ABSTRACT
La dermatomiositis juvenil es el subtipo más frecuente del grupo de las miopatías idiopáticas inflamatorias en la edad pediátrica. Clínicamente se caracteriza por la presencia de debilidad muscular proximal y simétrica con afectación cutánea característica. El diagnóstico clásicamente se ha basado en los criterios de Bohan y Peter (1975). Sin embargo, en 2017, el International Myositis Assessment and Clinical Studies Group (IMACS) ha publicado unos criterios de clasificación de las miopatías idiopáticas inflamatorias donde es posible clasificar a un paciente sin necesidad de biopsia. La patogenia de la enfermedad es compleja y no del todo conocida. Están involucrados el sistema inmune innato y adaptativo, donde juega un papel muy importante el interferón, y donde existen agentes externos como infecciones o factores medioambientales que actuarían como desencadenantes en sujetos genéticamente predispuestos. El tratamiento ha de ser intensivo desde el inicio, con el objetivo de controlar la enfermedad cutánea y muscular de forma precoz, minimizar complicaciones como la calcinosis, más frecuente en niños que en adultos, y mejorar el pronóstico a largo plazo. Presentamos el caso de una niña prepúber en cuyo debut de la enfermedad solo presentaba dolor e impotencia funcional en pierna izquierda, con antecedente de traumatismo de baja energía, sin elevación de enzimas musculares, lesiones cutáneas ni una biopsia muscular típica (AU)
uvenile dermatomyositis is the most common idiopathic inflammatory myopathy subtype in the paediatric age group. Clinically, it is characterized by proximal and symmetrical muscle weakness with characteristic skin lesions. Traditionally, the diagnosis has been based on the Bohan and Peter criteria (1975). However, the International Myositis Assessment and Clinical Studies Group (IMACS) published new classification criteria in 2017 that allow classification of the disease without a biopsy. The pathogenesis of the disease is complex and has not been fully elucidated. It involves innate and adaptive immunity, with interferon playing a key role, and external agents like infections or environmental factors acting as triggers of the disease in genetically predisposed people.Treatment must be intensive from the beginning to achieve early control of cutaneous and muscular disease, reduce complications such as calcinosis, which is more frequent in children than in adults, and improve long-term outcomes.We present the case of a prepubertal girl who had onset with pain and left leg dysfunction and reported a history of low-energy trauma, without muscle enzyme elevation, skin lesions or a typical muscle biopsy. (AU)
Subject(s)
Humans , Female , Child , Dermatomyositis/diagnosis , Methylprednisolone/therapeutic use , Glucocorticoids/therapeutic use , Hydroxychloroquine/therapeutic use , Antirheumatic Agents/therapeutic use , Dermatomyositis/drug therapy , Treatment OutcomeABSTRACT
No disponible
Subject(s)
Humans , Male , Female , Pregnancy , Infant, Newborn , Adult , Phenylketonuria, Maternal/diagnosis , Fused Kidney/diagnosis , Ultrasonography, Prenatal , Neonatal Screening/methods , Kidney/diagnostic imaging , Fused Kidney/etiologySubject(s)
Fused Kidney/diagnosis , Phenylketonuria, Maternal/diagnosis , Adult , Female , Fused Kidney/etiology , Humans , Infant, Newborn , Male , PregnancyABSTRACT
No disponible
Subject(s)
Humans , Male , Child , Splenic Infarction/diagnostic imaging , Splenic Infarction/etiology , Infectious Mononucleosis/complicationsABSTRACT
No disponible
Subject(s)
Humans , Female , Child, Preschool , Mycobacterium marinum/pathogenicity , Mycobacterium Infections/diagnosis , Paronychia/diagnosis , Mycobacterium Infections, Nontuberculous/etiology , Mycobacterium Infections, Nontuberculous/prevention & control , Cloxacillin/therapeutic use , Amoxicillin-Potassium Clavulanate Combination/therapeutic use , Mycobacterium marinum/isolation & purification , Rifampin/therapeutic use , Clarithromycin/therapeutic use , Anti-Bacterial Agents/therapeutic useSubject(s)
Mycobacterium Infections, Nontuberculous , Mycobacterium marinum , Skin Diseases, Bacterial , Child, Preschool , Female , Humans , Mycobacterium Infections, Nontuberculous/diagnosis , Mycobacterium Infections, Nontuberculous/drug therapy , Skin Diseases, Bacterial/diagnosis , Skin Diseases, Bacterial/drug therapySubject(s)
Infectious Mononucleosis/complications , Pharyngitis/complications , Splenic Infarction/etiology , Antibodies, Viral/blood , Child , Herpesvirus 4, Human/immunology , Herpesvirus 4, Human/isolation & purification , Humans , Immunoglobulin G/blood , Immunoglobulin M/blood , Infectious Mononucleosis/drug therapy , Male , Pharyngitis/virology , Splenic Infarction/diagnostic imaging , Tomography, X-Ray Computed , UltrasonographyABSTRACT
No disponible
Subject(s)
Humans , Male , Child , Enteral Nutrition , Tuberculosis, Pulmonary/therapy , Mycobacterium tuberculosis/pathogenicity , Antitubercular Agents/therapeutic use , Abdominal Pain/etiology , Asthenia/etiology , Crohn Disease/diagnosis , Tuberculosis, Pulmonary/diagnosisABSTRACT
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