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The family physician diagnosed Stevens-Johnson syndrome (SJS) showing mucosal involvement of the eyes and mouth.
Drugs most commonly known to cause SJS and TEN are sulfonamide antibiotics, allopurinol, nonsteroidal anti-inflammatory agents, amine antiepileptic drugs (phenytoin and carbamazepine), and lamotrigine. Mycoplasma pneumoniae has been identified as the most common infectious cause of SJS. Other agents or triggers include radiation therapy, sunlight, pregnancy, connective tissue disease, and menstruation.
SJS is diagnosed when <10% of the body surface area is involved, SJS/TEN when 10%-30% is involved, and TEN when >30% is involved. The pathogenesis of SJS and TEN remains unknown, however recent studies have shown that it may be due to a host-specific, cell-mediated immune response to an antigenic stimulus that activates cytotoxic T-cells and results in damage to keratinocytes.
In both SJS and TEN, patients may have blisters that develop on dusky or purpuric macules. The epidermal detachment (skin peeling) seen in SJS and TEN appears to result from epidermal necrosis in the absence of substantial dermal inflammation. Lesions may progress to form areas of central necrosis, bullae, and areas of denudation.
Fever of >39° C is often present, as was seen with this boy. In addition to skin involvement, there is involvement of at least 2 mucosal surfaces such as the eyes, oral cavity, upper airway, esophagus, gastrointestinal tract, or the anogenital mucosa.
Large areas of epidermal detachment occur. Severe pain can occur from mucosal ulcerations, but skin tenderness is minimal. Skin erosions lead to increased insensible blood and fluid losses, as well as an increased risk of bacterial superinfection and sepsis.
Early diagnosis is imperative so that triggering agents can be discontinued and supportive therapy can be started. Treatment is mainly supportive and if there is a large amount of desquamation, the patient may require intensive care or placement in a burn unit.
This boy was immediately hospitalized and given IV fluids. The penicillin was stopped and an infectious disease consult was called. His oral lesions were managed with mouthwashes and glycerin swabs. His skin lesions were cleansed with saline and he was examined daily for secondary infections. An ophthalmologist was consulted due to the high risk of ocular sequelae.
Photo courtesy of Dan Stulberg, MD. This case was adapted from: Milana, C. Smith M. Hypersensitivity syndromes. In: Usatine R, Smith M, Mayeaux EJ, et al., eds. The Color Atlas of Family Medicine. New York, NY: McGraw-Hill; 2009:750-755.
To learn more about The Color Atlas of Family Medicine, see:
* http://www.amazon.com/Color-Atlas-Family-Medicine/dp/0071474641
The family physician diagnosed Stevens-Johnson syndrome (SJS) showing mucosal involvement of the eyes and mouth.
Drugs most commonly known to cause SJS and TEN are sulfonamide antibiotics, allopurinol, nonsteroidal anti-inflammatory agents, amine antiepileptic drugs (phenytoin and carbamazepine), and lamotrigine. Mycoplasma pneumoniae has been identified as the most common infectious cause of SJS. Other agents or triggers include radiation therapy, sunlight, pregnancy, connective tissue disease, and menstruation.
SJS is diagnosed when <10% of the body surface area is involved, SJS/TEN when 10%-30% is involved, and TEN when >30% is involved. The pathogenesis of SJS and TEN remains unknown, however recent studies have shown that it may be due to a host-specific, cell-mediated immune response to an antigenic stimulus that activates cytotoxic T-cells and results in damage to keratinocytes.
In both SJS and TEN, patients may have blisters that develop on dusky or purpuric macules. The epidermal detachment (skin peeling) seen in SJS and TEN appears to result from epidermal necrosis in the absence of substantial dermal inflammation. Lesions may progress to form areas of central necrosis, bullae, and areas of denudation.
Fever of >39° C is often present, as was seen with this boy. In addition to skin involvement, there is involvement of at least 2 mucosal surfaces such as the eyes, oral cavity, upper airway, esophagus, gastrointestinal tract, or the anogenital mucosa.
