Severe epidermolysis bullosa acquisita in a patient with graft-versus-host disease: causal or coincidental?

Graft-versus-host disease (GvHD) is common after haematopoietic cell transplantation (HCT). Mucocutaneous manifestations are variable and may simulate autoimmune bullous dermatoses. However, the association of GvHD with autoimmune disorders, including bullous dermatoses, is also well recognized. We describe a patient with GvHD in whom severe and relapsing epidermolysis bullosa acquisita (EBA) was diagnosed 3 years after transplant and propose a causal association with GvHD. A 66-year-old woman developed GvHD following allogeneic HCT for acute myeloid leukaemia in 2016. This affected her gastrointestinal tract and skin but improved with oral corticosteroids and ciclosporin. In 2019 she presented with a widespread rash consisting of large, tense, haemorrhagic blisters. Histological features were in keeping with EBA. Direct immunofluorescence was also consistent with EBA, demonstrating linear positivity for IgG and C3 confined to the blister base, as was detection of collagen VII antibodies on indirect immunofluorescence. She was admitted and treated with high-dose oral steroids, ciclosporin and intravenous immunoglobulin (IVIg) with eventual resolution of blistering. Although further IVIg administration was planned as an outpatient, this coincided with the start of the COVID-19 pandemic and she elected not to attend and also stopped all medication. Despite this, her EBA remained quiescent until September 2021 when she was readmitted with a severe deterioration in blistering and significant dysphagia due to an oesophageal stricture, with a weight of 31.7 kg. Once again, she responded rapidly to oral prednisolone and IVIg. Dapsone was considered but precluded by G6PD deficiency and there were clinical and adherence concerns about using mycophenolate mofetil. Upon discharge she was again nonadherent to medication and failed to attend for planned IVIg. She flared and was admitted for a third time in December 2021, requiring gastrostomy for nutritional support;her weight at this time was 26.4 kg. Her EBA is currently well controlled on prednisolone and IVIg. EBA is a rare, acquired blistering disorder secondary to autoantibodies targeting type VII collagen. Previous studies have found circulating basement membrane zone (BMZ) antibodies in 24% of chronic GvHD patients, possibly generated in response to chronic BMZ damage (Hofmann SC, Kopp G, Gall C et al. Basement membrane antibodies in sera of haematopoietic cell recipients are associated with graft-versushost disease. J Eur Acad Dermatol Venereol 2010;24: 587-94). Corresponding clinical manifestations are rare, with bullous pemphigoid the most frequently reported. EBA is much less common with four previously reported cases [Brassat S, Fleury J, Camus M, et al. (Epidermolysa bullosa acquisita and graftversus- host disease). Ann Dermatol Venereol 2014;141: 369-73 (in French)]. As a fifth case of EBA, our patient provides further evidence of a likely pathophysiological relationship between GvHD and autoimmune subepidermal bullous dermatoses, and highlights the significant challenges of managing these vulnerable patient groups during the COVID-19 pandemic.

Esophageal Strictures in Children, a Six Year Case Series Experience in a Tertiary Center in Mexico City

