So, I'm going to talk to you about the unusual, uncommon, and atypical infections. Bobby just kind of alluded that to a little bit. How many of you have actually treated a neck fasciitis case? Last case we had, I don't know if you were involved in this care, Russ, was one of my anesthesia colleagues, totally healthy, nicked his leg, and presented to urgent care with cellulitis, was treated with oral antibiotics, sent home, came back with worsening cellulitis, treated with oral antibiotics, sent home. And then, somehow, because of his connections to other physicians, was told, no, you need to come in, and so on, and he almost died. I mean, he's back now working, but he almost died from a minor nick on his leg, neck fasciitis that was mistreated in urgent cares twice. So, I'm going to talk to you about unusual, uncommon, and atypical infections. This one should scare you, particularly if you're going out for seafood, oysters, like I did last night. So, and this is Vibrio vulnificans, and this is, it sooner or later is going to appear on the self-assessment test and or in training, but this is Vibrio. It can occur following exposure to warm seawater, marine animals, fish handling, if you go down there in Pike's Place there, and you see the guys throwing the fish, they have gloves on, and certainly consuming raw seafood, or having an open wound in contact with this area. It's not exclusively in patients with immunocompromised status, but it is typically seen in patients who are immunocompromised. It is a fairly acute onset, and you have to be able to recognize it. It starts with this rapidly progressive cellulitis, and it can then move on to significant soft tissue necrosis, and as Bobby mentioned, you can get a necrotizing fasciitis, and it exists in our literature from a minor Vibrio superficial cellulitis that then progressed on to life-threatening necrotizing fasciitis. The majority of the patients, again, have very acute onset. They appear ill, but not febrile, and so they can start out looking like a cellulitis. You have to ask the right questions. You have to be very curious in thinking about possibilities of did they have exposure to Vibrio. This is a case with Vibrio as a complication from just a minor dermatologic contact, and then went on to full-blown fasciitis involving the hand necrotizing fasciitis. This is Vibrio vulnificans. This is what it can appear now with these hemorrhagic bulla, and again, this can look like a burn area. It can look like a chemical burn or exposure, and so the key is that you ask the right questions. You have to spend the time to ask the right questions in exposure. Treatment is emergent fasciotomy, extensive surgical debridement, amputation has been described, and very high mortality rates in patients with diabetes or hepatic dysfunction, or if they present with septicemia at the time, and then those with upper extremity infections is a 50 and a 15 percent mortality rate with Vibrio vulnificans, and the key again is recognition, asking the right questions, at least thinking about could this be given the right patient clinical scenario. Sporothecosis, I suspect that some of us have treated this. Again, it's often referred to as the alcoholic gardener syndrome because of the rose exposure, the alcoholic gardener being immunocompromised, and it's due to the fungus sporothoraxcheniae which is ubiquitous in soil, garden material, clippings, and so on, and usually the patient ends up with a thorn, a nick, a cut, something very minor, and then sometime thereafter develops this granulomatous process, and they usually have an ulcerative lesion that then progresses along the lymphatic chain, so you'll see sequentially over time, if you ask the right questions when they present, did it start out as one or two and then progress, it usually is along the lymphatic chain ascending approximately up the limb. Carpal tunnel syndrome with flexor extensor tenosynovitis has been described. There are plenty of cases of extensor tenosynovitis, I've experienced with both, I've also written about the case with osteoarticular involvement, and again, that can be seen in patients with AIDS and alcohol abuse. One of the things that Bobby didn't mention about diabetes is I have seen patients present with bad infections and HIV patients who either don't disclose to you their HIV status or risk factors, or they don't know they have diabetes and it's the first presentation of their diabetes, and so please, another take-home message is to think about in the patient with unusual infections or recurrent infections, they've had treatment elsewhere, recalcitrant infections sent to you, think about an HIV test and possibly diabetes, glucose, and consider HIV testing. For spore trochosis, again, we talked about communicating with your lab and communicating with your ID guys, if you can think about it, then you need to communicate that it needs to be grown accordingly on special agar, and Sebron's agar at 25 to 30 degrees, oftentimes has to be specially ordered. So if you put on your order, rule out or please evaluate for spore trochosis or fungal infection, at least