Hospitalists aren't always the most knowledgeable when it comes to their patients' skin changes. “The commonest consult that dermatologists get in the hospital is ‘Rash: Please assess and advise,’” quipped Mark Davis, MD, a professor of dermatology and chair of the clinical division at Mayo Clinic in Rochester, Minn.
He offered clinical pearls for hospitalists on 11 dermatological conditions, ranging from mild to deadly, during his session at Internal Medicine Meeting 2016.
Intertrigo, or intertriginous dermatitis, is a rash that affects intertriginous folds, such as under the breasts or around the inguinal areas. “These are areas where patients sweat, and the area gets wet and sometimes macerated, especially if it's very warm in the hospital,” Dr. Davis said. “And then what likes to thrive in this area is Candida infection, so it's a combination of an irritant contact dermatitis and superimposed Candida infection.”
The best management of this type of rash is to apply a topical antifungal, such as ketoconazole 2%, and a mild corticosteroid cream, such as hydrocortisone 1%, to the affected area twice daily, he said. “It doesn't matter which order [patients] use them in,” Dr. Davis said. “Once the intertrigo is cleared up, I tell them to stop the creams and then to keep this area dry,” often with powders such as talc, so the rash does not return. Angular cheilitis also responds to this treatment, he added. Dr. Davis cautioned, “We don't want the patient to use a very strong topical corticosteroid here, as it's going to be under occlusion in these intertriginous areas and could cause skin atrophy.”
Vascular damage and palpable purpura are symptoms of leukocytoclastic vasculitis, which doesn't always need to be treated, Dr. Davis said. But it's important to understand the cause. “We consider vasculitis to be a clinical sign,” Dr. Davis said. “The commonest underlying causes are drugs and bugs: medications and [viral or bacterial] infectious diseases.” Connective tissue disease and underlying malignancy are other typical causes, and Henoch-Schönlein purpura is a rarer cause that usually occurs after an infectious disease. “About 50% of leukocytoclastic vasculitis is idiopathic, though, even after looking for all these different causes,” Dr. Davis added.
If a vasculitis rash is very mild with only a few areas of palpable purpura and its cause is known, clinicians may be able to simply observe the patient, he said, offering the example of a mild leg rash that occurs with the flu and resolves over time. But severe and progressive cases need to be treated. Criteria for severe cases include vasculitis associated with systemic disease (e.g., hematuria), rapid progression on the skin, or association with ulcerations. Treat these cases with intensive wound care and a relatively low dose of systemic corticosteroids—about 0.5 mg per kilogram of body weight per day, recommended Dr. Davis.
Dr. Davis once saw a college student who had been feeling fine during class earlier in the day but presented at the hospital with myalgia, sore throat, weakness, pyrexia, hypotension, and tachycardia, with rapidly progressive purpura all over her body, a clinical representation of purpura fulminans. “Purpura fulminans can be due to a few things, but the No. 1 thing we have to be worried about is meningitis and Neisseria meningitides, which can lead to this clinical presentation,” Dr. Davis said. “It is so virulent in its onset that this patient will die unless she rapidly receives an antibiotic.”
Purpura fulminans is due to disseminated intravascular coagulation (DIC). “There are many causes of DIC, but in this type of story we have to cover for meningococcemia,” said Dr. Davis, noting that the patient's family thought this diagnosis was impossible because she had been vaccinated for meningococcemia. In fact, this is not impossible, he said, as this patient had serotype B, which most routinely available meningococcal vaccines still do not cover. The FDA has recently approved a couple of vaccines that protect against serotype B in response to outbreaks of meningococcemia serotype B at universities, Dr. Davis noted, but they are expensive.
Intravenous ceftriaxone is the most important and lifesaving treatment to be administered, Dr. Davis said. “This is an emergency, and no more than 30 minutes should elapse before the administration of appropriate antibiotics,” he said. “And we should treat it empirically—don't wait for the results of tests,” although a lumbar puncture should still be performed. “The pearl here is, when you see purpura fulminans, think meningitis or other underlying infection. Pneumococcal infection may also underlie this too,” he said.
