Stevens–Johnson syndrome
| Stevens–Johnson syndrome | |
|---|---|
| Man with characteristic skin lesions of Stevens–Johnson syndrome | |
| Specialty | Dermatology |
| Symptoms | Fever, skin blisters, skin peeling, painful skin, red eyes[1] |
| Complications | Dehydration, sepsis, pneumonia, multiple organ failure.[1] |
| Usual onset | Age < 30[2] |
| Causes | Certain medications, certain infections, unknown[2][1] |
| Risk factors | HIV/AIDS, systemic lupus erythematosus, genetics[1] |
| Diagnostic method | <10% of the skin involved, skin biopsy[2] |
| Differential diagnosis | Chickenpox, staphylococcal epidermolysis, staphylococcal scalded skin syndrome, autoimmune bullous disease, Smallpox[3] |
| Treatment | Hospitalization, stopping the cause[2] |
| Medication | Pain medication, antihistamines, antibiotics, corticosteroids, intravenous immunoglobulins[2] |
| Prognosis | Mortality ~7.5%[1][4] |
| Frequency | 1–2 per million per year (together with TEN)[1] |
Stevens–Johnson syndrome (SJS) is a type of severe skin reaction.[1] Together with toxic epidermal necrolysis (TEN) and Stevens–Johnson/toxic epidermal necrolysis (SJS/TEN) overlap, they are considered febrile mucocutaneous drug reactions and probably part of the same spectrum of disease, with SJS being less severe.[1][5][3] Erythema multiforme (EM) is generally considered a separate condition.[6] Early symptoms of SJS include fever and flu-like symptoms.[1] A few days later, the skin begins to blister and peel, forming painful raw areas.[1] Mucous membranes, such as the mouth, are also typically involved.[1] Complications include dehydration, sepsis, pneumonia and multiple organ failure.[1]
The most common cause is certain medications such as lamotrigine, carbamazepine, allopurinol, sulfonamide antibiotics and nevirapine.[1] Other causes can include infections such as Mycoplasma pneumoniae and cytomegalovirus, or the cause may remain unknown.[2][1] Risk factors include HIV/AIDS and systemic lupus erythematosus.[1]
The diagnosis of Stevens–Johnson syndrome is based on involvement of less than 10% of the skin.[2] It is known as TEN when more than 30% of the skin is involved and considered an intermediate form when 10–30% is involved.[3] SJS/TEN reactions are believed to follow a type IV hypersensitivity mechanism.[7] It is also included with drug reaction with eosinophilia and systemic symptoms (DRESS syndrome), acute generalized exanthematous pustulosis (AGEP) and toxic epidermal necrolysis in a group of conditions known as severe cutaneous adverse reactions (SCARs).[8]
Treatment typically takes place in hospital such as in a burn unit or intensive care unit.[2] Efforts may include stopping the cause, pain medication, antihistamines, antibiotics, intravenous immunoglobulins or corticosteroids.[2] Together with TEN, SJS affects 1 to 2 people per million per year.[1] Typical onset is under the age of 30.[2] Skin usually regrows over two to three weeks; however, complete recovery can take months.[2] Overall, the risk of death with SJS is 5 to 10%.[1][4]
- ^ a b c d e f g h i j k l m n o p q "Stevens-Johnson syndrome/toxic epidermal necrolysis". Genetics Home Reference. July 2015. Archived from the original on April 27, 2017. Retrieved April 26, 2017.
- ^ a b c d e f g h i j k "Stevens-Johnson syndrome". GARD. Archived from the original on August 28, 2016. Retrieved August 26, 2016.
- ^ a b c "Orphanet: Toxic epidermal necrolysis". Orphanet. November 2008. Archived from the original on April 27, 2017. Retrieved April 26, 2017.
- ^ a b Lerch M, Mainetti C, Terziroli Beretta-Piccoli B, Harr T (2017). "Current Perspectives on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis". Clinical Reviews in Allergy & Immunology. 54 (1): 147–176. doi:10.1007/s12016-017-8654-z. PMID 29188475. S2CID 46796285.
- ^ Creamer, D.; Walsh, S.A.; Dziewulski, P.; Exton, L.S.; Lee, H.Y.; Dart, J.K.G.; Setterfield, J.; Bunker, C.B.; Ardern-Jones, M.R.; Watson, K.M.T.; Wong, G.A.E.; Philippidou, M.; Vercueil, A.; Martin, R.V.; Williams, G. (June 2016). "U.K. guidelines for the management of Stevens–Johnson syndrome/toxic epidermal necrolysis in adults 2016". British Journal of Dermatology. 174 (6): 1194–1227. doi:10.1111/bjd.14530. ISSN 0007-0963. PMID 27317286.
- ^ Schwartz, RA; McDonough, PH; Lee, BW (August 2013). "Toxic epidermal necrolysis: Part I. Introduction, history, classification, clinical features, systemic manifestations, etiology, and immunopathogenesis". Journal of the American Academy of Dermatology. 69 (2): 173.e1–13, quiz 185–6. doi:10.1016/j.jaad.2013.05.003. PMID 23866878.
- ^ Hyzy, Robert C. (2017). Evidence-Based Critical Care: A Case Study Approach. Springer. p. 761. ISBN 9783319433417.
- ^ Adler NR, Aung AK, Ergen EN, Trubiano J, Goh MS, Phillips EJ (2017). "Recent advances in the understanding of severe cutaneous adverse reactions". The British Journal of Dermatology. 177 (5): 1234–1247. doi:10.1111/bjd.15423. PMC 5582023. PMID 28256714.