Redefining Emergency Dermatology

There are some cutaneous syndromes that can be life-threatening if not diagnosed quickly. Outside dermatology, the term “dermatologic emergency” is often regarded as an oxymoron. Unless a patient’s skin is sloughing off — as in Stevens-Johnson syndrome/toxic epidermal necrolysis — a dermatologist is not typically the first physician called when a patient presents in the emergency room. Yet, according to Ronni Wolf, MD, there are such emergencies, which are “best categorized into primary skin diseases and severe systemic disorders with cutaneous manifestations.” Dr. Wolf, who is Associate Clinical Professor of Ophthalmology and Head, Dermatology Unit at the Kaplan Medical Center in Rechovot, Israel, noted in a recent paper, that physicians need to promptly recognize these kinds of diseases. “A single decision can make an important difference between life and death,” he wrote, adding, “The topic of dermatologic emergencies is indeed broad and covers almost all the fields of modern medicine.” Certain rashes should be considered emergencies simply because dermatologists are the specialists who can best identify them, says Lilly Rose Paraskevas, MD, Director of Dermatology, New York Hospital of Queens. “These include bullous arthropod reactions, acute generalized exanthematous pustulosis (AGEP), drug rash with eosinophilia and systemic symptoms (DRESS), pityriasis lichenoides et varioliformis acuta (PLEVA), and acute allergic contact dermatitis, just to name a few.” Indeed, numerous syndromes with cutaneous manifestations that may be life-threatening often need to be treated across numerous specialties. It is the dermatologist, however, who is best able to effectively integrate the clinical information and pathology to determine the correct diagnosis, maintains Joseph Jorizzo, MD, Professor of Dermatology at Wake Forest University School of Medicine in Winston-Salem, NC. However, if a community-based hospital does not have a dermatology department, the responsibility for initial diagnosis often falls to the critical care physician, contends Robert Buka, MD, Section Chief, Department of Dermatology, Mount Sinai School of Medicine in New York. “One of the most important things we do as physicians is make that immediate determination — Is the disease dangerous/non-dangerous, cancerous/non-cancerous? In the case of a dermatologic eruption, the first 30 seconds of diagnosis are crucial.” Because dermatologists “tend to be the only ones who really understand the systemic problems of skin diseases,” partnering with others in the community is essential, Dr. Jorizzo says. In a hospital setting where a drug rash is involved, the “dermatologist is usually consulted within the first week of the appearance of the rash, especially if the patient is not improving and the team wants a second opinion,” Dr. Paraskevas maintains. “There are a few times that the primary medical team is comfortable managing an adverse drug reaction without a dermatologist’s input, in which case we are never part of the team at all.” She adds that in cases of SJS/TEN, both internists and ophthalmologists are as likely to be consulted as dermatologists.

