Stevens-Johnson syndrome overview: Difference between revisions
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==Overview== | ==Overview== | ||
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Common risk factors in the development of SJS include | Common risk factors in the development of SJS include Immunodeficiency, HIV infection, Active malignancy (particularly Hematological cancers), Genetic Predisposition ( HLA-B 1502 and HLA-B 5801 variants in particular) on exposure to antiepileptic medications or Allopurinol. Other risk factors may include past or family history of SJS/TEN, Radiation therapy and Rapid use of medications. | ||
==Epidemiology and Demographics== | ==Epidemiology and Demographics== |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anila Hussain, MD [2]
Overview
Stevens-Johnson syndrome is a life-threatening condition affecting the skin, in which due to cell death the epidermis separates from the dermis. The syndrome is thought to be a hypersensitivity complex affecting the skin and the mucous membranes. Although the majority of cases are idiopathic, the main class of known causes is medications, followed by infections and (rarely) cancers.
Historical Perspective
It is named for Dr. Albert Mason Stevens and Dr. Frank Chambliss Johnson, American pediatricians who jointly published a description of the disorder in 1922.[1][2]
Classification
There is agreement in the medical literature that Stevens-Johnson syndrome can be considered a milder form of toxic epidermal necrolysis (TEN). However, there is debate whether it falls on a spectrum of disease that includes erythema multiforme and erythema multiforme major (also known as erythema multiforme majus).[3][4][5][6][7]
Pathophysiology
SJS, like toxic epidermal necrolysis and erythema multiforme, are characterized by confluent epidermal necrosis with minimal associated inflammation. The acuity is apparent from the (normal) basket weave-like pattern of the stratum corneum.
Causes
Stevens-Johnson Syndrome is caused by drug reactions in the majority of the cases although it is also seen in certain viral and bacterial infections, and certain malignancies. Most commonly implicated drugs include sulfa drugs, Allopurinol, Anti-Epileptics (Phenobarbital, Carbamazepine, Lamotrigine), Antibiotics (for example Penicillin), and NSAIDs. Infections that are associated with SJS may include HIV, HSV, Mumps, Mycoplasma, Mycobacteria, Hepatitis and Streptococcal Infection.
Risk Factors
Common risk factors in the development of SJS include Immunodeficiency, HIV infection, Active malignancy (particularly Hematological cancers), Genetic Predisposition ( HLA-B 1502 and HLA-B 5801 variants in particular) on exposure to antiepileptic medications or Allopurinol. Other risk factors may include past or family history of SJS/TEN, Radiation therapy and Rapid use of medications.
Epidemiology and Demographics
SJS is a rare condition, with a reported incidence of around one case per 100,000 people per year.
Natural History, Complications and Prognosis
SJS proper (with less than 10% of body surface area involved) has the mortality rate of around 5%. The risk for death can be estimated using the SCORTEN scale, which takes a number of prognostic indicators into account. Other outcomes include organ damage and blindness.
Diagnosis
History and Symptoms
Stevens–Johnson syndrome (SJS) usually begins with fever, sore throat, and fatigue, which is misdiagnosed and usually treated with antibiotics. Ulcers and other lesions begin to appear in the mucous membranes, almost always in the mouth and lips but also in the genital and anal regions. Those in the mouth are usually extremely painful and reduce the patient's ability to eat or drink. Conjunctivitis of the eyes occurs in about 30% of children who develop SJS. A rash of round lesions about an inch across arises on the face, trunk, arms and legs, and soles of the feet, but usually not the scalp.[8]
Physical Examination
Common physical examination findings of SJS include rash or redness that is seen as red or reddish purple macules that enlarge to form bullae, vesicles and may rupture later to leave denuded skin underneath. Nikolsky sign may be positive which shows Sloughing of skin by gentle lateral pressure on erythematous skin. In addition to skin, Mucosal involvement is found mostly in oral mucosa in addition to nasopharynx and ocular involvement-can be seen as edema, erythema, blistering, sloughing, necrosis and ulceration. In additon to these changes: fever, hypotension and tachycardia can be seen in patients with SJS
Laboratory Findings
SJS is mainly diagnosed through the clinical skin findings. However, a skin biopsy is helpful in making the definitive diagnosis. Other tests are done to either rule out the superimposed bacterial infection, measure disease severity or detect the causative agent or organism. Tests include CBC, BMP, Blood cultures, ESR, CRP, IL-6, ELISA for virus detection among others.
References
- ↑ Template:WhoNamedIt
- ↑ [http://dictionary.reference.com/browse/Stevens-Johnson%20syndrome Stevens-Johnson syndrome - Definitions from Dictionary.com
- ↑ Carrozzo M, Togliatto M, Gandolfo S (1999). "[Erythema multiforme. A heterogeneous pathologic phenotype]". Minerva Stomatol. 48 (5): 217–26. PMID 10434539.
- ↑ Farthing P, Bagan J, Scully C (2005). "Mucosal disease series. Number IV. Erythema multiforme". Oral Dis. 11 (5): 261–7. PMID 16120111.
- ↑ Bastuji-Garin S, Rzany B, Stern RS, Shear NH, Naldi L, Roujeau JC (1993). "Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme". Archives of dermatology. 129 (1): 92–6. doi:10.1001/archderm.129.1.92. PMID 8420497.
- ↑ Ogundele O, Silverberg MA. Erythema Multiforme. eMedicine.com. URL: http://www.emedicine.com/emerg/topic173.htm. Accessed on: May 6, 2007.
- ↑ Auquier-Dunant A, Mockenhaupt M, Naldi L, Correia O, Schröder W, Roujeau JC (2002). "Correlations between clinical patterns and causes of erythema multiforme majus, Stevens-Johnson syndrome, and toxic epidermal necrolysis: results of an international prospective study". Arch Dermatol. 138 (8): 1019–24. PMID 12164739. Unknown parameter
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