|Year : 2020 | Volume
| Issue : 2 | Page : 88-90
Response to intravenous immunoglobulin in a patient of drug reaction eosinophilia systemic symptom syndrome with renal involvement complicated by steroid-induced avascular necrosis of femur
Suman Patra, Richa Rupla, Saumya Narula, Dinesh P Asati
Department of Dermatology, All India Institute of Medical Sciences, Bhopal, Madhya Pradesh, India
|Date of Submission||23-Oct-2019|
|Date of Acceptance||18-Sep-2020|
|Date of Web Publication||28-Dec-2020|
Dinesh P Asati
Department of Dermatology, All India Institute of Medical Sciences, Bhopal - 462 020, Madhya Pradesh
Source of Support: None, Conflict of Interest: None
Drug reaction eosinophilia systemic symptom (DRESS) is one of the most serious adverse drug reactions and corticosteroid is the mainstay of management. Here, we report a case of DRESS with renal involvement complicated by steroid-induced avascular necrosis of femur. The patient responded significantly with two courses of intravenous immunoglobulin given at monthly interval, and his corticosteroid dose could be tapered early.
Keywords: Cyclosporine, drug reaction eosinophilia systemic symptom, intravenous immunoglobulin
|How to cite this article:|
Patra S, Rupla R, Narula S, Asati DP. Response to intravenous immunoglobulin in a patient of drug reaction eosinophilia systemic symptom syndrome with renal involvement complicated by steroid-induced avascular necrosis of femur. Indian J Drugs Dermatol 2020;6:88-90
|How to cite this URL:|
Patra S, Rupla R, Narula S, Asati DP. Response to intravenous immunoglobulin in a patient of drug reaction eosinophilia systemic symptom syndrome with renal involvement complicated by steroid-induced avascular necrosis of femur. Indian J Drugs Dermatol [serial online] 2020 [cited 2021 Apr 11];6:88-90. Available from: https://www.ijdd.in/text.asp?2020/6/2/88/305128
| Introduction|| |
Drug reaction eosinophilia systemic symptom (DRESS) is one of the most serious adverse drug reactions. Till now, oral corticosteroids is the mainstay of management, which should be tapered slowly to prevent any flare up. Owing to multiple comorbidities, we need drugs other than corticosteroids which can be used in special circumstances. Here, we report a case of DRESS with renal involvement complicated by steroid-induced avascular necrosis of femur and its improvement with intravenous immunoglobulin (IVIG).
| Case Report|| |
A 24-year-old boy presented to the emergency department with a history of fever and generalized erythema, exfoliation of skin for 10 days, and yellowish discoloration of the eye for 7 days. He was diagnosed with sputum-positive pulmonary tuberculosis 4 months back for which he was started on anti-tubercular therapy (rifampicin, isoniazid, pyrazinamide, and ethambutol). There was a history of head trauma with subdural hemorrhage 2 months back, and he was started on phenytoin 100 mg once daily for that. Levetiracetam (for the last 1½ months) and acetazolamide (for 1 month) were added later.
The rash started as erythematous macules and papules from the trunk, which progressed to the bilateral upper and lower limbs within the next 2 days. In the next 5 days, he developed generalized exfoliation along with facial swelling [Figure 1]. Clinical examination did not reveal any mucosal involvement. Lymph nodes in the inguinal, cervical, and jugular area were palpable. The patient had intermittent fever with a temperature varying between 37.7°C and 39°C. Laboratory analysis revealed total leukocyte count of 13,860 with 18% of eosinophils, thrombocytopenia (50,000/mL), elevated serum glutamic pyruvic transaminase (587 U/L), serum glutamic oxaloacetic transaminase (870 U/L), and total bilirubin 13.8 g/dL (conjugated – 7.8; unconjugated – 6.0) with hypoalbuminemia (1.77 g/dL). Renal function test (RFT) (serum creatinine – 0.6 mg/dL; blood urea – 21 mg/dL) was within the normal range. Based on these findings, he was diagnosed with DRESS syndrome fulfilling the REGISCAR criteria with a score of 8; all suspected drugs were stopped, and he was started on prednisolone 50 mg daily (1 mg/kg). He had improvement in the skin rash and a decrease in the liver enzyme levels in the next 10 days. He was discharged on oral prednisolone 40 mg once daily after 4 weeks.