One big and not-uncommon pitfall is mistaking this purpura for vasculitis, Dr. Davis warned. “Of course, it can be very difficult to tell what's vasculitis and what's purpura fulminans,” he said. Systemic corticosteroids, while effective for vasculitis, have not been shown to improve survival in patients with purpura fulminans, Dr. Davis noted.
Another serious concern surrounds survivors of purpura fulminans, who may develop symmetrical peripheral gangrene on the acral parts of the body (e.g., hands, feet, back of head). “Why exactly this happens, we're not entirely sure,” Dr. Davis said, but of 12 patients presenting at Mayo with this complication, the majority required multi-limb amputation, and some required amputation of all 4 limbs. The only treatment that may prevent symmetrical peripheral gangrene is management of the underlying purpura fulminans, DIC, and underlying causes of DIC, he said. “Once it has occurred, autoamputation or active amputation of gangrenous areas are the only options.”
A common scenario in the hospital is morbilliform skin eruption, which is usually caused by medications, Dr. Davis said. “Drug eruptions are extremely common, and it's probably the most common reason you ask us as dermatologists to see the patient,” he said. The key to getting these rashes under control is stopping the offending drug, which can be a challenge since hospitalized patients are often on many, Dr. Davis said. “Any drug that was started before that rash began is a possible culprit,” he said. “The 1 thing you can do is [rule out] anything that was started after the skin eruption started.”
Drug eruptions can persist for up to 2 to 6 weeks, Dr. Davis noted. A morbilliform or maculopapular pattern is the most common type of drug eruption, but other examples include baboon syndrome (symmetrical drug-related intertriginous exanthema), erythroderma, fixed drug eruptions, acute generalized exanthematous pustulosis, and toxic epidermal necrolysis. A life-threatening yet rare condition, toxic epidermal necrolysis is best managed by moving the patient to a burn unit for care, Dr. Davis said. Not much convincing data support interventions beyond stopping the offending drug, if possible, and managing patients' symptoms until they resolve, regardless of the specific drug eruption.
Graft-versus-host disease (GVHD)
In many cases of widespread skin eruption in patients who've had bone marrow transplants, drug rashes and GVHD are indistinguishable both clinically and histologically, Dr. Davis said. “So what we really have to do is look for other signs of GVHD, and that would be gastrointestinal involvement, which would usually manifest as diarrhea, or liver involvement, which would manifest as raised liver function tests, and they would point more toward a GVHD problem,” he said. “And then, if that's the case, we would increase their immunosuppression.”
Dr. Davis said physicians shouldn't depend on a skin biopsy to distinguish GVHD from drug reaction. “It's more helpful to check whether there's signs of GVHD in other organs....If you're unable to distinguish which [condition] it is, it's best to manage the patients as GVHD by increasing the immunosuppression, since GVHD is more likely to be life-threatening.”
Patients with lipodermatosclerosis, a long-term consequence of venous stasis that causes bound-down sclerotic skin in the lower extremities, present with “piano legs” that taper at the ankle, Dr. Davis said. This condition is strongly associated with being female and middle-aged and having a high body mass index and venous abnormalities, he noted.
Lipodermatosclerosis is underdiagnosed and often misdiagnosed as cellulitis—which is not helped by its former name, indurated cellulitis, he said. “Putting [a patient] in the hospital for intravenous antibiotics is going to help—not because of the intravenous antibiotics, but because she has her legs up in the hospital.... In general, with very few exceptions, cellulitis is not bilateral, and compression is what's needed here,” Dr. Davis said, suggesting knee-high support stockings with 30 to 40 mm Hg at the ankle.
Dr. Davis reviewed the 4 different stages of pressure ulcers, as established by the National Pressure Ulcer Advisory Panel: erythema, superficial skin ulcer, skin ulcer with visible fat at the base, and skin ulcer with visible bone, tendon, and/or muscle at the base. “And it's unstageable if the base of the ulcer is covered by a slough or eschar,” Dr. Davis said. “You need to debride to stage this kind of ulcer.”
Clinicians taking care of these patients must ensure that urinary and fecal incontinence are well controlled and that pressure is addressed, he said. “I think it's really important to make sure those patients are turned on their side every couple of hours, ideally, and I think everything else is inferior to that,” Dr. Davis said. “Otherwise, the principles of wound care apply: As for any ulcer, debride the necrotic tissue, and keep it clean and moist.”