the lab then knows to grow out on specific agar. This is what it can look like, relatively nonspecific finding, but again, when you see this and think about this, this is a spore trochoid appearance, this ascending area in the lymphatic drainage, think about spore trochosis. It's not exclusive to spore trochosis, I'll show a case of nocardia, but at least this should raise your suspicion for something unusual and atypical. This is another case, it's undergone debridement already, patient was not sent for fungal cultures, so this came back and then subsequently on repeat debridement was identified as spore trochosis. Treatment for lymphocutaneous disease, at least the last time I checked Russ was oral potassium iodide, and the deep infections, the tenosynovitis, the bone involvement, it has to be debrided surgically, specimen has to be sent, you have to request specifically if you're thinking about spore trochosis, and then absolutely this is where you get the ID guys involved, especially if I can't say or name the antibiotic regimen that they're supposed to be on, that's where I get my colleagues involved, and the treatment can be long, they can be on for multiple months. Aspergillosis, you may encounter a case of aspergillosis or two in your career, we are seeing this in immunocompromised patients, again there are several species, it's abundant in soil and water, and the host again is usually immunocompromised but not exclusively, they can have transplant, hematopoietic malignancies that are first diagnosed, first presentation of a hematopoietic malignancy can be an aspergillosis infection. And again this progresses skin necrosis surrounding erythema and it can involve, it can spread and can involve deep fascia, fascial involvement and muscle and tendon. This can often be mistaken as a malignancy or progressive malignancy, again if you're sending it as a biopsy for possible cancer, think also about atypical infection, fungal infection and you can ask for that and they can do on the staining, they can look for hyphae or even send a second specimen to microbiology as well. Again very innocuous appearance, when I look at that I think of basal cell or squamous cell carcinoma, it should just at least cross your mind, and again we see squamous cell carcinoma in immunocompromised patients, so at least think about these unusual infections. The diagnosis again is the fungal biopsy or smear from the border of the lesion, so taking specimens from multiple different areas is absolutely okay and may raise your incidence of coming to a correct diagnosis, may increase your corrective, may increase the ability to correct, have a correct diagnosis. Second is wide excision, debridement, occasionally patients require amputation, and again an ID involvement for what particular antifungal agents are going to be involved, and it's been said that it's very, very important to excise all of the hyphae to get clean margins, the margins are not only clean but no hyphae are detected in the margins, otherwise you can end up with metastasis to the lungs. Mucormycosis, I've seen case of mucormycosis again in post-transplant patients, it's an aggressive cutaneous fungal infection, again this common theme of immunocompromised patients, burn patients, but it can occur in immunocompetent hosts. There are several species from the order mucorales, so rhizopus and mucor are the two most common ones that have been reported in the hand and upper extremity. In the beginning it can form this black eschar, almost like a spider bite or some unusual bite, and then progresses on again to this necrotizing cellulitis. It's again ubiquitous in soil, in contaminated wounds, if you have patients who present farm agriculture or road rash injuries, where the arm has been out the window, open fractures, breaking of the skin barrier, in this kind of an agricultural setting, then think about these sorts of infections in mucor. This is how it can start out, this is the cutaneous form, and again you're looking at that, and again we'll have this talk, and maybe tomorrow someone gets one, but most of the time we don't, and this is going to show up maybe a year from now, and I have to admit in my mind as well, I'm not thinking about this a year from now, but at least unusual appearance, please just remember, possibility of unusual infection. The prognosis is concerning because the mortality rate can be 33% in cutaneous disease only in the immunocompromised patient. The diagnosis again is routine fungal cultures. They can often be unreliable, and so again the biopsy of the tissue, asking specifically for staining for hyphae or staining for fungal elements, and also culture for fungal elements. Steve Moran wrote a paper about polytrauma patients with open fractures who had deep mucormycosis infections that were very, very difficult to ablate. These patients took an average of 10 debridements, and the range was 4 to 20 in their series out of the Mayo Clinic, and amputation was required in 4 of the 7 cases ultimately to control this unusual infection, and it was open fractures with agricultural exposure. So the key point