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Information about Stevens Johnson Syndrome

Stevens-Johnson syndrome
Classification & external resources
ICD-10L51.1
ICD-9695.1
DiseasesDB4450
MedlinePlus000851
eMedicineemerg/555  derm/405
MeSHD013262
Stevens-Johnson syndrome (SJS) is a severe and life-threatening condition. It is thought to be a hypersensitivity complex affecting the skin and the mucous membranes.

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;[1][2] many consider erythema multiforme (EM) to be unrelated to SJS and TEN.[3][4]

Some classify SJS as a severe expression of erythema multiforme, and it occasionally referred to as erythema multiforme major.

Epidemiology

SJS is a rare condition, with a reported incidence of around one case per million people per year.

Causes

SJS can be caused by infections (usually following viral infections such as herpes simplex virus, influenza, mumps, cat-scratch fever, histoplasmosis, Epstein-Barr virus, or similar), allergic reactions to drugs, (fluconazole,[5] valdecoxib, penicillins, barbiturates, sulfas, phenytoin, lamotrigine, nevirapine, Ibuprofen, ethosuximide, carbamazepine)[6][7], malignancy (carcinomas and lymphomas), or idiopathic factors (up to 50% of the time). SJS has also been consistently reported as an uncommon side effect of herbal supplements containing ginseng. SJS may also be caused by cocaine usage.[8] [Images"[1]][2]

Treatment

Discontinue all medications, particularly those known to cause SJS reactions. Treatment is initially similar to that of patients with thermal burns, and continued care can only be supportive (e.g. IV fluids) and symptomatic (e.g. analgesic mouth rinse for mouth ulcer); there is no specific drug treatment (2002). Treatment with corticosteroids is controversial since it might aggravate the condition or increase risk of secondary infections. Other agents have been used, including cyclophosphamide and cyclosporine, but none have exhibited much therapeutic success. Intravenous immunoglobulin (IVIG) treatment has shown some promise in reducing the length of the reaction and improving symptoms. Other common supportive measures include the use of topical pain anesthetics and antiseptics, maintaining a warm environment, and intravenous analgesics. An ophthalmologist should be consulted immediately, as SJS frequently causes the formation of scar tissue inside the eyelids leading to corneal vascularization and impaired vision, as well as a host of other ocular problems. Also, an extensive physical therapy program ensues after the patient is discharged from the hospital

The latest treatment for this syndrome is OOKP. Osteo-odonto-keratoprosthesis is a revolutionary treatment which involves the usage of the patients own mucosal cells from the buccal cavity as a culture over the eye and placing a cylinder crafted from the patients canine tooth{ also called as the eye toth } in the place of the pupil. This treatment gives the patient a very good vision and in some cases may completely restore 6/6 vision.

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.[8] Other outcomes include organ damage and blindness.

Eponym

It is named for Albert Stevens and Frank Johnson.[9][10]

People with Stevens-Johnson Syndrome

  • Padma Lakshmi, actress, model, television personality, and cookbook writer[11]
  • Tessa Keller of MTV show Laguna Beach

References

1. ^ Carrozzo M, Togliatto M, Gandolfo S (1999). "[Erythema multiforme. A heterogeneous pathologic phenotype]". Minerva Stomatol 48 (5): 217-26. PMID 10434539. 
2. ^ Farthing P, Bagan J, Scully C (2005). "Mucosal disease series. Number IV. Erythema multiforme". Oral Dis 11 (5): 261-7. PMID 16120111. 
3. ^ 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. PMID 8420497. 
4. ^ Ogundele O, Silverberg MA. Erythema Multiforme. eMedicine.com. URL: http://www.emedicine.com/emerg/topic173.htm. Accessed on: May 6, 2007.
5. ^ Medsafe Data Sheet March 8, 2005. Accessed April 26, 2007.
6. ^ Fagot J, Mockenhaupt M, Bouwes-Bavinck J, Naldi L, Viboud C, Roujeau J (2001). "Nevirapine and the risk of Stevens-Johnson syndrome or toxic epidermal necrolysis". AIDS 15 (14): 1843-8. PMID 11579247. 
7. ^ Devi K, George S, Criton S, Suja V, Sridevi P (2005). "Carbamazepine--the commonest cause of toxic epidermal necrolysis and Stevens-Johnson syndrome: a study of 7 years". Indian J Dermatol Venereol Leprol 71 (5): 325-8. PMID 16394456. 
8. ^ Stevens-Johnson Syndrome - emerg/555 at eMedicine
9. ^ Stevens-Johnson syndrome at Who Named It
10. ^ A. M. Stevens, F. C. Johnson. A new eruptive fever associated with stomatitis and ophthalmia; report of two cases in children. American Journal of Diseases of Children, Chicago, 1922, 24: 526-533.
11. ^ Jess Cartner-Morley, "Beautiful and Damned", The Guardian, 8 April 2006

