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55. Stevens Johnson Syndrome – “Steven Who? And Why I Should Care About His Johnson?”
Keywords
RashSteven-Johnson SyndromeToxic Epidermal NecrolysisErythema MultiformeCase
Sick Patient with a Rash
Pertinent History
A 22-year-old female presents with a diffuse rash including desquamation of mucous membranes and lips. She was recently started on lamotrigine (Lamictal®) at an initial dose of 100 mg, Clonazepam 0.5 mg, and Doxycycline approximately 2 weeks ago. She reports that the rash began 3 days prior to presentation. She was seen at an outside hospital and was diagnosed with hand, foot, and mouth disease and treated with supportive care. Her rash continued to worsen, spreading to her trunk, groin, and mouth. She is unable to tolerate drinking liquids due to severe pain. She describes the rash on her body as pruritic. She has no past medical history of skin disorders.
PMH
Seizures, Anxiety Disorder
Meds
Lamotrigine, Clonazepam, Doxycycline
Pertinent Physical Exam
Blood pressure 130/70, pulse 116, temperature 99.9 °F (37.7 °C), RR 16, SpO2 99%.
Except as noted below, the findings of a complete physical exam are within normal limits.
Mouth: Multiple lesions in mouth, yellow crusting and desquamation of mucous membranes of lips, tongue, and buccal mucosa.
Eyes: Conjunctival injection present bilaterally.
Cardiovascular: Tachycardia present, regular rhythms, no murmurs.
GU: Vaginal mucosal lesions noted.
Skin: Skin is warm and dry. No erythema. Diffuse maculo papular rash with pink papules coalescing into plaques with superimposed vesicles and bullae.
ED Management
The most likely diagnoses considered at the time of presentation were Erythema Multiforme Major and Stevens-Johnson Syndrome. Dermatology was consulted and evaluated the patient at the bedside. Based on the patient’s clinical history and physical exam they felt the patient was more likely exhibiting Erythema Multiforme Major. The patient was admitted for further management and workup.
Learning Points
Priming Questions
- 1.
What is the difference between Erythema Multiforme, Stevens-Johnson Syndrome, and Toxic Epidermal Necrolysis?
- 2.
What are specific causes and risk factors of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis that I can look for in a patient’s history?
- 3.
What is the treatment and disposition of Stevens-Johnson/Toxic Epidermal Necrolysis?
Introduction/Background
- 1.
Erythema Multiforme (EM) versus Stevens-Johnson Syndrome (SJS) versus Toxic Epidermal Necrolysis (TEN) is a confusing set of definitions. The definition has undergone revision over the years such that the final consensus definition divides the entities into two spectra [1].
Erythema Multiforme
Erythema Multiforme Minor: Typically presents with target lesions, however early on initial lesions may be raised erythematous papules. Typical target lesions contain three concentric zones. Lesions are most commonly on extensor surfaces of acral extremities (finger, hands, toes, feet) (Image 1) and spread centripetal [2, 3].
Erythema Multiforme Major: Combines the findings of EM minor with the involvement of one or more mucosal membranes. In one study, 63% of patients with EM had mucosal involvement [4]. Also by definition EM cannot have the detachment of skin greater than 10% of total body surface area (TBSA) [5].
Erythema Multiforme Minor of the Hand
James Heilman, MD (https://commons.wikimedia.org/wiki/File: Erythema_multiforme_minor_of_the_hand.jpg), “Erythema multiforme minor of the hand”, https://creativecommons.org/licenses/by-sa/3.0/legalcode
SJS/TEN.
SJS/TEN are considered to be a single entity, just along different ends of the severity spectrum: When <10% of TBSA is involved, the disease is labeled as SJS. If >30% of TBSA is involved, it is called TEN. Between 10 and 30%, there is overlap between the two labels [6, 7].
Lesions predominate on the trunk and face and spread symmetrically to other areas of the body and the extremities (Image 2). This spectrum typically presents with diffuse erythema or purpuric macules and blistering. Ninety percent of the time one or more mucous membrane is affected with erosions (Image 3) [8, 9]. As the disease progresses, bullae form and coalesce, forming flaccid blisters with full thickness epidermal necrosis.
Stevens-Johnson Syndrome
Dr. Thomas Habif (https://commons.wikimedia .org/wiki/File:Stevens-johnson syndrome.jpg), “Stevens-johnson-syndrome”, https://creativecommons.org/licenses/by-sa/3.0/legalcode
Stevens-Johnson Syndrome
James Heilman, MD (https://commons.wikimedia. org/wiki/File: Mucosal_desquamation_in_a_person_with_Stevens–Johnson_ syndrome.jpg), “Mucosal desquamation in a person with Stevens–Johnson syndrome”, https://creativecommons.org/licenses/by-sa/3.0/legalcode
- 2.
Ninety percent of cases of EM are thought to be triggered by infection with Herpes Simplex Virus (HSV) being the most commonly identified pathogen [2, 10]. Certain classes of medication, particularly barbiturates, hydantoins, nonsteroidal anti-inflammatory drugs, penicillin’s, phenothiazines, and sulfonamides, can also trigger EM [11].
- 3.
The incidence of SJS/TEN is roughly 4–6 cases per million person/year [12, 13]. Mortality is approximately 5% for Stevens-Johnson syndrome, and anywhere from 30–50% for toxic epidermal necrolysis [7]. Approximately 50% of cases of SJS and 80–90% of cases of TEN are drug induced [14]. Mycoplasma pneumonia is likely the second most common cause, particularly in children, though this is somewhat controversial as it also can be the cause of EM [15].
Physiology/Pathophysiology
- 1.
The pathogenesis of SJS/TEN is not completely understood. It appears that through an unknown mechanism, cytotoxic T cells and natural killer cells begin to attack keratinocytes and cause massive apoptosis [16].
- 2.
By and large, when suspecting possible SJS/TEN in patients and hunting for causes, it is all about the drug history. Certain drugs are more likely to cause SJS/TEN.
Short-Term Drug Use vs Long-Term Drug Use.
Lamotrigine (Lamictal®) and SJS/TEN.
The patient in this case had been started on Lamotrigine around 2 weeks prior to her presentation to the ED. Seven to ten percent of patients started on Lamotrigine will develop a rash, but only 3 in 1000 will require hospitalization [19]. Rash and other skin conditions are more common in children, so this medication is often reserved for adults [20].
Appropriate dosing and dosing adjustment can prevent or decrease the risk of lamotrigine-associated rashes. In adults, the recommended initial dose of lamotrigine alone (in the absence of cytochrome P-450 inhibitors or enhancers) is 25 mg po daily for the first 2 weeks, 50 mg daily during weeks 3 and 4, and then weekly increases of 50–100 mg per day as clinically indicated. The patient in this case was started at 100 mg from day 1.
Drug | Relative risk |
|---|---|
TMP/sulfa and other sulfonamide abx | 172 |
Carbamazepine | 90 |
Oxicam-NSAIDS | 72 |
Corticosteroids | 54 |
Phenytoin | 53 |
Allopurinol | 52 |
Phenobarbital | 45 |
Valproic acid | 25 |
Nevirapine | 22 |
Cephalosporins | 14 |
Pantoprazole | 18 |
Tramadol | 20 |
Lamotrigine | 14 |
Sertraline | 11 |
Quinolones | 10 |
Aminopenicillins | 6.7 |
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