is a high index of suspicion in necrotic wounds in the immunocompromised patient or high energy, open agricultural wounds with soil contamination, soft tissue trauma loss. Those organisms that are ubiquitous in these environments think about this unusual and rare infection. Marinum, Bobby talked a little bit about. It's often asked on in-training and board questions, research. It's the most common atypical mycobacterial species. My most recent case was actually one of my hand therapists. Her fiancé is an airline pilot. He was docked in Florida. He had something grow on his hand. The physician IND'd his hand, did not send off for fungal cultures. This thing progressed, looked really bad and aggressive. I thought he had a malignancy, and I debrided him aggressively, and it came back as mycobacterium marinum. The interesting thing is that in the area that they had lived or that he spent some time, there was a rash of these infections from the water that was heavily contaminated. It was actually reported in the news, and he had no recollection of any trauma to his hand. Denied it. Denied fish tank. Denied everything. The only clue I had was the fact that he had actually done some fishing in Florida. I'll show you another case. Those that are exposed to that kind of environment, fish tank, aquarium workers are at risk, and it's a result of this direct skin penetration. You can have these painless papules, and it usually occurs two to four weeks after the inoculation period. Many times these patients present months later, and the papules can grow and look like sporotrichosis, so they can actually ascend up the limb and mimic sporotrichosis. So the cutaneous lesions and spread via the lymphatics, just like sporotrichosis. It can be self-limiting, but I've seen a case of ulcerations, and I'll show you a couple. Again, the subcutaneous granulomas may mimic sporotrichosis. Deep infections can certainly occur with tenosynovitis, osteomyelitis, and septic arthritis. This is a presentation of a gentleman that saw me. He spent six months in Florida, six months in Michigan. The only risk factor he had was that he liked to clean his boat, and he presented with this chronic swollen finger, dactylitis. Dactylitis, think about mycobacterium or atypical. And this was his findings at surgery. This thickened tenosynovium, the thickening of the pulleys, and this was after an extensive tenosynovectomy, preserving the A2, A4 specimen sent off and came back as mycobacterium marinum and was subsequently treated accordingly. These are the cutaneous nodules, these cutaneous lesions, again, starting to ascend, mimicking sporotrichosis or the sporotrichoid pattern. This is a patient of mine. It was sent by my ID colleagues while I was at the University of Michigan to see if there was anything I could offer him. He had purely lymphocutaneous involvement, no deep involvement, and was on agents. This was several months in and was having a difficult time eradicating this. This is not something that you can eradicate with major surgery. He required prolonged antifungal regimen and modification of the regimen itself. Similar lesion. Again, could look like sporotrichosis, can look like nocardia, looks like mycobacterium marinum. The diagnosis, another test question, the Lowenstein-Jensen medium at 31 degrees, and again, you have to communicate with your ID guys. You have to communicate with the lab. You can't just put it on there, send the specimen. You have to have some communication and collegiality. For superficial lesions, there's combination chemotherapy. The agents may have changed. Russ, maybe you can comment on that at the end here. And then the deep, deep infections, the tenosynovitis, the bone involvement, joint involvement, require surgery in conjunction with the chemotherapy. Avium is the second most common atypical mycobacterial species. It is the most common opportunistic infection seen in AIDS patients. Again, if you see these patients, you think they're risk factors, they should require an HIV testing. There's variable resistance. It's commonly involved in the deep cases, so if you see deep tissue involvement, think about not only marinum, but also avium. And again, poor prognosis in the immunocompromised patient, but the treatment is the same. surgical debridement or debridements, ID consultation, and appropriate antifungal agents. This is a patient that underwent a surgical procedure, multiple attempts at drainage. You can see the prior incisions. Wound just didn't heal. Wound kept draining. Repeat debridements, nothing sent off for fungal agents, and finally came back as mycobacterium avium. Choloniae, just comment very briefly, again, can present with dactylitis, so the presentation is nonspecific. It's a very rapid grower. It's rare, but it has been reported in the hand. The infection is more difficult to treat, requires multiple surgeries. There are 11 published cases that were managed by a combination of surgery and systemic antibiotics. These patients required multiple surgeries, and they required months of antifungal treatment. They have to be monitored. The regimen has to be