| Autor: |
Ceresetto JM; Servicio de Hematología y Hemoterapia, Hospital Británico de Buenos Aires, Argentina., López RA; Servicio de Clínica Médica, Hospital Británico de Buenos Aires, Argentina., Humphreys AR; Servicio de Ginecología y Obstetricia, Hospital Británico de Buenos Aires, Argentina., Duboscq C; Servicio de Hematología y Hemoterapia, Hospital Británico de Buenos Aires, Argentina., Rabinovich OM; Servicio de Hematología y Hemoterapia, Hospital Británico de Buenos Aires, Argentina., Emery NC; Servicio de Clínica Médica, Hospital Británico de Buenos Aires, Argentina., Rausch A; Servicio de Gastroenterología, Hospital Británico de Buenos Aires, Argentina., Zapata Tapia L; Servicio de Hematología y Hemoterapia, Hospital Británico de Buenos Aires, Argentina., Dezanzo P; Servicio de Anatomía Patológica, Hospital Británico de Buenos Aires, Argentina., Young P; Servicio de Clínica Médica, Hospital Británico de Buenos Aires, Argentina. E-mail: pabloyoung2003@yahoo.com.ar.; Centro Integral de Enfermedades Poco Frecuentes, Hospital Británico de Buenos Aires, Argentina. |
| Abstrakt: |
Plasminogen deficiency is a very rare multisystem entity that affects different tissues of the economy through the deposition of fibrin-rich pseudomembrane and determines a heterogeneous and diverse clinical presentation. It is transmitted in an autosomal recessive manner by mutations of the PLG gene on chromosome 6 and can be divided into hypoplasminogenemia or type I and dysplasminogenemia or type II, the latter not related to clinical pathology. Severe plasminogen deficiency has a prevalence of 1.6 individuals per million inhabitants and although it can be diagnosed in adulthood, the most severe symptoms are observed in infants and children. The most common form of onset is the so-called woody conjunctivitis, characterized by fibrin membranes that are deposited on the eyelids since childhood, causing exophytic lesions that affect vision. It can also affect other mucous membranes such as the gingival, respiratory, oropharyngeal, digestive and genital mucosa, among others. We present a rare case of severe plasminogen deficiency with conjunctivitis and woody cervicitis who was admitted with clinical acute abdominal symptoms, associated with a tumor mass due to pseudomembranous deposition in the ascending colon that simulated inflammatory bowel disease and resolved spontaneously. |
