Traschan Falls, NC
PIC QUESTION OF THE WEEK: 1/10/11 Q: Please define the DRESS syndrome and identify the drugs most often associated with the disease. A: Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is synonymous with the condition
previously known as drug-induced hypersensitivity syndrome (DIHS). The incidence of this severe cutaneous drug reaction ranges from 1 in 1,000 to 1 in 10,000 with an estimated mortality rate of ~10-20%. The syndrome typically begins within 3-8 weeks of drug administration and usually presents as a symmetrical maculopapular rash associated with pruritus. It subsequently progresses to resemble exfoliative dermatitis and may be accompanied by edema of the face and limbs. Unlike other severe drug-related dermatologic reactions such as Stevens-Johnsons syndrome (SJS) and toxic epidermal necrolysis (TEN), hemorrhagic mucocutaneous involvement is absent while eosinophilia (>1500/ÂľL) is always present. Systemic symptoms include high fever, involvement of at least one internal organ, atypical lymphocystosis, lymphadenopathy at two or more sites, and human herpes virus reactivation. Organs associated with DRESS syndrome include the liver, eyes, kidneys, lungs, heart, and pancreas. Multiple organ involvement is not uncommon. DRESS syndrome is considered a type IV hypersensitivity reaction and is initiated after activation of T-lymphocytes in susceptible individuals. Symptoms often resolve within one-two weeks after discontinuation of the offending agent and early recognition improves prognosis. Treatment is generally supportive; however, corticosteroids (efficacy unsubstantiated) and intravenous immune globulin (IVIG) have been beneficial in some severe cases. Drugs most commonly implicated include anticonvulsants (e.g. phenytoin, carbamazepine, lamotrigine, etc.), NSAIDs, allopurinol, and sulfonamides. Several case reports identify DRESS syndrome secondary to nitrofurantion, sulfasalazine, minocycline, bosentan, etc. Because of the known genetic component, a drug that produced DRESS syndrome should probably be avoided or used carefully in first-degree relatives. DRESS syndrome is a severe and potentially fatal drug-induced dermatologic reaction that bears resemblance to SJS and TEN. It is important for healthcare providers to be aware of its possible occurrence, its clinical presentation, and the common medications associated with its development. References: Sylvia LM. Drug allergy, pseudoallergy, and cutaneous diseases. In: Tisdale Je, Miller DA. Drug-Induced Diseases. 2nd ed. Bethesda, MD: ASHP; 2010:51-97. Teraki Y, Shibuya M, Izaki S. Stevens-Johnson syndrome and toxic epidermal necrolysis due to anticonvulsants share certain clinical and laboratory features with drug-induced hypersensitivity syndrome despite differences in cutaneous presentations. Clin Exp Dermatol 2010; 35:723-8. Velema MS, Voerman HJ. DRESS syndrome caused by nitrofurantoin. Neth J Med 2009;67:147-9. Laban E, Hainaut-Wierzbicka E, Pourreau F, et al. Cyclophosphamide therapy for corticoresistant drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome in a patient with severe kidney and eye involvement and Epstein-Barr virus reactivation. Am J Kidney Dis 2010;55:e11-e14. Photo by: fincher69: used under Creative Commons License; http://www.flickr.com/photos/fincher69/3132086186/ (Accessed January 5th, 2011)
TrisAnn Rendulic and Rachel E. Werner, Pharm.D. Candidates The PIC Question of the Week is a publication of the Pharmaceutical Information Center, Mylan School of Pharmacy, Duquesne University, Pittsburgh, PA 15282 (412.396.4600).