changed, and so those are patients that you really need help with in terms of your infectious disease colleagues. Choloniae, and there are susceptibilities, are highly variable, but again, it can vary, and that's why you need to get, in my opinion, the infectious disease folks involved. Tuberculosis can occur following hematogenous spread or direct inoculation. Many of these patients don't have the systemic symptoms you think of, pulmonary symptoms, weight loss, fevers, and chills. Again, chronic flexor extensor tenosynovitis. Think about atypical tuberculosis, merinum, choloniae, and so on, and I've seen a case of dactylitis in a child with TB, and it has been asked on tests and in training before. Again, cultures, communicate with your lab, and then looking for staining in this granulomas with the central caseating area, so on histopathology, if you see that or they report that to you, think about this is something a little bit different, and this might be a TB infection. This is a case with chronic flexor tenosynovitis. Here it is at the time of surgery. You see something like this at the time of surgery. This is not your standard tenosynovitis. It has a very different appearance to it, and again, send specimen off and ask the right questions of your lab. This is at post-debridement. Nocardia, an opportunistic infection. It has a predilection for the immunocompromised host, and again, is ubiquitous in this decomposing vegetation, fresh in saltwater, and again, in sand, and beach sand has been reported, in soil. So again, the common themes are there's some common environments where these organisms reside, and so you need to think about nocardia, particularly if you see post-transplant diabetic immunocompromised patient. There are variable presentations. It can mimic neoplasm. It can mimic spore trichosis. It can mimic merinum, and the mechanism of injury, again, is usually direct inoculation, but it can be very, very trivial and minor, and the patient may not even recall actually having an injury. The presentation can be very similar to a pyogenic infection, lymphocutaneous presentation, as I'll show you. This so-called spore trichoid nocardiosis is fairly common. The most common organism is nocardia brasiliensis, and it may progress to deep infections. So the longer the superficial lesions are present, and you'll see the slide, the longer that's misdiagnosed, mistreated, it allows direct invasion and creation of sinus tracts and soft tissue destruction. It can metastasize. It can metastasize to the brain, as opposed to some of the other organisms that more commonly metastasize to the lung, and this is what it looks like. This is nocardia in the upper extremity. Very nonspecific presentation, in my opinion. Can look like spore trichosis, can look like marinum, can look like a lot of things. You see something like this, think about this lecture and think about something unusual. The diagnosis, Gramstein, you must discuss with microbiology if you're considering it, because it is a very slow grower. Treatment again, ID involvement, surgical debridement, and then the right regimen. Key points, have a high index of suspicion, particularly in the immunocompromised, not exclusively, but think about it. Immunocompromised is a broad term, as we've talked about. It can be the first presentation of a patient that does not know their HIV status, or that they have diabetes, or a malignancy. They've been reported as the first presentation of patients with an occult malignancy. Suspect the unusual or atypical infection in chronic non-healing wounds or monarthritis, or if the patient has chronic or recurrent tenosynovitis. The other one that's frequently missed is following a corticosteroid injection. There are reports in the literature of contaminated steroid bottles that had expired, that had been reused. One bottle was contaminated, and multiple patients developed different sites of infections with mycobacterial species. So your office is supposed to comply with when they open the bottle. It has an expiration date. I would encourage you to get rid of them on the expiration date. Track it carefully. Always clean it. Make no assumptions that the person before you has used it and cleaned it accordingly. And then lymphocutaneous involvement is not always spore trichosis. Good communication is required, collaboration between us, the rheumatologist, as Bobby alluded to, the pathologist with specimens if you're asking for permanent or H&E stains, the infectious disease specialist, and frequently in the transplant patient, you have to have them involved as well. They're also very, very protective of their patients. And so they literally want to be involved in every decision and know what's going on because they're concerned about rejection or possible loss of the transplanted organ. And again, MRI can be helpful in selected cases, but it's the high index of suspicion. And consider these in these unusual cases, unusual presentations with the proper risk factors. Thank you very much.

unusual infections

neck fasciitis

Vibrio vulnificans

sporotrichosis

aspergillosis

mucormycosis