Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

Abstract

Severe ENT form is associated with pulmonary infection and is easily detected by nasal fiberoptic endoscopy. ENT evaluation should be suggested when dysphonia or dyspnea is observed at the acute stage of SJS/TEN.

Epidermal necrolysis French national diagnosis and care protocol (PNDS; protocole national de diagnostic et de soins)

“…A rigorous clinical evaluation of the mucosal lesions is crucial during the acute phase and should be performed daily for accessible sites, including the outer ear. A specialized examination should be performed once weekly during the acute phase, for ENT and genital mucosae [ 43 ]. Antiseptic and analgesic mouth washes [ 44 ] should be used several times daily.…”

“… ► Dermatological examination of the skin, hair and nails [ 58 ]; ► Systematic ophthalmological examination, including checks for dry-eye syndrome and symblepharons, and a slit-lamp examination to guide subsequent surveillance and treatment; ► Buccal examination, including a dental examination, checks for dryness syndrome and a panoramic dental X ray in children [ 59 ]; ► Gynecological examination and checks for synechia and vulvovaginal dryness [ 60 ]; ► ENT examination [ 43 ]; ► Clinical pulmonary examination and respiratory function testing, preferably 2 months after the acute phase and at 1 year if the patient has symptoms; ► Psychiatric evaluation and offer of psychological support to the patient and his/her family (due to the high frequency of post-traumatic stress syndrome in these patients) [ 61 ]. …”

Oropharyngolaryngeal manifestations in severe toxic epidermal necrolysis: a single‐center's retrospective case series

“…To our knowledge, this is only the second case series reporting ENT lesions and their complications in adult patients with toxic epidermal necrolysis. Bequignon et al 5 were the first to publish a case series of 49 patients who suffered of SJS or TEN and ENT mucosa involvement, and few others reporting hypopharyngeal stenosis. 11,12 In Bequignon's case series, 5 only 16% of the patients were treated with mechanical ventilation (vs. 81% in our case series) and had a significantly lower mean TBSA involved (20% vs. 58%) and median SCORTEN-score (1 vs. 3).…”

“…SJS and TEN universally involve skin and mucous membranes, such as in oral cavity, ocular surface, genitals, respiratory, and gastrointestinal tract. 5 There seems to be a slightly higher prevalence of the disease in female patients (ratio 1.5 : 1). 4 Cutaneous involvement appears as erythematous lesions, mainly on the sign, and sheet of denuded epidermis.…”

“…6 In up to 80% of cases, there is also mucosal involvement consisting of mucositis and ulceration, 4,7 with two or more sites involved but involvement of the oral cavity is found in almost all cases. 4,5 The SCORTEN score 8 is used to assess the severity and the prognosis of the disease, and patients with SCORTEN ≥ 2 or a detachment >10% TBSA should preferably be managed in specialized centers or burn intensive care units, as it could decrease the mortality. 9 Rapid withdrawal of the causative drug and supportive care are the mainstay of the management involving multiple specialties (intensive care unit, dermatology, immunology, ophthalmology, gynecology, and ENT).…”