Cryptogenia multifocal ulcerous stenosing enteritis: An entity on its own as a cause of abdominal pain, iron deficiency anemia and protein-losing enteropathy

We studied a patient with edema secondary to protein losing enteropathy, and recurrent bouts of bloating and abdominal pain secondary to intestinal subocclusion episodes. After the clinical study, the patient was diagnosed of cryptogenic multifocal ulcerous stenosing enteritis (CMUSE), that is a rare disease, probably caused by mutations in the gene PLA2G4A, and characterized by multiple short stenosis of the small bowel with superficial ulcers, which do not exceed the submucosa layer. Inflammatory bowel disease (Chron's disease), intestinal tuberculosis and intestinal ulcers secondary to non-steroidal anti-inflammatory drugs are the main differential diagnosis. To sum up, physicians should included CMUSE in the differential diagnosis of recurrent abdominal pain, iron deficiency anemia, occult intestinal bleeding, edema and protein losing enteropathy.

ResumenA propósito del estudio de un paciente con anasarca, enteropatía pierde proteínas y dolor abdominal recurrente secundario a episodios de suboclusión intestinal, al que se le diagnostica de enteritis ulcerosa criptogénica, estenosante y multifocal (CMUSE), se revisa esta enfermedad rara y poco conocida, probablemente causada por mutaciones en el gen de PLA2G4A, que se caracteriza por múltiples estenosis cortas del intestino delgado con ulceraciones que no sobrepasan la submucosa. La enfermedad inflamatoria intestinal (enfermedad de Crohn), la tuberculosis intestinal y las ulceraciones intestinales asociadas a la toma de antiinflamatorios no esteroides son los principales diagnósticos diferenciales. En conclusión, CMUSE debería ser incluida en el diagnóstico diferencial del dolor abdominal recurrente, anemia ferropénica con sangrado intestinal oculto, edemas y enteropatía pierde proteínas.