RESUMO
A 51-year-old uninsured, otherwise healthy male who works in the fishing industry presented with a two-month history of pruritic scaly plaques on his face, scalp, and trunk and mild photosensitivity. A biopsy of a scalp lesion revealed acantholysis consistent with pemphigus foliaceus. Laboratory testing demonstrated elevated anti-desmoglein 1, positive antinuclear antibodies (ANA and anti-dsDNA), and elevated Sjögren's anti-SS-A antibodies. The patient was diagnosed with pemphigus erythematosus. The patient was not optimally responsive and was unable to discontinue systemic corticosteroids despite a maximum dosage of mycophenolate mofetil of 3000 mg/day. Hence, rituximab was added as a rescue treatment with the rheumatoid arthritis protocol. Three months after starting rituximab, there was a marked improvement in symptoms with complete resolution of cutaneous lesions.
RESUMO
Surgical repair of type C esophageal atresia (EA) with distal tracheoesophageal fistula (TEF) is complicated by an anastomotic leak in 10%-30% of cases with associated morbidity. A novel procedure in the pediatric population, endoscopic vacuum-assisted closure (EVAC), accelerates the healing of esophageal leaks by using the effects of VAC therapy, including fluid removal and stimulation of granulation tissue formation. We report 2 additional cases of chronic esophageal leak treated with EVAC in EA patients. The first is a patient with a previously repaired type C EA/TEF and left congenital diaphragmatic hernia complicated by an infected diaphragmatic hernia patch erosion into the esophagus and colon. Additionally, we discuss a second case using EVAC for early anastomotic leak following type C EA/TEF repair in a patient who was later found to have a distal congenital esophageal stricture.
