Management of Dysphagia in Toxic Epidermal Necrolysis (TEN) and Stevens-Johnson Syndrome (SJS)

Abstract

Toxic epidermal necrolysis (TEN) is a rare and potentially fatal skin disorder, precipitated by severe allergic drug reaction, and is one of a spectrum of conditions, which includes Stevens-Johnson syndrome (SJS). Mucosal involvement is common, resulting in extreme pain on swallowing and poor oral intake. The aim of this study was to describe swallow function in TEN and SJS and define the role of Speech Pathology in management. The Burns Unit database was reviewed for patients that presented over a five-year period with TEN and SJS. Diagnosis of TEN and SJS was confirmed by skin biopsy. Information specific to swallow function, treatment approaches, and adequacy of oral intake was collected. Fourteen patients' medical records were studied: eight TEN, two TEN/SJS spectrum, and four SJS. The majority had mucosal involvement causing odynophagia, poor oral intake, an ability to tolerate fluids more easily than solids, and increased aspiration risk. These symptoms were confirmed by Speech Pathology swallowing assessment. Severe mucosal involvement resulting in odynophagia, dysphagia, and poor oral intake is common in TEN and SJS. The speech pathologist is able to assess swallow function and provide recommendations to promote safe oral intake, minimize odynophagia, and facilitate nutritional input critical to optimizing recovery.

Management of Oral Lesions Associated With Carbamazepine Related Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis Overlap Patient

“…12,14 The extensive erosions and haemorrhagic crusting can interfere oral functions causing odynophagia, inability to tolerate solid foods, and increased aspiration risk. 12,15 All the clinical manifestations of SJS/ TEN overlap in our patient is concordant with the literature explained above.…”

“…17 Specific oral care strategies can be used to minimize trauma during oral functions such as using smaller utensils and syringes. 15 Corticosteroids play a central role in the treatment of vesiculo erosive oral lesions of autoimmune and or immunologically mediated diseases, but the evidence for the efficacy of TC in oral medicine is limited, and many of the protocols followed are drawn from experience gained in a dermatological use. 17 Those major challenges becomes more problematic as there are very few commercial products are currently available for the topical treatment of the oral mucosal lesions.…”

Nutrition in Toxic Epidermal Necrolysis

“…PN appears to be recommended due to concern that the extensive epithelial loss in SJS/TEN affects the entire gastrointestinal tract and results in poor absorption and intolerance as well as difficulty placing enteral feeding tubes 2 , 14 – 16 . Studies frequently note oral mucosal involvement that causes sloughing and associated pain 2 , 14 , 17 . Clayton and Kennedy reviewed dysphagia rates in patients with SJS/TEN 14 .…”

“…Studies frequently note oral mucosal involvement that causes sloughing and associated pain 2 , 14 , 17 . Clayton and Kennedy reviewed dysphagia rates in patients with SJS/TEN 14 . They reported that 86% of their study group had mucosal involvement, with nearly all (93%) complaining of odynophagia.…”

Severe Cutaneous Adverse Reactions

“…Phimosis can occur in men [41]. Bronchopulmonary complications confer a poor prognosis and include chronic bronchitis, bronchiolitis obliterans, bronchiolitis obliterans with organizing pneumonia, bronchiectasis [42], [43] Oesophageal stricture and webbing has also been described and can result in dysphagia [44]. …”