|Figure 1: Facial edema and severe exfoliative dermatitis in a patient of drug reaction eosinophilia systemic symptom syndrome|
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After 2 weeks, he was admitted again with a reactivation of similar rash along with hematuria. On further evaluation, he had 100 red blood cells/high-power field with 3+ proteinuria in urine and normal serum urea and creatinine values. Ultrasound revealed medical renal disease (increased echogenicity and loss of corticomedullary differentiation in both the kidneys). He developed intermittent episodes of fever during the hospital stay, but no focus of infection was found. Overall, a diagnosis of interstitial nephritis as a part of DRESS was suspected. He was initially managed on prednisolone at the same dose for a week, but due to inadequate response, the dose was increased to 50 mg once daily. After 2 weeks, he developed pain and stiffness of the left thigh and hip. Magnetic resonance imaging suggested avascular necrosis of the bilateral femoral head (left Grade 2 and right Grade 1). In view of that, steroid dose was decreased to 30 mg/day and started on cyclosporine 4 mg/kg to prevent any impending flare. In the next 2 weeks, he did not have much improvement in skin lesions with persisting transaminitis and worsening hematuria. He also developed hypertension and renal impairment for which the dose of cyclosporine was decreased to 3 mg/kg/day. In view of persistent disease activity and lack of therapeutic options, he was given two cycles of IVIG (21.6 g/day for 5 days) 1 month apart and other treatments were continued. His skin lesions improved markedly with decrease in erythema, scaling, and facial swelling with in 6 weeks of the first dose [Figure 2]. Hematuria and proteinuria also resolved in 1 month with normalization of hematological parameters including resolution of eosinophilia (3%). The steroid dose was tapered and stopped in 1 months' time, and he was maintained on only cyclosporine 150 mg daily. At 2-month follow-up, he did not have any flare, continuing cyclosporine 50 mg once daily which we have planned to stop after 1 month.
| Discussion|| |
DRESS syndrome usually presents with fever, skin rash, eosinophilia, lymphadenopathy, and involvement of visceral organs such as liver, kidney, and heart. The time of onset of rash is usually 2–6 weeks after the initiation of suspected drug. The cutaneous manifestations typically consist of a maculopapular eruption and in some instances, vesicles, bullae, pustules, purpura, facial edema, cheilitis, and erythroderma. Kidney is the second-most common organ to be involved after liver and involvement can range from hematuria, interstitial nephritis, to acute renal failure in the severe form.
Management of DRESS syndrome is usually according to the severity of systemic involvement. Initial suspicion and identification of the culprit drug(s) and its immediate withdrawal is of paramount importance. The expert opinion of French Society of Dermatology, 2010, recommended systemic corticosteroid use (prednisone or equivalents at a dose equivalent to 1 mg/kg/day) in the case of organ involvement, such as liver (transaminases >5 times upper limit of normal), kidney, lungs, and heart.
Cyclosporine may be considered a second-line therapy for patients with severe organ involvement, who do not respond to systemic corticosteroids, and for patients in whom corticosteroids are contraindicated. Periodical monitoring of both clinical and laboratory parameters such as complete blood count (CBC), liver function tests, and RFTs is necessary to assess the severity of disease and treatment response. IVIG has been used in many occasions in the management of DRESS either alone or as adjuvant to corticosteroid., In a recent series, it was not supported to be used as monotherapy due to the risk of significant viral reactivation. We could not do investigations to look for reactivation of viruses due to lack of resource, but we found it useful in our case to save our patient. IVIG can be considered in selected cases as an add-on therapy in the management of DRESS syndrome.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]