External links

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Diseases of the skin and subcutaneous tissue (integumentary system) (, )
InfectionsStaphylococcus (Staphylococcal scalded skin syndrome, Impetigo, Boil, Carbuncle) - Cellulitis - Acute lymphadenitis - Pilonidal cyst - Corynebacterium (Erythrasma)
Bullous disordersPemphigus - Pemphigoid (Bullous pemphigoid) - Dermatitis herpetiformis
Dermatitis and eczemaAtopic dermatitis - Seborrhoeic dermatitis (Dandruff, Cradle cap) - Diaper rash - Urushiol-induced contact dermatitis - Contact dermatitis - Erythroderma - Lichen simplex chronicus - Prurigo nodularis - Itch - Pruritus ani - Nummular dermatitis - Dyshidrosis - Pityriasis alba
Papulosquamous disordersPsoriasis (Psoriatic arthritis) - Parapsoriasis (Pityriasis lichenoides et varioliformis acuta, Pityriasis lichenoides chronica) - Pityriasis rosea - Lichen planus - Pityriasis rubra pilaris
Urticaria and erythemaUrticaria (Dermatographic urticaria, Cholinergic urticaria) - Erythema (Erythema multiforme, Stevens-Johnson syndrome, Toxic epidermal necrolysis, Erythema nodosum, Erythema annulare centrifugum, Erythema marginatum)
Radiation-related disordersSunburn - Actinic keratosis - Polymorphous light eruption - Radiodermatitis - Erythema ab igne
Disorders of skin appendagesNail disease - Alopecia areata - Telogen effluvium - Lichen planus - Hypertrichosis (Hirsutism) - Acne vulgaris - Rosacea (Rhinophyma) - Pseudofolliculitis barbae - Hidradenitis suppurativa - Miliaria - Anhidrosis - Body odor
Otherpigmentation (Vitiligo, Melasma, Freckle, Caf au lait spot, Lentigo/Liver spot) - Seborrheic keratosis - Acanthosis nigricans - Callus - Pyoderma gangrenosum - Bedsore - Keloid - Granuloma annulare - Necrobiosis lipoidica - Granuloma faciale - Lupus erythematosus - Morphea - Calcinosis cutis - Sclerodactyly - Ainhum - Livedoid vasculitis
see also (Q80-Q84, )
For other uses of "ICD", see ICD (disambiguation).


The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD
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List of ICD-10 codes. The version for 2007 is available online at [1]

Chapter Blocks Title
I Certain infectious and parasitic diseases
II Neoplasms
III Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
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For other uses of "ICD", see ICD (disambiguation).


The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD
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The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. These codes are in the public domain.

See also


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The Diseases Database is a free website that provides information about the relationships between medical conditions, symptoms, and medications.

It directly integrates the Unified Medical Language System.

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MedlinePlus is a website containing health information from the world's largest medical library, the United States National Library of Medicine. The site is intended to be used by health care providers and patients, and designed to provide up-to-date, authoritative information.
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Condition can refer to:
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  • Living condition, see Quality of life.
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MeSH D006967 Hypersensitivity refers to undesirable (damaging, discomfort-producing and sometimes fatal) reactions produced by the normal immune system. Hypersensitivity reactions require a pre-sensitized (immune) state of the host.
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The mucous membranes (or mucosae; singular: mucosa) are linings of mostly endodermal origin, covered in epithelium, and are involved in absorption and secretion. They line various body cavities that are exposed to the external environment and internal organs.
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Toxic epidermal necrolysis
Classification & external resources

ICD-10 L 51.2
ICD-9 695.1

DiseasesDB 4450

eMedicine emerg/599   med/2291
MeSH D004816 Toxic Epidermal Necrolysis
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Erythema multiforme is a skin condition of unknown etiology, but some authors suggest that the disease is mediated by deposition of immune complex ( mostly IgM ) in the superficial microvasculature of the skin and oral mucous membrane that usually follows an antecedent infection or
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Erythema multiforme is a skin condition of unknown etiology, but some authors suggest that the disease is mediated by deposition of immune complex ( mostly IgM ) in the superficial microvasculature of the skin and oral mucous membrane that usually follows an antecedent infection or
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Herpes simplex virus 1 (HSV-1)
Herpes simplex virus 2 (HSV-2)

This article is about the virus. For information about the disease, see Herpes simplex.

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Influenza
Classification & external resources

TEM of negatively stained influenza virons, magnified approximately 70,000 times
ICD-10 J 10. , J 11.
ICD-9 487

DiseasesDB 6791
MedlinePlus 000080
eMedicine med/1170   ped/3006
MeSH
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Mumps virus

For other uses of the word MUMPS, see .


Mumps or epidemic parotitis is a viral disease of humans.
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MeSH D002372 Cat scratch fever is a usually benign infectious disease caused by the intracellular parasite Bartonella. It is most commonly found in children 1-2 weeks following a cat scratch. It was first described in 1889 by Henri Parinaud.
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MeSH D006660 Histoplasmosis, also known as Darling's disease,[1] is a disease caused by the fungus Histoplasma capsulatum. Its symptoms vary greatly, but the disease primarily affects the lungs.
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Human herpesvirus 4 (HHV-4)

The Epstein-Barr virus (EBV), also called Human herpesvirus 4 (HHV-4), is a virus of the herpes family (which includes Herpes simplex virus and Cytomegalovirus
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Fluconazole (INN) (IPA: [fluˈkɒnəzoʊl]) is a triazole antifungal drug used in the treatment and prevention of superficial and systemic fungal infections.
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Valdecoxib is a prescription drug used in the treatment of osteoarthritis, rheumatoid arthritis, and painful menstruation and menstrual symptoms. It is classified as a nonsteroidal anti-inflammatory drug, or NSAID, and should not be taken by anyone allergic to these types of
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Barbiturates are drugs that act as central nervous system depressants, and by virtue of this they produce a wide spectrum of effects, from mild sedation to anesthesia. Some are also used as anticonvulsants.

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Nevirapine, also marketed under the trade name Viramune® (Boehringer Ingelheim), is a non-nucleoside reverse transcriptase inhibitor (NNRTI) used to treat HIV-1 infection and AIDS.
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Ibuprofen (INN) (IPA: [ˈaɪbjupɹofɛn]) (from the earlier nomenclature iso-butyl-propanoic-phenolic acid) is a non-steroidal anti-inflammatory drug (NSAID) originally marketed as Nurofen
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Ethosuximide is a succinimide anticonvulsant, used mainly in absence seizures. It is sold by Pfizer under the name Zarontin® and was once also sold under the name Emeside
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