RESUMEN
Abstract: Eosinophilic annular erythema is a rare, benign, recurrent disease, clinically characterized by persistent, annular, erythematous lesions, revealing histopathologically perivascular infiltrates with abundant eosinophils. This report describes an unusual case of eosinophilic annular erythema in a 3-year-old female, requiring sustained doses of hydroxychloroquine to be adequately controlled.
Asunto(s)
Humanos , Femenino , Preescolar , Enfermedades Cutáneas Genéticas/patología , Enfermedades Cutáneas Genéticas/sangre , Eosinofilia/patología , Eritema/patología , Eritema/sangre , Piel/patología , Biopsia , Eosinófilos/patologíaRESUMEN
Abstract BACKGROUND: Actinic prurigo is an idiopathic photodermatosis, the pathophysiology of which has been hypothesized to involve subtype IV type b (Th2) hypersensitive response, whereby IL4, IL5, and IL13 are secreted and mediate the production of B cells, IgE, and IgG4. OBJECTIVES: To examine the association of serum IgE levels and the clinical severity of injuries. METHODS: This case-control study comprised patients with a clinical and histopathological diagnosis of actinic prurigo, as well as clinically healthy subjects, from whom 3cc of peripheral blood was taken for immunoassay. Cases were classified by lesion severity as mild, moderate, and severe. Descriptive statistics were analyzed, and chi-square test was performed. RESULTS: We included 21 actinic prurigo patients and 21 subjects without disease; 11 patients with actinic prurigo had elevated serum IgE levels, and 10 had low serum levels. Six actinic prurigo (AP) patients with elevated serum levels of IgE had moderate injuries, 4 had severe injuries, and 1 had minor injuries. Eight out of 10 patients with normal IgE levels presented with minor injuries in the clinical evaluation. The 21 controls did not have increased serum IgE levels. CONCLUSIONS: Elevated IgE levels are associated with moderate to severe clinical lesions, suggesting that actinic prurigo entails a type IV subtype b hypersensitivity response in which Th2 cells predominate.
Asunto(s)
Adolescente , Adulto , Niño , Femenino , Humanos , Masculino , Persona de Mediana Edad , Adulto Joven , Inmunoglobulina E/sangre , Trastornos por Fotosensibilidad/sangre , Trastornos por Fotosensibilidad/fisiopatología , Enfermedades Cutáneas Genéticas/sangre , Enfermedades Cutáneas Genéticas/fisiopatología , Estudios de Casos y Controles , Inmunoensayo , Inmunosupresores/uso terapéutico , Trastornos por Fotosensibilidad/patología , Valores de Referencia , Índice de Severidad de la Enfermedad , Enfermedades Cutáneas Genéticas/patología , Talidomida/uso terapéuticoRESUMEN
Alagille syndrome, a rare genetic disorder with autosomal dominant transmission, manifests 5 major features: paucity of interlobular bile ducts, characteristic facies, posterior embryotoxon, vertebral defects and peripheral pulmonic stenosis. We report a 6-year-old male child who presented with a history of progressive jaundice since infancy, generalized pruritus and widespread cutaneous xanthomata. He was also found to have obstructive jaundice, pulmonary stenosis with ventricular septal defect and paucity of bile ducts in liver biopsy. Histopathology confirmed skin lesions as xanthomata. The child was diagnosed as a case of Alagille syndrome. This particular syndrome with prominent cutaneous manifestations has been rarely reported in the Indian literature.