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      Lamotrigine induced DRESS syndrome

      case-report

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          Abstract

          Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is a rare and life-threatening delayed drug hypersensitivity reaction characterized by skin eruption, fever, lymphadenopathies, and visceral involvement. Here, we are presenting a 12 year old boy, who developed rare but life threatening DRESS syndrome due to Lamotrigine. Early detection and treatment led to his rapid recovery. This case is presented to highlight the importance of early detection of rare fatal syndrome.

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          Drug-induced pseudolymphoma and drug hypersensitivity syndrome (Drug Rash with Eosinophilia and Systemic Symptoms: DRESS).

          H. Bocquet, M Bagot, J C Roujeau (1996)
          Since the first description by Saltzstein in 1959, the denomination of drug-induced pseudolymphoma was used to describe two cutaneous adverse drug reactions with a histological picture mimicking malignant lymphoma. On the basis of clinical presentation, this term includes two different patterns: (1) hypersensitivity syndrome which begins acutely in the first 2 months after the initiation of the drug and associates fever, a severe skin disease with characteristic infiltrated papules and facial edema or an exfoliative dermatitis, lymphadenopathy, hematologic abnormalities (hypereosinophilia, atypical lymphocytes) and organ involvement such as hepatitis, carditis, interstitial nephritis, or interstitial pneumonitis. The cutaneous histological pattern shows a lymphocytic infiltrate, sometimes mimicking a cutaneous lymphoma, and the mortality rate is about 10%. When organ involvement exists, corticosteroids are often prescribed with dramatic improvement. Relapses may occur. (2) drug-induced pseudolymphoma which has a more insidious beginning with nodules and infiltrated plaques appearing several weeks after the beginning of the drug without constitutional symptoms. A pseudolymphoma pattern is seen on cutaneous histological slides. Complete improvement is usual after drug withdrawal, but a delayed lymphoma is possible. To decrease the ambiguity of the denomination of hypersensitivity syndrome, we propose the term of DRESS (Drug Rash with Eosinophilia and Systemic Symptoms).
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            Anticonvulsant hypersensitivity syndrome.

            L Muglia, C Vittorio (1995)
            Anticonvulsant hypersensitivity syndrome is a potentially fatal drug reaction with cutaneous and systemic reactions (incidence, one in 1000 to one in 10,000 exposures) to the arene oxide-producing anticonvulsants--phenytoin, carbamazepine, and phenobarbital sodium. In most cases, the hallmark features of fever, rash, and lymphadenopathy are accompanied by multiorgan-system abnormalities. Fatal outcomes are most often associated with liver failure. Recognition of the syndrome, which may have variable presentations, is the key to prompt discontinuation of the drug, close monitoring, and management. The reaction may be genetically determined, and siblings of patients with anticonvulsive hypersensitivity syndrome may be at increased risk of developing this syndrome. The timely recognition of anticonvulsant hypersensitivity syndrome is important, because accurate diagnosis avoids potentially fatal reexposure and affects subsequent anticonvulsant treatment options.
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              Anticonvulsant hypersensitivity syndrome: incidence, prevention and management.

              N. Shear, S. Knowles, Nina Shapiro (1999)
              Although the anticonvulsant hypersensitivity syndrome was first described in 1950, confusion still abounds regarding the syndrome. The triad of fever, rash and internal organ involvement occurring 1 to 8 weeks after exposure to an anticonvulsant heralds this rare (1 in 1,000 to 10,000 exposures) but serious reaction. Aromatic anticonvulsants [phenytoin, phenobarbital (phenobarbitone) and carbamazepine] are the most frequently involved drugs; however, there have also been several cases of anticonvulsant hypersensitivity syndrome associated with lamotrigine. Fever, in conjunction with malaise and pharyngitis, is often the first sign. This is followed by a rash which can range from a simple exanthem to toxic epidermal necrolysis. Internal organ involvement usually involves the liver, although other organs such as the kidney, CNS or lungs may be involved. Hypothyroidism may be a complication in these patients approximately 2 months after occurrence of symptoms. The aromatic anticonvulsants are metabolised to hydroxylated aromatic compounds, such as arene oxides. If detoxification of this toxic metabolite is insufficient, the toxic metabolite may bind to cellular macromolecules causing cell necrosis or a secondary immunological response. Cross-reactivity among the aromatic anticonvulsants may be as high as 75%. In addition, there is a familial tendency to hypersensitivity to anticonvulsants. Discontinuation of the anticonvulsant is essential in patients who develop symptoms compatible with anticonvulsant hypersensitivity syndrome. A minimum battery of laboratory tests, such as liver transaminases, complete blood count and urinalysis and serum creatinine, should be performed. Corticosteroids are usually administered if symptoms are severe. Patients with anticonvulsant hypersensitivity syndrome should avoid all aromatic anticonvulsants; benzodiazepines, valproic acid (sodium valproate) or one of the newer anticonvulsants can be used for seizure control. However, valproic acid should be used very cautiously in the presence of hepatitis. There is no evidence that lamotrigine cross-reacts with aromatic anticonvulsants. In addition, family counselling is a vital component of patient management.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Indian J Pharmacol
                Journal ID (iso-abbrev): Indian J Pharmacol
                Journal ID (publisher-id): IJPharm
                Title: Indian Journal of Pharmacology
                Publisher: Medknow Publications & Media Pvt Ltd (India )
                ISSN (Print): 0253-7613
                ISSN (Electronic): 1998-3751
                Publication date (Print): Nov-Dec 2012
                Volume: 44
                Issue: 6
                Pages: 798-800
                Affiliations
                [1]Department of Dermatology, Sri Dharmasthala Manjunatheshwara College of Medical Sciences and Hospital (SDMCMS and H), Sattur, Dharwad, Karnataka, India
                [1 ]Department of Paediatrics, Sri Dharmasthala Manjunatheshwara College of Medical Sciences and Hospital (SDMCMS and H), Sattur, Dharwad, Karnataka, India
                [2 ]Department of Pharmacology, Sri Dharmasthala Manjunatheshwara College of Medical Sciences and Hospital (SDMCMS and H), Sattur, Dharwad, Karnataka, India
                Author notes
                Correspondence to: Dr. Kikkeri Narayanasetty Naveen, E-mail: naveenkn80@ 123456yahoo.com
                Article
                Publisher ID: IJPharm-44-798
                DOI: 10.4103/0253-7613.103305
                PMC ID: 3523513
                PubMed ID: 23248415
                SO-VID: 904e5e75-e975-4ab6-ba82-045ebdc3def6
                Copyright © Copyright: © Indian Journal of Pharmacology
                License:

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 14 June 2012
                Date revision received : 19 July 2012
                Date accepted : 31 August 2012
                Categories
                Subject: Drug Watch

                ScienceOpen disciplines: Pharmacology & Pharmaceutical medicine
                Keywords: early detection,lamotrigine,dress
                Data availability:
                ScienceOpen disciplines: Pharmacology & Pharmaceutical medicine
                Keywords: early detection, lamotrigine, dress

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