IKZF1, a new susceptibility gene for cold medicine–related Stevens-Johnson syndrome/toxic epidermal necrolysis with severe mucosal involvement

Erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis are related disorders of skin and mucous membranes, which are typically associated with antecedent medication use or infection. We review 108 cases of erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis from Texas Children's Hospital, Houston, Texas, from 1981 to 1991, and illustrate the characteristic skin and mucosal lesions. In addition, we describe in detail two unusual cases requiring intensive airway management. Head and neck manifestations were present in 4 of 79 patients (5%) with erythema multiforme and 26 of 28 patients (93%) with Stevens-Johnson syndrome. In Stevens-Johnson syndrome, mucosal involvement of the lip (93%), conjunctiva (82%), oral cavity (79%), and nose (36%) were most common. Antecedent medication use was identified in 59% of erythema multiforme patients and 68% of Stevens-Johnson syndrome patients. We note a striking increase in the number of cases in our series caused by cephalosporins. Fifty percent of Stevens-Johnson syndrome patients required supplemental hydration or alimentation because of the severity of the oral cavity involvement. The head and neck mucosal manifestations largely respond to local care, and the routine use of prophylactic antibiotics or systemic steroids is not recommended.

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A clinical study of Stevens-Johnson syndrome and toxic epidermal necrolysis in a tertiary centre, South India

International Journal of Research in Dermatology ◽

10.18203/issn.2455-4529.intjresdermatol20170802 ◽

2017 ◽

Vol 3 (1) ◽

pp. 134

Author(s):

Rahima S. ◽

Abdul Latheef E. N. ◽

Pavithran K. ◽

Saleem P. M.

Keyword(s):

Toxic Epidermal Necrolysis ◽

South India ◽

Detailed Examination ◽

Stevens Johnson Syndrome ◽

Hiv Positive ◽

Tertiary Centre ◽

Johnson Syndrome ◽

Mucosal Involvement ◽

Common Causes ◽

Eye Involvement

Background: Stevens Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are considered as the severest end of spectrum of erythema multiforme. Various etiologies like infections, drugs and malignancies have been proposed. The aim of the present study was to know the incidence, common causes, clinical course of SJS and TEN and to estimate the morbidity and mortality.Methods: A 2 year study of patients presenting with SJS and TEN was carried out. A detailed examination to know the cutaneous and mucosal involvement was done. Biopsy was done in 3 patients.Results: There were fifty patients of SJS-TEN spectrum. Of which 31 were SJS, 3 had SJS-TEN overlap and 16 had TEN. Anticonvulsants were implicated in causing these reactions in 24 patients (48%) with carbamazepine being the most common i.e. in 16 patients (32%). Sparing of pressure areas like the strap area of brassier and waist was noticed in two patients (4%). The most common complication was due to eye involvement seen in 20 patients (40%). 46 patients were treated with steroids and of the remaining, 3 were children and one was HIV positive. Only three patients with TEN (6%) died. Conclusions: To conclude, TEN was less common than SJS, had more sequelae and more mortality compared to SJS.

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MANAGEMENT OF ORAL LESIONS ASSOCIATED WITH CARBAMAZEPINE RELATED STEVENS-JOHNSON SYNDROME / TOXIC EPIDERMAL NECROLYSIS OVERLAP PATIENT

Dentika Dental Journal ◽

10.32734/dentika.v19i2.456 ◽

2016 ◽

Vol 19 (2) ◽

pp. 154-159

Author(s):

Dewi Puspasari ◽

Irna Sufiawati

Keyword(s):

