An atypical presentation of pulmonary air‐leak syndrome and multisystem toxic epidermal necrolysis

Ellenburg, Joseph Trent; Josey, David

doi:10.1002/ppul.22908

Cited by 6 publications

(3 citation statements)

References 40 publications

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“…Mediastinal emphysema and pneumothorax in patients with SJS/TEN are rare, and their pathogenesis with SJS/TEN has been proposed to result from airway obstruction caused by detached airway mucosa, air trapping caused by coughing, and destruction of alveoli 14. In the present case, mediastinal and subcutaneous emphysema developed several days after admission, with no findings of pulmonary air leakage on presentation.…”

Section: Discussionmentioning

confidence: 51%

Rare case of Stevens-Johnson syndrome with bronchiolitis obliterans as a chronic complication

et al. 2022

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A young girl in her teens presented with fever, rashes and various mucocutaneous symptoms. Flat erythematous macules were seen mainly on the limbs, without blisters or skin detachments. The lips were swollen with crusts and haemorrhage. The oral cavity and pharynx showed ulcerative lesions with exudate. Severe bilateral ocular lesions with pseudomembrane formation and corneal epithelial defects were present. Also, urogenital lesion and gastrointestinal symptoms with frequent haematochezia were observed. Her symptoms and pathological findings were consistent with Stevens-Johnson syndrome. She was treated with prednisolone and methylprednisolone pulse therapy. Her ocular and cutaneous symptoms improved without severe chronic complications. However, 1 month later, she developed dyspnoea, and a pulmonary function test revealed severe obstructive ventilation disorder. After discharge, she was regularly followed up for respiratory complications. High-resolution chest CT performed 9 months after onset revealed mosaic perfusions and bronchiectasis, consistent with bronchiolitis obliterans.

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Section: Discussionmentioning

confidence: 51%

Rare case of Stevens-Johnson syndrome with bronchiolitis obliterans as a chronic complication

et al. 2022

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“…A retrospective study of 32 SJS/TEN cases found 50% to have abnormal lung function tests during routine follow-up (61). In the published literature, RS involvement following SJS/TEN has been characterized by chronic lung disease, bronchiolitis obliterans, interstitial lung disease, pulmonary air leak syndrome, laryngeal obstruction, and obliterative bronchitis, among others (57,(61)(62)(63)(64)(65)(66)(67)(68)(69)(70)(71)(72)(73)(74)(75)(76). The genitourinary system is also not spared in SJS/TEN.…”

Section: Internal Organ Involvement As An Additional Marker Of Steven...mentioning

confidence: 99%

Disease severity and status in Stevens–Johnson syndrome and toxic epidermal necrolysis: Key knowledge gaps and research needs

Lehloenya

2022

Front. Med.

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Stevens–Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are on a spectrum of cutaneous drug reactions characterized by pan-epidermal necrosis with SJS affecting < 10% of body surface area (BSA), TEN > 30%, and SJS/TEN overlap between 10 and 30%. Severity-of-illness score for toxic epidermal necrolysis (SCORTEN) is a validated tool to predict mortality rates based on age, heart rate, BSA, malignancy and serum urea, bicarbonate, and glucose. Despite improved understanding, SJS/TEN mortality remains constant and therapeutic interventions are not universally accepted for a number of reasons, including rarity of SJS/TEN; inconsistent definition of cases, disease severity, and endpoints in studies; low efficacy of interventions; and variations in treatment protocols. Apart from mortality, none of the other endpoints used to evaluate interventions, including duration of hospitalization, is sufficiently standardized to be reproducible across cases and treatment centers. Some of the gaps in SJS/TEN research can be narrowed through international collaboration to harmonize research endpoints. A case is made for an urgent international collaborative effort to develop consensus on definitions of endpoints such as disease status, progression, cessation, and complete re-epithelialization in interventional studies. The deficiencies of using BSA as the sole determinant of SJS/TEN severity, excluding internal organ involvement and extension of skin necrosis beyond the epidermis, are discussed and the role these factors play on time to healing and mortality beyond the acute stage is highlighted. The potential role of artificial intelligence, biomarkers, and PET/CT scan with radiolabeled glucose as markers of disease status, activity, and therapeutic response is also discussed.

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“…These air-leak syndromes are postulated to occur in the setting of alveolar rupture that results from air trapping due to the obstruction of airways by mucosal debris. Such complications are more frequently seen in pediatric patients, [37][38][39] possibly due to the occlusive effect on smaller airways.…”

Section: Acute Respiratory Complicationsmentioning

confidence: 99%

Managing respiratory complications in Stevens‐Johnson Syndrome and Toxic Epidermal Necrolysis

Lee

et al. 2021

Int J Dermatology

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In the recently published guidelines by the Society of Dermatology Hospitalists on the management of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), a brief section was included on airway management. These recommendations provide an easy reference on how to manage respiratory complications of the disease. Understanding the evidence that underlies these recommendations would offer physicians greater clarity on the considerations behind every decision and treatment offered. We present a review of the literature on respiratory manifestations associated with SJS and TEN. In addition, we aim to address specific concerns regarding the respiratory management of these patients.These include issues such as the indications and optimal timing of intubation, tracheostomy, role of flexible nasoendoscopy, bronchoscopy, ventilation strategies, and management of chronic respiratory complications.

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An atypical presentation of pulmonary air‐leak syndrome and multisystem toxic epidermal necrolysis

Cited by 6 publications

References 40 publications

Rare case of Stevens-Johnson syndrome with bronchiolitis obliterans as a chronic complication

Rare case of Stevens-Johnson syndrome with bronchiolitis obliterans as a chronic complication

Disease severity and status in Stevens–Johnson syndrome and toxic epidermal necrolysis: Key knowledge gaps and research needs

Managing respiratory complications in Stevens‐Johnson Syndrome and Toxic Epidermal Necrolysis

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