scholarly journals Correlation between cutaneous and ocular involvement during Lyell syndrome and Stevens-Johnson syndrome in children

2021 ◽  
Vol 7 (3) ◽  
pp. 90-95
Author(s):  
  Kenza Kandri Rody ◽  
Mariam Zakoun ◽  
Sarah Belghmaidi ◽  
Ahmed Ghazza ◽  
Ibtissam Hajji ◽  
...  
2016 ◽  
Vol 25 (3) ◽  
pp. 180-186
Author(s):  
Pınar ALTIAYLIK ÖZER ◽  
Bengi Ece KURTUL ◽  
Gönül TANIR ◽  
Türkan AYDIN TEKE ◽  
Zeynep Gökçe GAYRETLI AYDIN ◽  
...  

2020 ◽  
Vol 38 (5_suppl) ◽  
pp. 102-102
Author(s):  
Gabriel E. Molina ◽  
Zizi Yu ◽  
Ruth K. Foreman ◽  
Kerry Lynn Reynolds ◽  
Steven T. Chen

102 Background: Stevens-Johnson syndrome (SJS) is a rare, life-threatening mucocutaneous toxicity that can occur in patients receiving immune checkpoint inhibitors (ICIs). ICI-induced SJS is scarcely reported in the literature and thus remains poorly understood, particularly regarding features that may distinguish it from classic SJS. Methods: To describe the timing, clinical manifestations, and treatment course of ICI-induced SJS, this multicenter, retrospective study identified seven patients with SJS in the setting of ICI use from January 2011 through May 2019. Results: All seven patients presented initially as benign, limited drug eruptions after a median of 4 ICI cycles (range, 1-7) and 63 days (range, 13-253 days) from ICI initiation. While none of the patients had prior drug allergies, all 7 were receiving new, recently initiated – i.e., within two months – medications at the time of rash onset. Cases demonstrated characteristic histologic findings of SJS, such as epidermal necrosis, and occasional unusual features, including interface dermatitis. All patients responded favorably and rapidly to systemic therapy, primarily intravenous corticosteroids, with near immediate symptomatic resolution and cessation of progressive skin blistering or detachment. Median length of stay was 11 days and no patients died from SJS. Conclusions: Our cohort defines an atypical SJS-like reaction secondary to ICI use, which is distinct from classic SJS in its delayed onset, mild initial presentation, and rare ocular involvement. The association with concomitant medication use suggests a potential mechanism whereby ICIs reduce patient immune tolerance to subsequent drug exposures. Reassuringly, this atypical SJS-like phenomenon exhibits a benign clinical course and favorable response to standard treatments.


2020 ◽  
Vol 15 (1) ◽  
Author(s):  
D. Thorel ◽  
S. Ingen-Housz-Oro ◽  
G. Royer ◽  
A. Delcampe ◽  
N. Bellon ◽  
...  

Abstract Stevens-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) can lead to severe ophthalmologic sequelae. The main risk factor is the severity of the initial ocular involvement. There are no recommendations for ocular management during acute phase. We conducted a national audit of current practice in the 11 sites of the French reference center for toxic bullous dermatoses and a review of the literature to establish therapeutic consensus guidelines. We sent a questionnaire on ocular management practices in SJS/ TEN during acute phase to ophthalmologists and dermatologists. The survey focused on ophthalmologist opinion, pseudomembrane removal, topical ocular treatment (i.e. corticosteroids, antibiotics, antiseptics, artificial tear eye drops, vitamin A ointment application), amniotic membrane transplantation, symblepharon ring use, and systemic corticosteroid therapy for ophthalmologic indication. Nine of 11 centers responded. All requested prompt ophthalmologist consultation. The majority performed pseudomembrane removal, used artificial tears, and vitamin A ointment (8/9, 90%). Combined antibiotic-corticosteroid or corticosteroid eye drops were used in 6 centers (67%), antibiotics alone and antiseptics in 3 centers (33%). Symblepharon ring was used in 5 centers (55%) if necessary. Amniotic membrane transplantation was never performed systematically and only according to the clinical course. Systemic corticosteroid therapy was occasionally used (3/9, 33%) and discussed on a case-by-case basis. The literature about ocular management practice in SJS/ TEN during acute phase is relatively poor. The role of specific treatments such as local or systemic corticosteroid therapy is not consensual. The use of preservatives, often present in eye drops and deleterious to the ocular surface, is to be restricted. Early amniotic membrane transplantation seems to be promising.


2021 ◽  
Vol 8 ◽  
Author(s):  
Derek Metcalfe ◽  
Omer Iqbal ◽  
James Chodosh ◽  
Charles S. Bouchard ◽  
Hajirah N. Saeed

Stevens Johnson syndrome and toxic epidermal necrolysis are on a spectrum of a severe, immune-mediated, mucocutaneous disease. Ocular involvement occurs in the vast majority of cases and severe involvement can lead to corneal blindness. Treatment in the acute phase is imperative in mitigating the severity of chronic disease. Advances in acute treatment such as amniotic membrane transplantation have shown to significantly reduce the severity of chronic disease. However, AMT is not a panacea and severe chronic ocular disease can and does still occur even with aggressive acute treatment. Management of chronic disease is equally critical as timely intervention can prevent worsening of disease and preserve vision. This mini-review describes the acute and chronic findings in SJS/TEN and discusses medical and surgical management strategies.


1982 ◽  
Vol 100 (9) ◽  
pp. 1453-1454 ◽  
Author(s):  
B. J. Mondino ◽  
S. I. Brown ◽  
A. W. Biglan

2003 ◽  
Vol 27 (1) ◽  
pp. 71-76 ◽  
Author(s):  
K. Donta –Bakoyianni ◽  
A. Mitsea ◽  
K. Deodoropoulou-Papadimitriou

Erythema Multiforme (EM) is a rare mucocutaneous disease with a variety of clinical manifestations. EM it was recognized in the early 1800's, and still the etiology is unknown. It has been recently suggested erythema multiforme (EM) major and Stevens-Johnson Syndrome (SJS) could be separated as two distinct clinical disorders with similar mucosal erosions, but different patterns of cutaneous lesions. In particular SJS should be used for a syndrome characterized by mucous membrane erosions and widespread small blisters that appear on erythematous or purpuric maculae, which are different from classic targets. In SJS mouth, eyes, skin, genitalia and occasionally the esophagus and respiratory track may be affected. Oral lesions may cause severe pain and usually lips may become encrusted. Concerning ocular involvement, if there is conjunctivitis or uveitis this may lead to scarring and blindness. Also, the course of disease and the prognosis are in most cases severe.


2020 ◽  
Vol 3 (S2) ◽  
pp. 3-6
Author(s):  
Achehboune Kaoutar ◽  
◽  
Baybay Hanane ◽  
Douhi Zakia ◽  
Elloudi Sara ◽  
...  

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.


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