Stevens-Johnson syndrome medical therapy
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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 and Toxic Epidermal Necrolysis should be managed in a burn unit, Specialized dermatology or Intensive Care unit. Patient's fluid status, hemodynamic stability, wound surface area and pain should be assessed and managed immediately upon admission. 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. 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, among with cyclosporin has exhibited some 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.
Medical Therapy
Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis should be managed in a burn unit, specialized dermatology or Intensive Care Unit. Patient's fluid status, hemodynamic stability, wound surface area and pain should be assessed and managed immediately upon admission.
Stopping the Offending agent:
- First step in treatment of SJS is always discontinuation of all medications, particularly those associated with Stevens-Johnson syndrome or TEN
- Earlier withdrawl of offending agent decreases mortality and improves the long term prognosis of the patient[1]
- Drugs with long half-lives have increased risk of death in SJS/TEN independent of timing of drug withdrawl[2]
Supportive Care:
- Fluid and Electrolyte management
- Started with IV Fluids(Saline solutions and macromolecules)
- IV fluids are tapered and discontinued over a period of 1-2 weeks after starting oral intake by NG tube
- Less fluids are needed as compared to the burn patients with same body surface area involved[3]
- Urine output of 50-80 ml per hour should be mentained with 0.5% NaCl supplemented with 20 mEq of KCl[4]
- In case of common electrolyte abnormalities (hyponatremia, hypokalemia, hypophosphatemia) - aggressive replacement therapy is recommended
- Wound Care[5]
- Both conservative (no debridement) and operative (debridement) approaches exist
- Non adhesive wound dressings with gauze materials containing petrolatum, silver nitrate, or hydrogels are used as needed
- Temperature management
- Environmental temperature is raised to 30-32 degree Celsius[6]
- It minimizes the caloric loss through the skin
- Pain Control
- Nutritional Support
- Parenteral nutrition may be needed to replace protein loss and promote healing. Insulin is often needed to regulate hyperglycemia leading to glycosura or hyperosmolarity
- Early oral feeding with nasogastric tube is recommended when tolerated
- Pulmonary Care[7]
- Include aerosols and physical therapy. In case of involvement of trachea and bronchi, intubation and mechanical ventilation is needed
- Ocular care
- Early opthalmology consult
- Eye drops, saline rinses, daily lubrication and eyelid cleaning
- Eye medicines with topical corticosteroids and antibiotics if needed
Infection monitoring and Control:
- Sterile handling
- Use of anti-septic solutions
- Monitoring for signs of infection
- Frequent change and culture of catheters
- Repeated blood, catheter, gastric tube, urinary tube and skin cultures every 48 hours
- Prophylactic antibiotics are not recommended[8] - Indications for antibiotic treatment include increased number of single strain bacteria cultured from skin, hypothermia and hemodynamic instability or other signs of sepsis
Specific Treatment Options:
Corticosteroids
- Role of systemic steroids in treatment of SJS/TEN is controversial and unclear
- Previous studies have shown more harm than good in patients given systemic steroids[9]
- Some recent studies suggest that short-term pulsed dexamethasone therapy, given at an early stage of the disease, may be of benefit in reducing the mortality rate in SJS/TEN without increasing the healing time but the results are not statsitically significant[10]
- In conclusion, systemic steroids are of unproven benefit to date for treatment of SJS/TEN
Intravenous Immunoglobulins (IVIG)
- There has been some data in favor of using IVIG for treatment of SJS/TEN
- Many uncontrolled clinical trials showed reversal of disease progression and favorable outcome in patients with confirmed diagnosis of TEN (in precaution with certain risk factors like renal or cardiac insuffeciency, IgA deffeciency, or risk of thrombo-embolism) upon treatment with IVIG[11]. However no controlled study is found to confirm the data
- Another study showed no improvement in mortality of affected patients at any level of disease severity[12]
Cyclosporin
- Recently, cyclosporin has been under investigation as a potential treatment agent for patients with SJS/TEN
- Some studies have shown positive effect, lower mortality and shorter time to re-epithelization upon use of cyclosporin in patients with TEN[13]
- However, further studies are needed to confirm the drug efficacy in treatment of SJS/TEN
Other Immunomodulating therapies
Multiple immunotherapies have been tried in patients with SJS and TEN and various studies are published but current data shows no clear benefit and further investigation is needed.
