Drug reaction with eosinophilia and systemic syndrome: Systemic drug reaction

Drug reaction with eosinophilia and systemic (DRESS) syndrome is a potentially lethal drug reaction that occurs because of commonly used drugs like anticonvulsants and NSAIDS. Its features include severe skin rash associated with eosinophilia and systemic signs. Most common organ involved is the liver, leading to acute hepatitis-like condition. The other note-worthy features are a delayed onset, usually 2-6 weeks after the initiation of drug therapy, and the possible persistence or aggravation of symptoms despite the discontinuation of the culprit drug. It is also known as drug-induced hypersensitivity syndrome (DIHS). This case report highlights this syndrome and refl ects the fact that all physicians and public health workers should be aware of this condition and need to identify this early in the form of rash, lymphadenopathy, and systemic signs.


INTRODUCTION
Drug reaction with eosinophilia and systemic (DRESS) syndrome is a potentially lethal drug reaction that occurs because of commonly used drugs like anticonvulsants and NSAIDS.Its features include severe skin rash associated with eosinophilia and systemic signs.Most common organ involved is the liver, leading to acute hepatitis-like condition.The other note-worthy features are a delayed onset, usually 2-6 weeks after the initiation of drug therapy, and the possible persistence or aggravation of symptoms despite the discontinuation of the culprit drug.It is also known as drug-induced hypersensitivity syndrome (DIHS).This case report highlights this syndrome and refl ects the fact that all physicians and public health workers should be aware of this condition and need to identify this early in the form of rash, lymphadenopathy, and systemic signs.

CASE REPORT
A 22-year-old male patient presented with complaints of fever with chills and rigors of 7 days duration associated with an erythematous maculopapular rash all over body with itching.The patient gave no history of loose motions, cough, sore throat, headache, myalgia/arthalgia, mosquito bite, tick bite, or high-risk sexual behavior.The patient gave history of bilateral ankle swelling since 2 months prior to this presentation for which he was taking lefl unamide and NSAIDS in the form of tablet diclofenac (50 mg) twice daily.The patient had stopped diclofenac after 2 days but continued with lefl unamide.Patient was nonalcoholic and no history of any substance abuse.The patient was febrile, with an axillary temperature of 102 degrees F. There was cervical lympadenopathy, soft, around 2-4 cms, mildly tender and mild swelling at ankle joint.
There was an erythematous maculopapular rash seen all over the body, with exfoliation over the face.The mucous membranes were spared [Figures 1 and 2].
A complete hemogram showed hemoglobin of 12 gm% with total leukocyte count of 12000/cumm.The differential count showed 50% neutrophils, 28% lymphocytes, and 20% eosinophils, with absolute eosinophil count of 2400 cells/cumm.The platelet count was 1.The peripheral smear showed atypical lymphocytes.A bone marrow biopsy was performed, which was essentially normal.Urine examination and stool examination were normal.
Liver function tests continued to deteriorate further and reached a maximum of serum bilirubin of total 5.5 with serum ALT of 1190 IU/l.[Table 1] Blood and urine culture revealed no growth.
The exfoliation increased to involve the whole body by around 15 th day of admission.Patient was started on oral prednisolone 40 mg daily with multivitamin supplements and antihistaminics and liquid paraffi n for topical use.The rash started to decrease and the liver function tests also showed improvement after 15 th .The patient was discharged on 25 th day of admission with a fi nal diagnosis of DRESS possibly due to lefl unamide use or NSAIDS [Table 1].
There is no gold standard for diagnosis, and at least two diagnostic criteria have been proposed, the regiSCAR Criteria and the Japanese Consensus Group Criteria. [4]similar case was reported by Vaish, et al. [5,6] in which the authors reported a case of DRESS syndrome with severe exfoliative dermatitis with acute hepatitis associated with intake of lefl unomide.
Thus, this case shows the occurrence of DRESS syndrome in relation to possible use of lefl unomide and shows that early diagnosis is imperative for initiation of treatment in this severe drug reaction.
Thus, diagnosis of DRESS should be suspected with presence of skin rash, liver involvement, fever, hypereosinophilia, and lymphadenopathy.DRESS/DIHS is a serious illness with a mortality rate of close to 10%. [6] 2 lakhs/cumm.The liver function tests on fi rst day showed normal bilirubin levels with serum alanine transferase (ALT) of 157 IU/l and serum alkaline phosphtase (ALP) of 146 IU/l.Renal function tests and serum electrolytes were normal.An abdominal ultrasound showed bright echotexture of liver.International Journal of Medicine and Public Health | Jan-Mar 2013 | Vol 3 | Issue 1

Table 1 : Sequential rise in liver function tests LFTs Test/day On admission Day 2 Day 3 Day 7 Day 25
ALT = Alanine transferase, ALP = Alkaline phosphtase