Adil writes:
Dear Doctors,
It was wonderful to have a new episode with which to end out the year. I know you would hardly go fishing for compliments (sorry I had to get in on the puns too) but you certainly deserve them. Thank you for a wonderful year of podcasts. I am so glad I kept listening to NPR long enough to hear that segment which referenced your show as I’ve been hooked very since. I wish you the happiest of New Year’s and look forward to a 2019 full of the best kind of ear worms.
Case guesses:
Ivan writes:
Dear TWIP legends!
Let me first tell you how great you are!! I discovered your TWIX series podcasts about a year now, so far still haven’t had time to listen to all of the TWIPs, but enjoyed very much every episode I have. I’m a second year resident of ECVP (European college of veterinary pathologists) in Zagreb at Department of Veterinary Pathology, Veterinary Faculty, University of Zagreb, Croatia. Currently, I’m doing a lot of studying because my board exam is just a bit over one year ahead.
Now regarding the case, which really seems quite straightforward… The unlucky surfer from Long Island has mucocutaneous Leishmaniasis caused by L. braziliensis. Based on the history presented it is probably a smoldering infection (acquired obviously at the beaches of Costa Rica) initially causing a cutaneous lesion described from a years ago that seemed to heal. My guess is, however, that the protozoan was not completely cleared and remained safe somewhere within the macrophages or dendritic cells, and now caused the mucocutaneous form of the disease. It would be interesting to explore if the patient recalls any possible factors that would have led him to immuno-deficient/-compromised states lately, perhaps a described blow to the nose could also be contributing?? But it is more probable that the surfing board hit accident was actually a coincidence, and that the lesions on the nose were already developing at the time of the accident.
I guess it’s also impossible to rule out the possibility that this mucocutaneous manifestation is actually a newly acquired infection (independent of the first cutaneous manifestation) since the individual continually spends significant time in Costa Rica.
The unlucky surfer should not undergo any kind of surgery of his nose before some serious treatment, probably with liposomal amphotericin or any proposed medication in Vet Parasitology 6th ed, I am a vet pathologist so I don’t really talk often about the therapy (especially not in humans)
Even with a scrutinous therapy, there is a question if the Leishmania protozoan will be cleared. Probably, a negative result for Leishmania after the treatment is needed to proceed to any „nose job“ surgery.
As much as this case seems easy, I can’t wait to hear yours and listeners comments on this one.
Thank you very much! Please don’t cease your noble, educational and amusing work on the podcast(s)!
You forged a wonderful scientific podcast trio!
All the best in 2019!!
- Even though many parasites, just like the one in the today’s case, affect both humans and animals, I would really appreciate hearing more about parasites affecting other mammals (eg. Sarcocystis neurona, Neospora caninum, Spirocerca lupi etc.). It would be great if you would bring a veterinary parasitologist guest to the show once in a while .
Ivan from starry sky Zagreb
Iosif writes:
Dear Twip team,
I’m sorry I haven’t been as active; currently in residency at Northwell and it takes up a lot of time.
My guess for this case would be leishmaniasis given the history of cutaneous ulcers and now mucocutaneous involvement.
P.S. I hope to see you around in the Hospital Dr. Griffin.
Sincerely,
Iosif Davidov
Till writes:
Dear Twipsters,
When listening to twip 162 during my daily commute I was overjoyed to find I had actually won a signed copy of PD6; I was positively energized the rest of the day. Since you guys really inspired me to dig deeper into the wonderful world of worms and protozoans, I am looking forward to receive a hard copy of your book. For shipping, please find my mail-address and phone-number below.
