SKINIPEDIA - Erythema Multiforme Stevens Jonhson Sydrome

Erythema Multiforme (Stevens-Jonhson Syndrome), a Study by Don R Revis Jr, MD (Part IV, Treatment)

Treatment

Medical Care

In severely affected patients, care in a surgical specialty burn unit may provide the greatest likelihood of survival.

• The most important components of the treatment of Stevens-Johnson syndrome (SJS) are rapid recognition and aggressive treatment.
  • Death is frequently a consequence of inappropriate, incorrect, or delayed therapy.
  • Maintain a high index of clinical awareness for this rare but potentially life-threatening disorder.
• No specific treatment exists for this disease, and survival and recovery ultimately depend on aggressive supportive care and removal of the offending agent.
  • Symptomatic supportive care is administered to keep the patient alive through the initial phase of the disease and the subsequent healing process.
  • Transfer the patient to an intensive care burn unit under isolation precautions to decrease the risk of nosocomial infection.
  • The altered immunologic function of patients with SJS makes the likelihood of developing sepsis much greater. Sepsis is the leading cause of death in SJS.
• Immediately withdraw all potentially causative drugs.
  • This includes all medications begun during the preceding 2 months.
  • Discontinue all unnecessary medications.
• The healing process usually takes about 2 weeks, during which time proper skin care is essential.
  • Practice aseptic handling and avoid adhesive materials.
  • Use topical agents such as 0.5% silver nitrate solution or 0.05% chlorhexidine solution to cleanse the skin. Warm these solutions before application.
  • Avoid silver sulfadiazine because of its causative association.
• Fluid and electrolytes may be lost through the disrupted skin barrier, from profuse diarrhea, or from increased core body temperature. The patient's ability to increase fluid intake may be compromised secondary to oral eruptions.
  • Fluid and electrolyte resuscitation are approximately 66-75% of that required for a similarly sized burn wound.
  • Administer warmed fluids through a peripheral intravenous angiocatheter at a site removed from the skin eruptions.
  • Avoid central venous access if at all possible in order to decrease the risk of line infection.
  • Change all catheters, peripheral or central, at regular intervals.
  • Monitor the adequacy of fluid resuscitation with the use of a urinary bladder catheter.
  • Minimum urine output for adults is 0.5 mL/kg/h; for children, it is 1 mL/kg/h.
• Maintain thermoregulation by keeping the environmental temperature at 30-32°C, administering only warmed fluids, and using heating lamps or warming blankets.
• Patients with tracheobronchial involvement may present with hyperventilation and mild hypoxemia. Careful monitoring and aggressive pulmonary support may lead to early detection and treatment of diffuse interstitial pneumonitis and thus prevent the development of acute respiratory distress syndrome (ARDS).
• The use of a pressure support surface, an air or gel mattress, or a specialty bed is recommended to prevent pressure sores.
• Treat patients for HSV or M pneumoniae -related erythema multiforme.
• Provide adequate pain control.
• Administer subcutaneous heparin to prevent the development of deep venous thrombosis.
• Use antacids, proton pump inhibitors, or histamine 2 blockers to prevent stress ulceration.

Surgical Care

After the acute period of illness has passed and the patient has survived, mucous membrane sequelae may require surgical intervention.

Consultations

• Consult a burn or trauma surgeon familiar with caring for critically ill patients with burn wounds who have open wounds.
• An ophthalmologist should examine the patient daily for signs of ocular involvement. If necessary, disruption of synechiae can be accomplished by administration of wetting or antibiotic eyedrops.
• Physical or occupational therapists may be consulted.
• Infectious disease specialists evaluate for intercurrent infections and advise regarding treatment.
• If tracheobronchial involvement is likely, a respiratory therapist may be helpful.
• Psychologists, psychiatrists, or social workers may be helpful.

Diet

• Several issues make nutritional support critical in patients with SJS.
  • Widespread, painful oral erosions may make feeding difficult. In such cases, pass a soft, flexible feeding tube into the stomach or small bowel, and institute appropriate feedings.
  • Profuse diarrhea may result from gastrointestinal involvement, making oral or enteral feeding difficult. Parenteral nutrition may be appropriate.
  • Increased energy expenditure must be recognized and treated appropriately. Nitrogen balance and other nutritional parameters are useful to estimate nutritional needs and to evaluate the efficacy of nutritional therapy.
  • SJS often results in decreased insulin release and increased tissue resistance to insulin. Supplemental insulin may be necessary.

Activity

• Protect affected skin from any pressure or shear forces. Otherwise, early institution of physical and occupational therapies is appropriate.

Medication

No specific medicinal therapy is available for treatment of Stevens-Johnson syndrome (SJS). However, several controversial topics deserve mention.

• Corticosteroid use is of no benefit in these patients. In fact, a correlation of prior corticosteroid exposure and SJS has identified corticosteroid use as an independent risk factor for development of this disease. Furthermore, corticosteroid use impairs the immune system, and sepsis is the usual cause of death.
• Prophylactic antibiotics are not recommended because of the increased likelihood of selecting out resistant strains. However, evidence of infection should lead to prompt culturing and the selection of appropriate antimicrobial therapy based on culture and sensitivity results. Some authors recommend routine alternate-day skin biopsy for culture to distinguish simple skin colonization from true infectious invasion and to guide antimicrobial therapy.

Clinical (History)