Toxic Epidermal Necrolysis ◽

Fas Ligand ◽

Total Body Surface Area ◽

Stevens Johnson Syndrome ◽

Cytotoxic T Cell ◽

Mean Corpuscular Hemoglobin ◽

Oral Lesions ◽

Treatment Standard ◽

Johnson Syndrome ◽

Mucosal Involvement

Stevens–Johnson syndrome (SJS)/ Toxic Epidermal Necrolysis (TEN) are acute, self-limited, potentially life-threatening mucocutaneous disease. Oral mucosal involvement manifest as extensive erosions and haemorrhagic crusting, which can interfere oral functions causing odynophagia, inability to tolerate solid foods, and increased aspiration risk. A 40-year-old female patient was referred from Dermatology and Venereology department with diagnosis SJS/TEN overlap. The patient complained mouth opening difficulty due to mouth and lip sores. Drug history revealed positive intake of carbamazepine. Extraoral examination revealed multiple diffuse discrete facial lesions, conjunctival hyperemia, erosions and hemorrhagic crusting lips. Intraoral examination revealed white yellowish plaque, and erosions on buccal mucosa, palate, floor of the mouth, dorsal, ventral, and lateral tongue. Laboratory investigation revealed decrease of haemoglobin, hematocrite, Mean Corpuscular Volume (MCV), Mean Corpuscular Hemoglobin (MCH), thrombocyte, eosinophil, band of eosinophil, lymphocyte, natrium, potassium, and calcium. Oral lesions associated with SJS/TEN overlap diagnosis was made. Chlorhexidine gluconate 0,1%, nystatin oral suspension, vitamin B12, folic acid, and corticosteroid unguent compounding were given, which showed improvement of oral lesions in 3 weeks. SJS/TEN are the same disease spectrum of delayed hypersensitivity reaction leading to keratinocyte apoptosis through cytotoxic T-cell mediated Fas-Fas ligand, perforin/ granzyme B, and granulysin, which distinguished primarily by severity and percentage of total body surface area involved.Currently, an optimal treatment standard for SJS/TEN patients remains unavailable. Oral lesions management play significant role in enhancing patients’ quality of life and achieving better prognosis in SJS/TEN overlap patients through multidisciplinary approach.

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Toxic epidermal necrolysis associated with pembrolizumab

Journal of Oncology Pharmacy Practice ◽

10.1177/1078155219890659 ◽

2019 ◽

Vol 26 (5) ◽

pp. 1259-1265 ◽

Cited By ~ 1

Author(s):

Zhuo Ran Cai ◽

Julie Lecours ◽

Jean-Philippe Adam ◽

Isabelle Marcil ◽

Normand Blais ◽

...

Keyword(s):

Toxic Epidermal Necrolysis ◽

Surface Area ◽

Body Surface Area ◽

Body Surface ◽

Stevens Johnson Syndrome ◽

First Case ◽

Johnson Syndrome ◽

Programmed Death ◽

Mucosal Involvement ◽

Programmed Death 1

Introduction Stevens-Johnson syndrome and toxic epidermal necrolysis are severe cutaneous drug eruptions characterized by epidermal detachment. Pembrolizumab is a monoclonal antibody that binds to the programmed death-1 receptor, and it has been associated with numerous cutaneous adverse side-effects, including Stevens-Johnson syndrome. Case report We describe a 63-year-old male with metastatic lung adenocarcinoma who developed a rapidly progressing maculopapular rash three days after a first dose of pembrolizumab. On day 16, the rash affected more than 80% of body surface area with detachment of large sheets of necrolytic epidermis in 30–40% of body surface area. However, the patient only presented with mild mucosal involvement. Histopathologic examination of a skin biopsy showed a subepidermal blister with overlying prominent full thickness epidermal keratinocytic necrosis and a superficial perivascular infiltrate of lymphocytes. A toxic epidermal necrolysis secondary to pembrolizumab was then diagnosed. Management and outcome: In addition to supportive cares, the patient received corticosteroids and cyclosporine. The patient responded rapidly to the immunosuppressant therapy, and nearly complete re-epithelialization was achieved 24 days after the start of the reaction. Discussion In our review of the literature, 15 other cases of Stevens-Johnson syndrome/toxic epidermal necrolysis were reported with programmed death-1/programmed cell death ligand-1 inhibitors. To our knowledge, this is the first case of toxic epidermal necrolysis secondary to pembrolizumab published in the literature. The American Society of Clinical Oncology guidelines suggest that cyclosporine, in addition to corticosteroids, be initiated when toxic epidermal necrolysis is suspected. Clinicians should be aware of this rare dermatological emergency with the increasing use of pembrolizumab in oncology.

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