toxic (bullous) epidermal necrolysis (Lyell syndrome, TEN)
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Etiology
- allergic reaction, almost always to a drug
- pharmaceutical agents (~90%)[3]
- antibiotics: (21%)[10], 32%[11]
- anticonvulsants: (19%)[10]
- NSAIDs: (11%)[10]
- meloxicam, piroxicam, tenoxicam
- diclofenac, indomethacin, ionazolac, etodolac, aceclofenac, sulindac, ketorolac, phenylbutazone
- other:
- see pharmaceutical agents causing SCARs
- less common causes:
- infection (4%)[3]
- radiation, malignancy, vaccination, graft vs. host
Epidemiology
- all age groups affected; uncommon in children
- female predominance (1.5-2:1)
- high incidence in HIV+ patients (1/1000/year)
Pathology
Genetics
- drug-induced TEN (carbamazepine, phenytoin) linked to HLA-B*1502 in Han Chinese (Asians, South Asian Indians)
Clinical manifestations
- prodrome of malaise, anorexia, arthralgias, fever & upper respiratory tract infection 1-3 days before appearance of skin lesions
- skin & mucous membrane manifestations
- initially, warm, diffuse erythema, spreading from face & genitalia to trunk & extremities
- later, generalized tender erythema, often with bullae formation
- dusky patches with imminent blistering
- suggests incipient skin necrosis & detachment
- >= 30% of body surface involved
- Nikolsky's sign
- early blistering, eroding oral-labial lesions (90%)
- sloughing & necrosis of entire epidermis
- purulent conjunctivitis (75%)
Laboratory
- blood cultures
- complete blood count (CBC)
- basic metabolic panel
- liver function tests
- erythrocyte sedimentation rate may be elevated
- urinalysis
- skin biopsy to ensure appropriate diagnosis[3]
- do not screen for SJS or TEN with HLA-B*1502 & HLA-B*5801[3]
Complications
- death generally occurs from sepsis that results from epidermal & mucosal necrosis (25-39%)[3]
- GI hemorrhage
- pulmonary embolism
- long-term sequella SJS/TEN[9]
- post-inflammatory dsypigmentation: hyperpigmentation, hypopigmentation, or a combination
- hypertrophic or keloidal scars
- nail changes: onycholysis or onychomadesis, onychorrhexis, onychoschizia, koilonychia, erythronychia, oil-drop sign
- nail loss may be permanent (20%)
- hair changes: telogen effluvium is common
- eruptive nevi & atypical nevi
- other cutaneous manifestations: pruritus, hyperhidrosis, photosensitivity, heterotopic ossification, ectopic sebaceous glands
Differential diagnosis
- Stevens-Johnson syndrome (SJS) & toxic epidermal necrolysis (TEN) represent a continuum of a single disorder
- TEN is a severe form of SJS
- in SJS, skin detachment involves < 10% of body area
- in TEN, skin detachment involves > 30% of body area
- in SJS-TEN overlap syndrome, 10-30% of body area involved
- see Stevens-Johnson syndrome for more broad differential diagnosis
Management
- treat like a burn
- hospitalize in intensive care or burn unit
- discontinue offending agent(s)
- rehydrate & correct electrolyte imbalances
- aggressive treatment of suspected infections
- Staphylococcus
- Pseudomonas
- no role for prophylactic antibiotics[3]
- use of glucocorticoids is controversial
- intravenous immune globulins used[3]
- cyclosporine has been used[10]
Prognosis:
- overall mortality is 30% (10%-70%, 20%[10])
- poorer prognosis with advanced age, extensive surface area involved & azotemia
- see SCORTEN severity index score
More general terms
Additional terms
- pharmaceutical agents causing severe cutaneous adverse reactions (SCARs)
- SCORTEN severity index score
- Stevens-Johnson syndrome (SJS)
References
- ↑ Harrison's Principles of Internal Medicine, 13th ed. Companion Handbook, Isselbacher et al (eds), McGraw-Hill Inc. NY, 1995, pg 829-39
- ↑ H. Quinny Cheng, USSF Fresno lecture, Oct 21, 1998
- ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 Medical Knowledge Self Assessment Program (MKSAP) 11, 15, 16, 17, 18. American College of Physicians, Philadelphia 1998, 2009, 2012, 2015, 2018.
- ↑ Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 94
- ↑ 5.0 5.1 Cohen V, Bronze MS (images) Medscape: Toxic Epidermal Necrolysis http://emedicine.medscape.com/article/229698-overview
- ↑ 6.0 6.1 DermNet NZ. Toxic epidermal necrolysis (images) http://dermnetnz.org/doctors/emergencies/ten.html
- ↑ Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis: part I. Introduction, history, classification, clinical features, systemic manifestations, etiology, and immunopathogenesis. J Am Acad Dermatol 2013 Aug; 69:173.e1. PMID: https://www.ncbi.nlm.nih.gov/pubmed/23866878
Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis: part II. Prognosis, sequelae, diagnosis, differential diagnosis, prevention, and treatment. J Am Acad Dermatol 2013 Aug; 69:187.e1 PMID: https://www.ncbi.nlm.nih.gov/pubmed/23866879 - ↑ 8.0 8.1 NEJM Knowledge+ Question of the Week. Nov 8, 2016 http://knowledgeplus.nejm.org/question-of-week/1452/
- ↑ 9.0 9.1 Heymann E Stevens-Johnson Syndrome-Toxic Epidermal Necrolysis: The Aftermath. AAD Reading Room Content MedPage Today. June 22, 2021 https://www.medpagetoday.com/reading-room/aad/general-dermatology/93214
- ↑ 10.0 10.1 10.2 10.3 10.4 10.5 10.6 Kridin K, Bruggen MC, Chua SL et al Assessment of Treatment Approaches and Outcomes in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. Insights From a Pan-European Multicenter Study. JAMA Dermatol. Published online August 25, 2021. PMID: https://www.ncbi.nlm.nih.gov/pubmed/34431984 https://jamanetwork.com/journals/jamadermatology/fullarticle/2783034
- ↑ 11.0 11.1 Lee EU, Knox C, Phillips EJ Worldwide Prevalence of Antibiotic-Associated Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Systematic Review and Meta-analysis. JAMA Dermatol. Published online February 15, 2023. PMID: https://www.ncbi.nlm.nih.gov/pubmed/36790777 https://jamanetwork.com/journals/jamadermatology/fullarticle/2801093
- ↑ Noe MH, Micheletti RG. Diagnosis and management of Stevens-Johnson syndrome/toxic epidermal necrolysis. Clin Dermatol. 2020;38:607-12. PMID: https://www.ncbi.nlm.nih.gov/pubmed/33341195
- ↑ Charlton OA, Harris V, Phan K, et al. Toxic epidermal necrolysis and Steven-Johnson syndrome: A comprehensive review. Adv Wound Care (New Rochelle). 2020;9:426-439. PMID: https://www.ncbi.nlm.nih.gov/pubmed/32520664