These include:
- Plasmapharesis
- Cyclophosphamide
- TNF antagonists
References
- ↑ Gerdts B, Vloemans AF, Kreis RW (2007). "Toxic epidermal necrolysis: 15 years' experience in a Dutch burns centre". J Eur Acad Dermatol Venereol. 21 (6): 781–8. doi:10.1111/j.1468-3083.2006.02082.x. PMID 17567308.
- ↑ Garcia-Doval I, LeCleach L, Bocquet H, Otero XL, Roujeau JC (2000). "Toxic epidermal necrolysis and Stevens-Johnson syndrome: does early withdrawal of causative drugs decrease the risk of death?". Arch Dermatol. 136 (3): 323–7. PMID 10724193.
- ↑ Mayes T, Gottschlich M, Khoury J, Warner P, Kagan R (2008). "Energy requirements of pediatric patients with Stevens-Johnson syndrome and toxic epidermal necrolysis". Nutr Clin Pract. 23 (5): 547–50. doi:10.1177/0884533608323434. PMID 18849560.
- ↑ Harr T, French LE (2010). "Toxic epidermal necrolysis and Stevens-Johnson syndrome". Orphanet J Rare Dis. 5: 39. doi:10.1186/1750-1172-5-39. PMC 3018455. PMID 21162721.
- ↑ Ghislain, P., & Roujeau, J. (2002). Treatment of severe drug reactions: Stevens-Johnson Syndrome, Toxic Epidermal Necrolysis and Hypersensitivity syndrome. Dermatology Online Journal, 8(1). Retrieved from https://escholarship.org/uc/item/97d8t291
- ↑ Letko E, Papaliodis DN, Papaliodis GN, Daoud YJ, Ahmed AR, Foster CS (2005). "Stevens-Johnson syndrome and toxic epidermal necrolysis: a review of the literature". Ann Allergy Asthma Immunol. 94 (4): 419–36, quiz 436-8, 456. doi:10.1016/S1081-1206(10)61112-X. PMID 15875523.
- ↑ Ghislain, P., & Roujeau, J. (2002). Treatment of severe drug reactions: Stevens-Johnson Syndrome, Toxic Epidermal Necrolysis and Hypersensitivity syndrome. Dermatology Online Journal, 8(1). Retrieved from https://escholarship.org/uc/item/97d8t291
- ↑ Schulz JT, Sheridan RL, Ryan CM, MacKool B, Tompkins RG (2000). "A 10-year experience with toxic epidermal necrolysis". J Burn Care Rehabil. 21 (3): 199–204. PMID 10850900.
- ↑ Halebian PH, Corder VJ, Madden MR, Finklestein JL, Shires GT. Improved bum center survival of patients with toxic epidermal necrolysis managed without corticosteroids. Ann Surg 1986;204:503-512.
- ↑ Kardaun SH, Jonkman MF (2007). "Dexamethasone pulse therapy for Stevens-Johnson syndrome/toxic epidermal necrolysis". Acta Derm Venereol. 87 (2): 144–8. doi:10.2340/00015555-0214. PMID 17340021.
- ↑ Viard I, Wehrli P, Bullani R, Schneider P, Holler N, Salomon D; et al. (1998). "Inhibition of toxic epidermal necrolysis by blockade of CD95 with human intravenous immunoglobulin". Science. 282 (5388): 490–3. PMID 9774279.
- ↑ Brown KM, Silver GM, Halerz M, Walaszek P, Sandroni A, Gamelli RL (2004). "Toxic epidermal necrolysis: does immunoglobulin make a difference?". J Burn Care Rehabil. 25 (1): 81–8. doi:10.1097/01.BCR.0000105096.93526.27. PMID 14726744.
- ↑ Arévalo JM, Lorente JA, González-Herrada C, Jiménez-Reyes J (2000). "Treatment of toxic epidermal necrolysis with cyclosporin A." J Trauma. 48 (3): 473–8. PMID 10744287.