RESUMO
Pyostomatitis vegetans (PSV) is a rare disorder strongly associated with inflammatory bowel disease (IBD), most commonly ulcerative colitis, but has infrequently been found in patients with Crohn disease (CD). We present the case of a 7-year-old girl with PSV and extraintestinal findings suggestive of CD-chelitis granulomatosa, perianal and vulvar edema with biopsies revealing noncaseating granulomas, and anal skin tags-and an elevated calprotectin noted during a cutaneous flare. She did not have clinical or endoscopically identified underlying gastrointestinal involvement for 4 years after symptom onset. Given the paucity of data on managing PSV in a child without gastrointestinal findings of IBD, the literature is reviewed and treatment options discussed.
Assuntos
Colite Ulcerativa , Doença de Crohn , Doenças Inflamatórias Intestinais , Estomatite , Feminino , Humanos , Criança , Estomatite/diagnóstico , Doenças Inflamatórias Intestinais/complicações , Doenças Inflamatórias Intestinais/diagnóstico , Doença de Crohn/complicações , Doença de Crohn/diagnóstico , Doença de Crohn/patologia , Pele/patologia , Colite Ulcerativa/complicaçõesRESUMO
Over the past 20 years, the recognition of nitric oxide (NO) as an endothelial-derived vasodilator has led to remarkable advances in vascular biology awareness. The signaling molecule NO, produced by NO synthase, is a molecule that is widespread in the body and important in multiple organ systems. Soon after its discovery, investigators found NO to be a potent pulmonary vasodilator in term neonates. Nitric oxide has come to perform a key function in neonatal therapy and management since its identification, especially in those with respiratory failure. It is conventionally used in the neonatal population for the treatment of persistent pulmonary hypertension, resulting in hypoxic respiratory failure of the term or near-term newborn. Inhaled NO has been successful in acutely improving oxygenation and in reducing the need for extracorporeal membrane oxygenation treatment. In recent years, the efficacy of inhaled NO for the prevention of pulmonary disability as well as its neuroprotective capabilities in preterm infants has been explored.