Large areas of epidermal detachment occur. Severe pain can occur from mucosal ulcerations, but skin tenderness is minimal. Skin erosions lead to increased insensible blood and fluid losses, as well as an increased risk of bacterial superinfection and sepsis.
Early diagnosis is imperative so that triggering agents can be discontinued and supportive therapy can be started. Treatment is mainly supportive and if there is a large amount of desquamation, the patient may require intensive care or placement in a burn unit.
This boy was immediately hospitalized and given IV fluids. The penicillin was stopped and an infectious disease consult was called. His oral lesions were managed with mouthwashes and glycerin swabs. His skin lesions were cleansed with saline and he was examined daily for secondary infections. An ophthalmologist was consulted due to the high risk of ocular sequelae.
Photo courtesy of Dan Stulberg, MD. This case was adapted from: Milana, C. Smith M. Hypersensitivity syndromes. In: Usatine R, Smith M, Mayeaux EJ, et al., eds. The Color Atlas of Family Medicine. New York, NY: McGraw-Hill; 2009:750-755.
To learn more about The Color Atlas of Family Medicine, see:
* http://www.amazon.com/Color-Atlas-Family-Medicine/dp/0071474641
The family physician diagnosed Stevens-Johnson syndrome (SJS) showing mucosal involvement of the eyes and mouth.
Drugs most commonly known to cause SJS and TEN are sulfonamide antibiotics, allopurinol, nonsteroidal anti-inflammatory agents, amine antiepileptic drugs (phenytoin and carbamazepine), and lamotrigine. Mycoplasma pneumoniae has been identified as the most common infectious cause of SJS. Other agents or triggers include radiation therapy, sunlight, pregnancy, connective tissue disease, and menstruation.
SJS is diagnosed when <10% of the body surface area is involved, SJS/TEN when 10%-30% is involved, and TEN when >30% is involved. The pathogenesis of SJS and TEN remains unknown, however recent studies have shown that it may be due to a host-specific, cell-mediated immune response to an antigenic stimulus that activates cytotoxic T-cells and results in damage to keratinocytes.
In both SJS and TEN, patients may have blisters that develop on dusky or purpuric macules. The epidermal detachment (skin peeling) seen in SJS and TEN appears to result from epidermal necrosis in the absence of substantial dermal inflammation. Lesions may progress to form areas of central necrosis, bullae, and areas of denudation.
Fever of >39° C is often present, as was seen with this boy. In addition to skin involvement, there is involvement of at least 2 mucosal surfaces such as the eyes, oral cavity, upper airway, esophagus, gastrointestinal tract, or the anogenital mucosa.
Large areas of epidermal detachment occur. Severe pain can occur from mucosal ulcerations, but skin tenderness is minimal. Skin erosions lead to increased insensible blood and fluid losses, as well as an increased risk of bacterial superinfection and sepsis.
Early diagnosis is imperative so that triggering agents can be discontinued and supportive therapy can be started. Treatment is mainly supportive and if there is a large amount of desquamation, the patient may require intensive care or placement in a burn unit.
This boy was immediately hospitalized and given IV fluids. The penicillin was stopped and an infectious disease consult was called. His oral lesions were managed with mouthwashes and glycerin swabs. His skin lesions were cleansed with saline and he was examined daily for secondary infections. An ophthalmologist was consulted due to the high risk of ocular sequelae.
Photo courtesy of Dan Stulberg, MD. This case was adapted from: Milana, C. Smith M. Hypersensitivity syndromes. In: Usatine R, Smith M, Mayeaux EJ, et al., eds. The Color Atlas of Family Medicine. New York, NY: McGraw-Hill; 2009:750-755.
To learn more about The Color Atlas of Family Medicine, see:
* http://www.amazon.com/Color-Atlas-Family-Medicine/dp/0071474641