Toxic epidermal necrolysis associated with anticonvulsants

Background and Objetives: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are considered as a continuum of the same process. TEN or Lyell Syndrome is the most severe form. Both entities involve an acute mucocutaneous blistering reaction associated with systemic inflammation. Materials and Methods: We present a case of a young woman who developed TEN following concomitant treatment with valproate, lamotrigine, and phenobarbital. Despite the extensive mucocutaneous detachment (over 90%), prognostic evaluation was favorable (SCORTEN score 2; probability of survival 88%), and this patient evolved satisfactorily. Five days after admission, valproate was reinitiated without any subsequent adverse reaction. Results: Causality evaluation identified both lamotrigine and phenobarbital as “very probable” (ALDEN score = 6) causes and valproate as “very unlikely” (ALDEN score = 0) cause of TEN. Conclusions: SJS and TEN are true life-threatening medical emergencies. This case emphasizes the importance of early diagnosis and treatment, including the discontinuation of the causative agent, which can be lifesaving.

Allopurinol: Attention to the Prescription!

Allopurinol is a commun hypo-uricemic drug. However, it is main drug reported to be inducing toxidermy. Our goal is to encourage limiting the prescription of this drug and to reserve it for justified cases after some observations. A Retrospective study was conducted in the Dermatology department between 2012 and 2019. We collected all toxidermy cases following Allopurinol. During the study period,39 cases of severe Allopurinol toxidermia, including 22 women and 17 men, a sex ratio of 0.77. The average age was 65 years old. Most of the patients were "poly-medicated". The average time between medication and clinical symptoms was 28.25 days. Clinical manifestations were: macula-papular rash in 12 cases (30%), erythroderma in 14 cases (35%), purpura in 7 cases (17%) and mucosal involvement in 20 cases. Fever in 29 cases, a state of shock in 5 cases. 10 patients required a transfer in intensive care. On the balance sheet; eosinophilia was found in 23 cases, 18 cases of hepatic cytolysis, CPK mb was elevated in 17 cases, acute renal failure in 24 cases. The biopsy was performed in all cases confirming the toxidermy. The Drug reaction eosinophilia and systemic symptoms (DRESS) retained in 29 cases, Stevens-Johnson syndrome (SJS) in 2 cases, Lyell in 4 cases. The management involved stopping the incriminated drug (Allopurinol) and the introduction of an antihistamine and an emollient were prescribed in all patients. Topical steroid in 21 patients. Oral corticosteroid therapy in 14 patients. A bolus of corticosteroid was administered in 4 patients. The evolution was good for 30 patients (77%). However, we recorded 9 deaths in a context of septic shock and multi-visceral failure. In our series, Allopurinol was the cause of severe toxidermia including Lyell syndrome, StevensJohnson and DRESS syndrome. The severity of these diseases should encourage preserving it for the justified cases, and knowing how to adapt the dosage to the renal function, to introduce the treatment in a progressive way and to stop the treatment in case of less signs of toxidermy. The control of the use of this molecule would reduce the cases of this disease.

Vajra Bhasma, Ayurvedic medicine: a rare and unusual cause of Lyell’s syndrome and its successful management

Toxic epidermolysis necrosis (TEN) or Lyell syndrome is a potentially life-threatening immunological adverse skin disease, which mostly occurs secondary to the intake of an offending drug. It commonly manifests as a widespread exfoliating bullous lesion in skin and mucous membrane mimicking superficial burns and may result in hypovolemic and/or septic shock. Authors report an unusual case of Lyell’s syndrome in a 42-year-old woman, secondary to the intake of Ayurveda medicine ‘Vajra Bhasma’ (Diamond Ash) prescribed by an Ayurveda physician for treatment of her trigeminal neuralgia. After 8th day of continuous medication intake, she had prodromal illness and rapidly developing exfoliative skin lesion extended over 80% of total body surface area, breathing difficulty, dizziness and anuria. The case was successfully managed by timely diagnosis, adequate hydration and administration of immunoglobulins. After 17 days, the skin epithelium regenerated, and she improved clinically with some depigmented lesions at discharge, which were normalised without any sequel during her further follow-up visits in hospital. Identification and withdrawal of the suspected drug, adequate resuscitation and early immunoglobulin administration are critical in management of TEN.