Happy Holidays from Bored Review! This week’s post is so exciting it will make you shed…
It’s New Years Day and your first patient of the new year is a 45yo man with no significant prior PMH. However, it’s his third visit to your ED in the last month. On his first visit he was seen in Fast Track for a small cellulitis on the ankle and was discharged with antibiotics. That was clearing up when he was seen a week ago for “the flu,” treated symptomatically and discharged. Today he comes in looking sick, complaining he still has the flu and now has a painful rash, pain when eating, and painful bowel movements. Â
He is tachycardic to 106, otherwise vitals normal and afebrile. He has symmetric target lesions on his hands, chest, and face, erythema and sloughing skin around his mouth.
What does he have, and why does it hurt when he poops??
This patient has Steven Johnson Syndrome! SJS is a derm emergency, defined by:
- Immune reaction most often caused by drugs… especially sulfa drugs (like the TMP-SMXÂ our patient was prescribed for his prior cellulitis), penicillins, or anticonvulsants. Â Also caused by infections, especially URIs and herpes virus.
- Target lesions (red center, central clearing, then red or dark ring) and sloughing skin.
- Prodrome of flu-like symptoms
- Mucus membrane involvement – which is why are patient is having pain with pooping and eating.
- Less than 10% of total body skin involvement, otherwise…
What other disease are in the spectrum of this disease?
- Erythema Multiforme is also characterized by painful target lesions. However there is NO mucus membrane involvement.
- Toxic Epidermal Necrolyisis is on the other end of severity, with mucus membrane involvement and epidermal detachment of > 30% of the total body surface area. These patient are critically ill, prone to infection and hypovolemia, and need resuscitation similar to burn patients.
What is your treatment and dispo?
- Stop the offending agent (antibiotics in this patient’s case) if possible.
- Supportive care
- Admit, and get dermatology involved
- Although this is immune mediated, steroids have NOT been shown to benefit patients.
While examining you patient, you're gently pressing his rash when the skin sloughs off under your finger. What is this called? With what other derm emergencies would you see this?
-Nikolsky sign
-Also seen in Staph Scalded Skin Syndrome and Pemphigus Vulgaris
Fun facts: Who was Steven Johnson?
Actually, he was two people! Albert Mason Stevens and Frank Chambliss Johnson – these gentleman who first jointly published on SJS in the 1920s.
References
Tintinalli’s 7th Ed. Thomas JJ, Peron AD, and Brady WJ. Ch 245: Serious Generalized Skin Disorders
Hippo EM Board Review
Wikipedia!
As always, very special thanks to Dr Willis.
Kylie Birnbaum
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