“There are many weird causes of ulcerations,” Dr. Davis said. “Weirdest of all of them, I think, is pyoderma gangrenosum.” In pyoderma gangrenosum, patients with underlying conditions, such as inflammatory bowel disease, develop painful ulcerations that begin as tiny pustules and suddenly enlarge, with a characteristic purple border that can be overhanging, Dr. Davis said. Clinical clues of this condition can include an irregular, violaceous undermined border and pathergy, such as occurring at an incision site post-surgery, he said. “We always think of pyoderma gangrenosum as occurring on the legs, and we don't think of it as occurring elsewhere on the body, but...it can affect the breasts, which has a characteristic clinical appearance, and it can be underdiagnosed,” Dr. Davis said. Oral prednisone is usually very effective in healing ulcers due to pyoderma gangrenosum.
Pyoderma gangrenosum can also be overdiagnosed, as in the dramatic case of a patient with extensive ulcerations on her legs who was prescribed multiple immunosuppressive medications in high doses for a presumed diagnosis of pyoderma gangrenosum, Dr. Davis said. Her condition was not improving, and the ulcer did not have characteristic clinical clues, such as overhanging purple edges. “The patient was actually scheduled for amputation the next day, and we said ‘No, cancel the amputation,’” Dr. Davis said.
A skin biopsy and culture showed the patient actually had sporotrichosis, an infection that manifests as ulcerations overlying the lymphatic drainage areas of the legs (so-called sporotrichoid spread). The immunosuppression was allowing the sporotrichosis to go wild, Dr. Davis said. “We went ahead and stopped her immunosuppression completely, cleaned up the ulcerations, and grafted her, and she did beautifully. She walked out of the clinic,” he said. Do not accept the diagnosis of pyoderma gangrenosum if the ulcerations are not responding to high-dose immunosuppressants, and always do due diligence in terms of ruling out other causes of leg ulcerations, Dr. Davis advised.
Patients with atopic dermatitis are itching to know how to control their flares and avoid future flares, but management of this type of rash is controversial, Dr. Davis said. Although patients with atopic dermatitis may inquire about nutritional interventions, he advises that they not go on an elimination diet or undergo allergy testing, as there is very little evidence that food is relevant to flares. The 1 exception would be patients with immediate hypersensitivity reactions, who should avoid any offending foods, he noted.
Low-potency (hydrocortisone cream 1%) or mid-potency (triamcinolone cream 0.1%) topical corticosteroids applied twice daily can be more effective, Dr. Davis said. These will generally not lead to long-term thinning of the skin, which can occur with high-potency creams and overuse. Wet dressings can also be very helpful in reducing the itching and crusting associated with this rash. To soothe inflammation, patients can twice daily apply topical corticosteroids and wet dressings (damp pajamas, preferably cotton, put on over the creams for 30 to 60 minutes), he recommended.
“I'm sure you've all seen it,” Dr. Davis said of calciphylaxis, which causes painful ulcerations on the legs, abdomen, and hands and carries a dismal prognosis. The underrecognized condition occurs in about 4% of patients on hemodialysis and less commonly in patients without renal failure who have obesity or liver disease, he noted. “When you come into the room, you see the patient is in agony with these ulcerations and won't even let you touch these areas. But if you are allowed to touch them, the entire affected area is rock-hard to touch,” Dr. Davis said. The ulcerations most often happen in areas of adiposity, such as the breasts and abdomen, and in the lower extremities, he said.
Biopsies show that patients with calciphylaxis have calcium deposition in and around the blood vessels, Dr. Davis said, “but what we're realizing is that the critical tipping point in developing these infarcted ulcerations is actually a clot in the middle of the blood vessels.” Treatment strategies include correcting calcium and phosphate levels, and some clinicians are experimenting with thrombolysis (patients seem to do better but bleed from it) while others are looking into using anticoagulants, but nothing has consistently shown survival benefit, he said.
Out of 100 patients with calciphylaxis at Mayo, 1-year survival was 46%, and 2-year survival was just 20%, Dr. Davis noted. “Given the dire prognosis and the amount of pain these patients are in, pain control and palliative care is obviously very important....As one of my colleagues said, it's one of the worst ways to die,” he said. “Because we really don't have great treatments, these patients may die an agonizing death.”