Toxic Epidermal Necrolysis with Gastrointestinal Involvement

Abstract

Gastrointestinal involvement is a rare complication of toxic epidermal necrolysis syndrome (TENS) that results in sloughing of the intestinal epithelium. Prior case reports have noted the potential susceptibility of the entire gastrointestinal tract, from oropharynx and esophagus to sigmoid colon and rectum. Given its infrequency, the effect of gastrointestinal involvement on the treatment and prognosis of TENS is poorly understood. Here, the authors report a case of gastrointestinal symptoms in a patient diagnosed with toxic epidermal necrolysis, likely representing gastrointestinal involvement. In addition, the authors describe the histopathologic and endoscopic characteristics of the involved mucosa, clinical course, and present a review of the literature of this rare but potentially impactful complication of TENS.

Colonic Involvement of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: A Rare Cause of Gastrointestinal Bleeding

“…By contrast to the simultaneous presentation of epidermal, conjunctival, oropharyngeal, and urogenital lesions, individuals with colonic SJS/TEN have been reported to develop signs of GI bleeding 2–3 weeks after the appearance of skin lesions. 9 Owing to the high morbidity and mortality associated with colonic SJS/TEN, a prompt diagnosis is important, although an evidence-based treatment algorithm is yet to be defined. The offending agent should be discontinued and admission to the burn intensive care unit is recommended for wound management, monitoring of fluid and electrolyte balance, and nutritional support.…”

“…Three were managed exclusively medically with corticosteroids and IVIG, and 3 received corticosteroids before undergoing bowel resection for colonic SJS. 9,19 All 3 surgically managed patients survived to hospital discharge, but only one treated medically survived. 9…”

Spectrum of gastrointestinal involvement in Stevens - Johnson syndrome

“…Small bowel and colonic involvement are distinctly rare. We were able to find detailed reports of about 25 cases [age (range) 8-81 years; male: female ratio of 7:18] of SJS or TEN with GI involvement (Table 1 and Table 2)[11,12,21-39]. Details of patients with small bowel and colonic involvement are summarized in Table 2.…”

“…These include the hyperemia, erythema, congestion, friability, erosions, superficial or deep ulcerations and necrotic plaque formation with mucosal sloughing (Figure 1) (Table 1). Ulcer may be irregular, friable and covered with white fibrin-like exudates[21]. Whitish plaques and pseudomembrane formation over the damaged mucosa are the other endoscopic findings in SJS or TEN.…”

“…Lamina propria is relatively spared in these patients. HPE of healing colonic ulcer showed marked crypt architectural distortion and significant crypt loss, suggesting injury to the crypt stem cell population[21].…”