Recognizing Emergencies

People who don’t typically see a dermatologist may not think to call one if a strange rash develops. As Dr. Buka points out, “About 50% of patients with true dermatologic emergencies go straight to an ER.” But since the majority of cutaneous outbreaks are not life-threatening, “ER personnel are oftentimes less equipped to diagnose these acute conditions correctly,” he adds. “Most emergent rashes are caused by medication. Even common exanthematous drug rashes can progress to life-threatening SJS or TEN if the offending medication is not identified and halted,” Dr. Paraskevas states. Another reason to be included in emergency room diagnoses, explains Dr. Jorizzo, is to know what internal assessments need to be done, and what to do to determine the etiology of the outbreak. “There are at least 250 potential diseases that can be seen in an average year by a dermatology practitioner,” he says. “The FDA has approved treatments for about 10 of those. Our medical colleagues can sometimes be at a loss without published data telling them how to deal with an exact situation/presentation.” Dr. Jorizzo further mentions what he calls “the key to getting other specialties on board — confidence in your diagnosis.” “Dermatology makes a very important contribution in terms of excluding entities in a clinical diagnosis,” he explains. “In terms of understanding the internal manifestation, no other specialty is better; dermatologists can more readily assess the internal damage of a particular external presentation.” “For some of the most severe cutaneous syndromes — SJS/TEN, in particular — the ER physician on call must be able to make the diagnosis,” Dr. Paraskevas states. “Every hospital needs a few community dermatologists who can help teach the staff and remind them of the basics for diagnosis,” she says. “One to two lectures a year on the most dangerous rashes, their identification and treatment, can facilitate this. This will at least raise the hospital staff’s index of suspicion so that a dermatologist can be consulted in a timely manner. The lectures can also be used to pass on pertinent information, such as a list of nearby burn units and their requirements for patient transfer.” There are a number of dermatologic emergencies that rarely capture the emergency room doctors’ attention, Dr. Jorizzo says. One such striking disease is acute pustular psoriasis, or even a generalized type with acute symptoms, including fever and serum sickness-like features, he said. “The patient presents with a few plaques to suddenly having their whole body covered with pustules. It is a most impressive presentation, and very toxic when it presents with a fever. Most ER physicians will have a knee-jerk reaction and think they need antibiotics and antifungals to combat the outbreak. Dermatologists, however, would take a quick look and rapidly conclude it is psoriasis,” he states. “We know patients may also have arthritis. And they can be at risk for high output cardiac failure.” Pustular psoriasis has been associated with being prescribed systemic steroids and then suddenly discontinuing their use. “Pustular psoriasis needs acute intervention,” Dr. Jorizzo says. “The supportive care is followed by carefully monitored systemic therapy.” Another example of an extremely serious and potentially life-threatening disease is eczema herpeticum, typically considered one of the most serious complications of atopic dermatitis, Dr. Jorizzo maintains. “Considering how relatively common both herpes simplex and eczema are, it’s fortunate we don’t see this occur more often,” he says. Some primary care physicians are not exposed often to dermatologic emergencies, he adds. For instance, he says it’s not uncommon for him to get a call from a physician asking for help in diagnosing a child with a common problem, such as atopic dermatitis or psoriasis. With similar symptoms — itching, red and patchy rash, tiny blisters — what the pediatrician is looking at is atopic dermatitis. The condition has also been confused with scabies, contact dermatitis, fungal or bacterial infections or seborrheic dermatitis, he says. “Up to 3% of the children diagnosed with eczema herpeticum will have herpes disseminate to the internal organs – brain, liver or lungs,” he further explains. Other acute dermatology diseases include angioedema in association with urticaria, or autoimmune bullous disease. Pemphigus vulgaris, as another example, is an autoimmune disease that had a 2% 2-year survival rate before the introduction of prednisolone, Dr. Jorizzo observes. “Bullous arthropod reactions can be very exuberant, so I get a lot of calls from the ER about this eruption,” Dr. Paraskevas says. “The differential diagnosis can be huge depending on the distribution of the bites.” Noting some of the other acute diseases, she says, “DRESS can be difficult to discern, and often the patient has been suffering for a few months before the diagnosis is made. With my last patient with DRESS, I was consulted for the treatment of his ‘seborrheic dermatitis.’ He was an inpatient admitted with fevers, lymphadenopathy and lab abnormalities. He was scheduled for a lymph node biopsy as well as other studies to evaluate his high liver function tests and eosinophilia. On exam, not only did he have scale on his face, he was also erythrodermic, which was difficult to appreciate because he had type 4 skin. Once I made the diagnosis of DRESS and recommended the proper treatment, he quickly improved and was discharged the next day,” she says. AGEP, on the other hand, is usually mild, but “in some instances the patient can be quite sick and require hospitalization for supportive care and observation,” she adds. “PLEVA looks very alarming, as does acute allergic contact dermatitis, which often presents as a blistering rash, which must be distinguished from infection.”

Getting Involved

Dermatology is “not a specialty that has been structured to have 24-hour-a-day access outside the university settings,” Dr. Jorizzo says. In most cases of a true cutaneous emergency, “the patient needs to be managed jointly, where the dermatologist is the advisor. We’re very good at co-managing patients with lots of input to other specialists.” The impact a dermatologist has on diagnosing these types of diseases “will vary tremendously with the patient setting and the hospital setting,” Dr. Buka says, adding that the relationship the dermatologist has with the local community hospital will dictate the role the dermatologist will play. “If a hospital has a very good relationship with a dermatology department, they’ll call on a moment’s notice if they’re unsure about the diagnosis. But community-based clinics may only have one or two people who spend a couple of hours a month at the hospital. Those dermatologists are not going to be at the forefront during an in-patient consultation.” Where multiple specialties are needed to manage more difficult cases, “reaching out and speaking to the other teams in addition to writing your note in the chart helps facilitate a professional relationship and an open dialogue regarding treatment,” claims Dr. Paraskevas. “It also helps to be available. Being on staff at a hospital that has close proximity to one’s practice is ideal to see inpatient consults expeditiously. My affiliated hospital where I see most of my inpatient consults is on my way home, so usually I can see the patients that same evening. I often leave my number with my note and encourage others on the team to contact me with questions,” she says. According to Dr. Paraskevas, even dermatitis as commonplace as stasis dermatitis can be alarming when it is acute and the erythema and swelling seen in the lower extremities can be confused with cellulitis. “This case truly requires multiple disciplines work together,” she says, and explains that she will consult with vascular for evaluation of the arterial and venous circulation of the lower extremities and for clearance for compression of the legs, as stasis improves with compression; cardiology is involved if the swelling is secondary to congestive heart failure. “A nutritionist,” she says, “is needed to get the patient started on a weight loss program and maybe even a physical therapy and rehabilitation doctor must consult on how to get the person moving again, since often patients with chronic, extreme stasis have a hard time moving.”

Conclusion

“If you’re proud of your involvement with a local hospital, show up in the ER at least once a month and meet the new people who’ve started there,” advises Dr. Buka. “Most ER docs are enormously grateful for the option of knowing a dermatologist they can call on a moment’s notice.” By being available to the ER staff, it’s likely your own practice will grow as well, he adds. Editor’s note: None of the physicians has a financial interest directly related to their comments.