RESUMEN
Rhinoscleroma is a chronic, granulomatous infectious disease that responds poorly to treatment. In recent years an increasing number of cases have been reported in nonendemic areas, explained largely by major migratory movements. We describe rhinoscleroma in three siblings. They had ulcerated but painless lesions, which bled spontaneously, and hemorrhagic scabs or crusts in their noses. In one child, the lesions had destroyed the entire left nasal ala and alar cartilage and most of the right. Dermatopathologic study identified the Mikulicz macrophages that contained organisms. It is possible that disposing factors could have been the neutropenia common to the three children and their poor living conditions. They were treated with a combination of trimethoprim-sulfamethoxazole and cefalexin, for a period of 3 months. We present this unusual case history of three siblings affected by a process that is relatively infrequent in our area of practice and is not considered very contagious. It is important to recognize the clinical signs characteristic of this disease, the diagnosis of which is not easy. Improvements in living conditions, hygiene, and health standards are essential prerequisites for its control and prevention.
Asunto(s)
Rinoscleroma/diagnóstico , Hermanos , Niño , Preescolar , Femenino , Humanos , Lactante , Rinoscleroma/microbiología , Rinoscleroma/terapia , Staphylococcus aureus/aislamiento & purificación , Streptococcus pneumoniae/aislamiento & purificaciónAsunto(s)
Calcitriol/análogos & derivados , Calcitriol/administración & dosificación , Fármacos Dermatológicos/administración & dosificación , Síndrome de Sjögren-Larsson/diagnóstico , Síndrome de Sjögren-Larsson/tratamiento farmacológico , Administración Tópica , Adolescente , Relación Dosis-Respuesta a Droga , Esquema de Medicación , Estudios de Seguimiento , Humanos , Masculino , Índice de Severidad de la Enfermedad , Resultado del TratamientoRESUMEN
La paniculitis lipomembranosa o esteatonecrosis membranoquística es una alteración del tejido adiposo que se suelen manifiestar en forma de placasescleróticas de tendencia crónica, localizadas en la parte inferior de las piernas. Es casi exclusiva de mujeres en su mayoría obesas, diabéticas y con unsevero componente de estasis venoso. Los hallazgos histológicos característicos son los de una paniculitis lobulillar en la que por confluencia se vanformando cavidades quísticas, rodeadas de tejido graso con signos claros de esteatonecrosis. Presentamos el caso de una mujer de 36 años que habíadesarrollado en la zona de un traumatismo previo una lesión dolorosa que a la palpación estaba constituida por múltiples formaciones nódulo-quísticas.El estudio dermatopatológico puso de manifiesto la presencia de estructuras quísticas localizadas en los lóbulos y a nivel de los septos, rodeadasde tejido graso, con signos evidentes de esteatonecrosis, presencia significativa de histiocitos espumosos y rotura de las membranas celulares con formaciónde microquistes. Las cavidades quísticas estaban delimitadas en su cara interna por una membrana serpiginosa, anuclear, homogénea y eosinofílica,PAS+, de aspecto granulado, que incluso en algunas áreas llegaba a formar pseudopapilas que se proyectaban hacia el interior del quiste. Seha sugerido que la isquemia es la causa patogénica fundamental de la paniculitis lipomembranosa, aunque también se especulan otros factores comoun trauma previo. La razón de porqué estos cambios son más prominentes en determinados enfermos y no en otros, que padecen también la mismaenfermedad, sigue sin conocerse en la actualidad (AU)
TLipo-membranous panniculitis or membrano-cystic steatonecrosis is an alteration of the adipose tissue. Sclerotic plaques with tendency to becomechronic, do usually appear located on the lower area of the legs. It is almost exclusive in female mostly obese and diabetic and with a severe componentof venous stasis. It has the histologic features of a lobule panniculitis, cystic cavities surrounded by fatty tissue with clear signs of steatonecrosisare formed by confluence. We present the case of a 36 years old female who developed a painful lesion on the location of a previous lesion, it hadnumerous nodule-cystic formations we noticed on palpation. The dermato-pathological study showed up the presence of cystic structures located onthe lobules and at the level of the septum, surrounded by fatty tissue, with clear signs of steatonecrosis, a significant presence of frothy histiocytes andbreakage of the cellular membranes with formation of micro-cysts. The cystic cavities were limited in their inner side by a serpiginous membrane, nonuclear, homogeneous and eosinophilic, PAS+, with a granulated appearance. It even formed in some areas pseudo-papillae, which projected themselvestowards the interior of the cyst. It has been suggested that ischemia is the main pathogenic cause for lipo-membranous panniculitis, althoughother reasons are also considered such as a previous trauma. Nowadays, we do not still know the reason why these changes are more prominent incertain patients than in other with the same disease (AU)