Introduction: Esophageal strictures in children are in most cases associated with a benign etiology. There are multiple conditions that are associated with esophageal strictures including congenital stenosis, secondary to surgical repair of esophageal atresia, caustic burns following ingestion of acids or alcalis, radiation therapy and secondary to some pathologies as gastroesophageal reflux disease, eosinophilic esophagitis, scleroderma, epidermolysis bulllosa and idiopathic. Esophageal dilation can be performed with different techniques that include endoscope dilators, dilations performed over the wire and using the endoscope itself. Some cases require other adjunctive techniques that improve the results after failed progressive dilation. This therapies includes medical treatment and surgical derivations, with some cases known as recalcitrant. Also, esophageal strictures and its management could produce motility disorders. There is no consensus in the literature regarding the management process, especially in frequency of interventions, use of adjunctive therapies and alternatives for conservative management. This problem is more evident in developing countries. Objective(s): The objective of this study was to describe the cases of esophageal strictures and its management in children between 2016 and 2022 in the Instituto Nacional de Pediatria in Mexico City. Method(s): This was a six year retrospective study involving pediatric patients with esophageal stricture. We retrospectively reviewed the medical records of 23 pediatric patients who underwent endoscopic treatments for esophageal strictures, between January 2016 and May 2022 in the Comprehensive Pediatric Gastroenterology Diagnostic Unit in the Instituto Nacional de Pediatria in Mexico City. Result(s): The mean age at diagnosis was 24 months (Q1 15, Q3 35), 12 patients were male (52%) and 11 patients were female (48%). The most prevalent etiology was caustic strictures in 10 patients (43%). Six patients (26%) had esophageal atresia (4 type III, 1 type I and 1 type V), all whose received surgical management in the first days of life. All required repeated pneumatic dilation (between 1 and 11) for the management of postsurgical stenosis. Other etiologies that were found include Schatzki Ring, congenital stenosis, esophageal fibrosis associated with congenital dyskeratosis, epidermolysis bullosa, graft-versus-host disease and gastroesophageal reflux disease (one patient for each cause). In one patient the etiology remains unknown. Seventeen patients had one stricture, 5 patients had two strictures and 1 had 3 strictures. Ten patients had esophageal pseudodiverticula and two had mucosal fold. Six patients underwent dilation with Savary-Guilliard dilators combined with pneumatic balloon dilation. Four patients received mitomycin- C as an adjuvant therapy during dilations. The average diameter of stenosis was increased from 7 mm (range 4-15 mm) to 13,5 mm (range 8-18mm). Two patients had severe complications, one had a esophageal perforation associated with dilation. The other one had a pneumothorax related with anesthetic management. In the outcome 6 patients are asymptomatic, 1 patient persist with dysphagia after completed treatment, 9 patients are under treatment, 1 patient died secondary to its underlying disease and 6 patients lost follow up. Conclusion(s): Post-corrosive esophagitis and post-esophageal atresia anastomotic strictures were the most frequent types of cicatricial esophageal strictures. The conservative treatment was the first management strategy in the majority of patients, being the endoscopic balloon dilation the first choice. The SARS-COV-2 sanitary emergency limited the progressive intervention rate and appropriate clinical follow up of patients, reason why there is an important loss of follow up in the described group. A number of patients are currently on management, reason why their outcomes will be assessed in the future.

PEG placements during the COVID-19 pandemic An audit of the procedures performed by a single consultant operator at a tertiary teaching hospital

PEG feeding provides a valuable nutritional access for patients with a functional gastrointestinal tract. The aim of this project was to audit all the PEG procedures performed by a single consultant operator during the Covid-19 pandemic including the indications, outcomes and complications. All the procedure reports were accessed to identify the patients, indications and immediate outcomes. For every patient, all the letters from all specialties were accessed for the dates following the procedure through the clinical records platforms to identify any later complications. A total of 92 procedures were performed between 15/3/2020 and 31/4/2021 in a total of 84 patients. Lists were operating at less than half capacity compared to pre Covid-19. 65 were planned PEG insertions, 17 were planned removals, and 10 were planned replacements. 5 of the procedures were for PEG-J insertion or replacement. The main indication was Head and Neck Ca in 59/92 procedures followed by CVA 9/92, chronic nausea/vomiting/gastroparesis in 6/92, dysphagia with or without aspiration risk in 4/92, MND in 4/92, CNS tumour post-op (pineal gland) in 2/92, cerebral palsy in 1/92, multiple sclerosis in 1/92, neurodegenerative disorder in 1/92, neuromuscular disorder in 1/92, chronic pancreatitis in 1/92, cystic fibrosis in 1/92, depression with poor oral intake in 1/92 and learning difficulties in 1/92. 83/92 procedures were completed successfully. 2 procedures had a failed intubation, 1 because of a subglottal stricture. The rest of the abandoned procedures were due to patient distress (2/92), high oesophageal stricture (1/92), failed cannulation (1/92), body habitus (1/92), stomach not translluminated and patient desaturation (1/92). One of the planned replacements failed because of a buried bumper. In two patients there was a small leak around the PEG site, 1 identified in the endoscopy room, 1 a few weeks later but both were managed conservatively and the PEG was kept in place. No other complications identified. From October 2020 the consistent use of Corflo PEGs reduced the service demands as these can be easily removed in the community. Lists during the COVID-19 pandemic were significantly impacted, especially UGI procedures, as these are aerosol generating procedures. The vast majority of the procedures are completed successfully and there are no significant complications. Most failed procedures are due to patient related factors such as tolerance and anatomical factors. The use of PEGs that can be removed in the community avoiding further endoscopic procedures is a valuable tool especially in this pandemic and early post-pandemic setting.