emDocs.net Script:
Brit: Welcome back to the emDocs.net podcast. I’m Brit Long, and I’m joined by Manny Singh. You can access the podcast from our homepage on emDocs.net and subscribe in iTunes. We have included a summary of all of these points with respective links for further reading. Manny, what are we looking at today?
Manny: We have 3 great posts: SJS and TEN, the 5 minute rapid neuro hand exam, and penile injuries.
This first one focuses on SJS and TEN, as well as several other dangerous skin conditions that can trip up emergency physicians…..
SJS is on a spectrum of allergic reaction when 10% of the body develops blistering rash that begins with prodrome of non-specific flu like symptoms. The rash starts on the face and torso and spreads to rest of body sparing soles and palm. The hallmark mucosal involvement is what we tend to look for, especially conjunctiva involvement. Usually the trigger is some medication, so med reconcillation is key in patients that are taking many meds, especially active cancer patients. Treatment is basically supportive...stop the offending agent, local wound care, pain control and IV fluids. 2 ml/kg x % epididermal detachment. These patients can become really sick and tools such as the SCORTEN tool can help you in prognosticating patieints who may benefit from a transfer to ICU or burn center.
So let’s talk about the mimics...
The first one is Acute Generalized Exanthematous Pustulosis (AGEP), an uncommon yet severe skin reaction that is often confused with SJS/TEN. It is a drug reaction presenting with non-follicular, sterile pustules on an erythematous and edematous base. These are commonly seen on flexural surfaces and is often seen first in intertriginous areas that spread to the trunk. It usually develops quickly and presents within 24 to 48 hours of starting a medication, unlike SJS which can start days to weeks after being on a medication. Oral mucosal involvement is seen in 25%, as opposed to involving the majority of those with SJS Conveniently, the management is very similar to SJS/TEN — primarily consisting of discontinuing the offending agent and supportive care which usually leads to resolution of the rash within 2 weeks. Topical steroids are useful, but again systemic steroids having unclear benefit.
Brit: The next one is erythema multiforme (EM), which is an immune-mediated condition that presents with the classic target-toid lesions, which can sometimes have mucosal involvement. The target lesions are classic but can have varied presentation as it evolves over the course of the disease. A significant distinction is that the lesions of EM tend to be papular as opposed to the atypical target lesions of SJS which tend to be macular in nature. When these lesions affect mucosal areas, it is typically called erythema multiforme major. When there is little to no mucosal involvement it can be described as erythema multiforme minor. The lesions typically develop over 3 to 5 days and resolve within 2 weeks. There are a variety of risk factors that contribute to the development of EM including but not limited to infections, medication, malignancy, and autoimmune disease. However, infections account for approximately 90% of cases with HSV being most common in adults and mycoplasma in children.
Manny: Our next is Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS). As the name hints, is another severe drug induced reaction that may be difficult to differentiate. This syndrome presents with fever, eosinophilia or elevation in atypical lymphocytes, lymphadenopathy, facial edema, and generalized malaise. This rash is usually delayed 2 to 8 weeks after drug exposure and may continue or even worsen after withdrawal of the causative agent. We are most concerned about organ involvement in these patients, often the liver, lungs or the kidneys. DRESS usually presents with a morbilliform rash starting from the trunk and upper extremities. The rash becomes edematous causing periorbital edema in the face. It can take many forms and presents with pustules, folliculitis, or scaling or even a rare form with mucosal involvement. Therefore, it can be very difficult to diagnose and differentiate from other rashes, and requires a high index of suspicion.
Brit: Next is, PV. Pemphigus vulgaris is a chronic autoimmune disease that is characterized by acantholysis, where the desmosomes that hold intercellular connections are destroyed. Thus the epidermis loses the keratinocyte to keratinocyte adhesion resulting in painful blisters. These blisters are Nikolsky sign positive. Important distinctions of this rash from others we have discussed is that pruritis is typically absent and there is often mucosal involvement, primarily of the buccal mucosa.
Manny: Up next is meningococcemia, specifically from N. meningitidis. Unlike many of the rashes we have discussed thus far, the meningococcemia rash is a symptom of a much larger disease process rather than the disease itself. One of the first symptoms of meningitis to present is a classical rash that can rapidly progress from nonspecific to petechial to hemorrhagic in a matter of hours. The rash begins as 1-2 mm non-blanching petechiae on the trunk and lower extremities. These petechiae can then coalesce into larger purpura. Purpura fulminans is a severe complication characterized by the acute onset of cutaneous hemorrhage, DIC and vascular thrombosis. Bullae and vesicles form that can eventually lead to gangrenous necrosis.
