Less Known Gastrointestinal Manifestations of Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome: A Systematic Review of the Literature

Abstract

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a potentially life threatening severe cutaneous drug reaction. Most patients develop eosinophilia, a rash, a fever, lymphadenopathy and variable visceral organ involvement 2–6 weeks following exposure to the inciting medication. Unlike other severe cutaneous drug reactions, internal organ involvement that leads to high mortality is a unique feature of DRESS syndrome. While the liver is the most common internal organ involved, literally every other visceral organ can be affected in this syndrome. The lesser-known gastrointestinal manifestations of this syndrome include esophagitis, gastritis, enteritis, colitis, pancreatitis and a late autoimmune sequela due to pancreatic injury such as fulminant type 1 diabetes mellitus, autoimmune type 1 diabetes mellitus and type 2 diabetes mellitus. While these entities are less common, they are associated with equally severe complications and adverse patient outcomes. In this review, we synthetize data on these rare manifestations using Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The liver, the most common visceral organ involved, has been described as part of DRESS elsewhere and is not included in the scope of this article.

Erosive cheilitis as an early manifestation in DRESS syndrome

“…In mild forms of DRESS syndrome where cutaneous manifestations are predominant and without significant internal organs involvement, only the cessation of the culprit medication and symptomatic treatment using topical steroids, antihistamines, are sufficient. 10,31 Immunosuppressive therapy, most often using steroids, is required when visceral organ involvement is present. In 2010, the French Society of Dermatology recommended the administration of systemic corticosteroid in case of organ involvement such as liver (transaminases >5 times upper limit of normal), kidney, lungs, and heart.…”

“…Despite the rarity of these gastrointestinal entities in Dress, their development is associated with increased mortality. 10 …”

“…10 In contrast with other SCARs, the unique features of DRESS Syndrome are the involvement of various internal organs and the delayed onset of clinical manifestations after administration of the culprit drugs (generally 2-8 weeks). 10,11 The medications reported to be most frequently associated with DRESS syndrome are aromatic anticonvulsants, dapsone, sulfasalazine, and allopurinol. 12 This condition, which can lead to death in 10% of cases, 13,14 is commonly overlooked and misdiagnosed.…”

Lamotrigine Induced DRESS Syndrome in a Child: A Case Report and Literature Review

“…Myocarditis, thyroiditis, encephalitis, and type 1 diabetes mellitus have also been reported as manifestations of this syndrome. Other gastrointestinal organs are less frequently affected, but esophagitis, gastritis, enteritis, colitis, and pancreatitis have been reported in recent literature [6].…”

“…There are two sets of diagnostic criteria for the diagnosis of DRESS; one set consists of the RegiSCAR criteria and the other consists of the SCAR-J criteria developed by Japanese investigators [6][7][8]. The treatment for DRESS consists of immediate withdrawal of the culprit drug followed by the initiation of systemic steroids.…”