RESUMEN
Macrodystrophia lipomatosa is a rare, non-hereditary congenital anomaly characterized by localised gigantism in the form of macrodactyly affecting a part of limb and rarely entire limb due to overgrowth of all mesenchymal elements. Radiological and pathological hallmark is the disproportionate fibroadipose tissue proliferation in subcutaneous tissue, nerve sheaths, and periosteum that lead to soft tissue and bony enlargement. We present the case of a twenty years old female who presented with history of gradual enlargement of the medial aspect of right hand along ulnar nerve distribution since birth. MRI showed hypertrophy of bones and soft tissue with fatty proliferation, leading to the diagnosis of macrodystrophia lipomatosa.
Asunto(s)
Dedos/anomalías , Gigantismo/patología , Mano/inervación , Nervio Cubital/patología , Femenino , Humanos , Imagen por Resonancia Magnética , Enfermedades Raras , Adulto JovenRESUMEN
Thymic stromal lymphopoietin (TSLP) is a key pro-allergic cytokine that has recently been linked to chronic airway diseases, such as asthma and chronic obstructive pulmonary disease (COPD). High levels of TSLP were detected in bronchial mucosa of asthma and COPD patients suggesting TSLP's biological role beyond a signature 'Th2-favoring' or 'pro-allergic cytokine'. Besides inflammatory cells, airway structural cells produce and are targets of TSLP suggesting a potential autocrine loop that may have a profound effect on local inflammatory response and airway remodelling. This review sums up diverse mechanisms that mediate TSLP/TSLP receptor-signalling network in chronic airway diseases.