Brit: Next is Staphylococcal Scalded Skin Syndrome, a potentially deadly skin condition caused by a bacterial toxin from Staphylococcus aureus which undergoes hematogenous spread from the skin. It presents with erythematous painful skin that starts primarily in high friction areas such as skin folds. Flaccid bullae, desquamation, and a positive Nikolsky sign characterize the rash. Importantly, unlike SJS/TEN, there is no mucosal involvement and the rash is more superficial. This rash most often affects infants and children and may present early with irritability and poor oral intake.
Manny: Lastly, Erythroderma, also called exfoliative dermatitis, which is a rare rash identified by widespread scaling that covers most of the skin surfaces. It is most often seen in the elderly male population. Many causes can incite the rash, including underlying skin conditions, drug reactions, HIV and cutaneous T cell lymphoma (22). The skin is red, warm, pruritic and painful. The patient is often shivering due to heat loss from cutaneous vasodilation. There may be other exam findings that correlate with the underlying cause such as nail changes with psoriasis; OR lymphadenopathy and splenomegaly may be seen in T cell lymphomas. The rash has a quick onset if caused by medications, while it can take longer to develop when due to other etiologies.
Brit: My favorite part of the post is the summary at the end. The first major point is to take a thorough history. Identifying any new medication exposures over the past several months is valuable information, as well as how the rash has changed, the presence of systemic symptoms like fever, and travel. The second major point is the physical exam, including evaluating for mucosal involvement and the presence of a Nikolsky sign, which is skin sloughing with lateral pressure. Keep in mind that SJS/TEN, pemphigus vulgaris and SSSS are Nikolsky positive.
Regarding treatment
SJS/TEN: supportive care, treat like any other burn with burn center transferAGEP: supportive care, +/- topical steroidsEM: supportive care, consider IV or PO steroids if majorDRESS: topical steroids, systemic steroids if solid organ involvementPemphigus vulgaris: systemic glucocorticoids + nonsteroidal systemic immunomodulatory medicationsMeningococcemia: antibioticsSSSS: antibioticsErythroderma: supportive care, topical steroidsOur next post released by Anna Pickens of EM in 5 fame focuses on the rapid hand neuro exam. The great thing about this video is it provides you with tools you will probably use on every shift. For sensation, the median nerve can be tested at the tip of the index finger, while the radial nerve can be tested on the dorsal aspect of the hand, between the thumb and index finger. Sensation for the ulnar nerve can be tested along the tip of the pinky. For the motor exam, test the median nerve by having the patient make the ok sign, and try to pull their thumb and index finger apart. For the ulnar nerve, have the spread their fingers apart and have them resist you as you “squish” them together. Finally, for the radial nerve, have them make a fist and push down on their fist while they resist and pull upward.
Manny: Our final post for today comes from the Unlocking Common ED Procedures, where the authors look at penile zipper entrapment and injuries. While rare, these can be devastating...a lot of what’s out there has been shared through cases series rather than studies.
Once a penile entrapment is confirmed, the post suggests a stepwise approach to the management of such injuries. The steps include:
1) Identifying the nature of the injury
2) Pain management and analgesia
3) Approach to penile liberation
Two main types of injuries have been identified. These include penile tissue getting caught in the sliding mechanism itself as well as penile tissue getting caught between the zipper teeth. It is important to identify the exact mechanism as they have different approaches to liberating the patient from the entrapment. If the mechanism is not easily identifiable, better exposure should be obtained. In this case, it would mean cutting the clothing off around the zipper, leaving only the zipper and immediately surrounding fabric still attached to the patient. This will allow for different lines of sight to better identify the type and extent of injury.
Once the injury is identified and a plan for intervention has been made, attention should be turned to pain relief as well as anxiolysis if needed. Particularly in the patient population which this occurs in primarily, this may need to be addressed before you can better expose the injury to identify the mechanism. There are a variety of different methods we can use in the ED for pain control. Ultimately, more than 1 method may need to be used.
Pain control should be initiated utilizing minimally invasive measures before proceeding to sedative hypnotics or opioids. If your ED has a child life specialist, involve them in the case early as they are experts at keeping children calm and cooperative in the ED-particularly if they are about to undergo a procedure. The patient may not even let you examine them due to distress or pain. Start with IN and topical EMLA. Pain control can also be achieved through a dorsal penile nerve block. Your patient may still be in distress, even after a nerve block. This may be from inadequate analgesia or from the trauma of the injury/experience. At this point, the patient may require procedural sedation in the ED and/or consideration to go to the OR with urology for further management.
Lastly, the penile liberation...I am gonna leave this definitive treatment to you to read in further detail as it involves MacGuyver esquise maneuvers and visualization of the images in this post.
Brit: This rounds out our summary of the key emDOCs posts. Thanks for joining us, and stay tuned for our next episode. Feel free to comment on our site and let us know if you have any feedback. Stay safe and healthy everyone!