patients had made consultations to other specialties with a median of three per patient (range 1–7). Ten patients received “empirical” antiparasitic treatment and 8 received some type of psychopharmaceutical treatment. The evolution was very variable: in 3 patients, the delusional parasitosis was resolved; in 9 patients, the clinical manifestations persisted, and the remaining patients were lost to follow-up. Conclusions: Ekbom syndrome is a common process in infectious diseases, presenting some differences with other series evaluated by dermatologists and psychiatrists. Management of this disease should promote a multidisciplinary approach to enable a joint treatment, thus optimizing patient management and therapeutic adherence.(AU)

Introducción: La parasitosis delirante o síndrome de Ekbom es una afección descrita principalmente en los campos de la psiquiatría y la dermatología, con un enfoque diagnóstico y terapéutico complejo. Sin embargo, es poco frecuente evaluar a los pacientes con esta enfermedad en unidades de enfermedades infecciosas. El objetivo de este trabajo es describir la experiencia de 3 departamentos de enfermedades infecciosas con respecto a esta entidad. Métodos: Entre 2003 y 2017 se llevó a cabo un estudio descriptivo retrospectivo de 20 pacientes a los que se les diagnosticó parasitosis delirante en 3 servicios de enfermedades infecciosas. Resultados: La mediana de edad de los pacientes era de 54 años, con una proporción mujeres/varones de 1,5:1. En 9 pacientes se describió un delirio endoparasitario (principalmente digestivo), en 5 se describió una forma ectoparasitaria y en los 6 restantes una forma mixta. Catorce pacientes presentaban algún tipo de trastorno psiquiátrico. Cuatro pacientes presentaban un trastorno de alcoholismo o drogadicción. Todos los pacientes habían acudido a consultas de otras especialidades con una mediana de 3 por paciente (intervalo de 1-7). Diez pacientes recibieron tratamiento antiparasitario «empírico» y 8 recibieron algún tipo de psicofármaco. La evolución fue muy variable: en 3 pacientes se resolvió la parasitosis delirante; en 9 pacientes persistieron las manifestaciones clínicas y se perdió el seguimiento de los demás pacientes. Conclusiones: El síndrome de Ekbom es un proceso habitual en las enfermedades infecciosas, que presenta algunas diferencias con otras series evaluadas por dermatólogos y psiquiatras. El tratamiento de esta enfermedad debe promover un enfoque multidisciplinario que permita un tratamiento conjunto, optimizando así el tratamiento del paciente y el cumplimiento terapéutico.(AU)

Delusional parasitosis. A multicenter retrospective study in Spanish infectious disease services.

INTRODUCTION: Delusional parasitosis or Ekbom syndrome is a condition described mainly in the fields of psychiatry and dermatology, with a complex diagnostic and therapeutic approach. However, it is uncommon to assess patients with this disease in infectious disease units. The objective of this work is to describe the experience of three infectious diseases departments with respect to this entity. METHODS: A retrospective descriptive study of 20 patients diagnosed with delusional parasitosis in three Infectious Diseases Services was performed between 2003 and 2017. RESULTS: The median age of the patients was 54 years, with a female/male ratio of 1.5:1. In 9 patients, an endoparasitic delirium (mainly digestive) was described, in 5 an ectoparasitic form was described, and in the remaining 6, a mixed form was described. Fourteen patients presented some type of psychiatric disorder. Four patients had alcohol or drug abuse disorder. All patients had made consultations to other specialties with a median of three per patient (range 1-7). Ten patients received "empirical" antiparasitic treatment and 8 received some type of psychopharmaceutical treatment. The evolution was very variable: in 3 patients, the delusional parasitosis was resolved; in 9 patients, the clinical manifestations persisted, and the remaining patients were lost to follow-up. CONCLUSIONS: Ekbom syndrome is a common process in infectious diseases, presenting some differences with other series evaluated by dermatologists and psychiatrists. Management of this disease should promote a multidisciplinary approach to enable a joint treatment, thus optimizing patient management and therapeutic adherence.

Afectación hepática grave como debut de un síndrome hemofagocítico / Severe liver disease as first sign of a haemophagocytic syndrome

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