As for the case of twip 163, this was an easy one for the regular listener. The man in his 40s from NY who spent a lot of time in Costa Rica is most likely suffering from Mucocutaneous Leishmaniasis (MCL), also known as “espundia.” It occurs only in the Americas (from Mexico to Argentina) with the majority of cases occurring in Brazil. Although there are many Leishmania species, only a few of them are responsible for most of the cases of MCL; namely, L. brasiliensis and L. panamensis. The vectors are sandflies of different genus’, Luztomyia in the Americas, Pehlebotomus in Europe, Africa and Asia. It is typical for MCL to appear months or even many years after an initial episode of cutaneous leishmaniasis, so this time frame would fit our patients history (lesion an hand 20yrs ago). It is also typical for the lesion to first occur in the hyperemic frontal area of the nasal septum and the first signs are often a congested nose and nose-bleeding (epistaxis). If left untreated, the disease could quickly progress to the soft palate, causing severe disfigurement, morbidity and mortality especially in poor or immunocompromised patients.
Diagnosis is ideally confirmed through direct proof of the pathogen in the lesion. Unlike cutaneous Leishmaniasis, however, the pathogen can often not be found through direct microscopy from a tissue sample. In this case, PCR or culture could establish the diagnosis.
Treatment is analogous to the visceral form of leishmaniasis and should be initiated swiftly. In the setting described (NYC) this would most likely be with liposomal Amphotericin B: 20-30mg/kg total dose spread out to at least 5 injections over 21 days. We typically give five doses over 21 days and apply the infusions slowly over 1-3 hours to prevent adverse events like hypotension. In other settings (i.e. India), single-dose infusions of 5mg/kg have been tried with high reported success-rates of >90%.
The patient should definitely refrain from the reconstructive surgery since the physical manipulation is believed to be able to (re-) activate any parasites left. I would ask to see the patient for check-up after 3 and 12 months to make sure the treatment was successful.
Advice for prevention would be to restrain from sleeping out in the open/on the beach and to have raised beds (>1m above the ground) and finely woven bed-nets to prevent the sandflies from stinging.
Lastly, I was a bit unsure about the need to move his surfing location 😉 by which I understood a question of developing immunity. I know that patients develop immunity after self-healed cutaneous leishmaniasis, rendering them immune from at least the one leishmania strain that caused the initial infection. But after mucocutaneous infection that was treated? I would doubt that a lasting immunity could be assumed.
Once again thank you for your inspiring work. All the best,
Till.
Caitlin writes:
Dear TWiPsters,
At long last I write in another guess, after missing the deadlines for the last two episodes, to my shame. In my defense, I was and still am busy moving across the continent.
The unlucky surfer has mucocutaneous leishmaniasis, caused by one of the Leishmania species in the Vianna subgenus. Since he was probably infected in Costa Rica, my money is on either L. braziliensis or L. panamensis – this can be determined by PCR. He can be treated with paromomycin or sodium stibogluconate.
Since leishmaniasis is spread by sandflies, not surfboards, the blow to the nose must have been coincidental – and possibly lucky, as it might have caused the patient to seek treatment sooner than he might have otherwise!
If my name comes up for the book, please spin again! The PDF is more portable, and has the advantage of being Ctrl+F-able.
Incidentally, I won the copy of Red Mother last time I guessed, and I would highly recommend it to all parasite lovers. It’s deliciously creepy. Could we perhaps have Dickson read a few of the poems out loud, in a throwback to very early TWiP?
Ever a fan,
Caitlin (formerly of Waterloo, Canada, and now of Seattle!)
Kevin writes:
Chicago
Winter
SURF N’ PERF
A surfer sans septum. If you’ve lived through the 80s and have had any exposure to its voluptuary excesses, the absence of a septum immediately brings cocaine to mind. Similarly, the stereotypes that dog the surfing community as being thrill seeking hedonists might, in the more traditionally minded, bring up the great pretender, i.e. syphilis. Perhaps, if you’re older, and eponymically inclined, you might be tempted to use the discredited term Wegener’s granulomatosis (see endnote). Finally, because the parasitically minded often enjoy a gross-out, why not consider the lowly maggot dining on someone’s nasal mucosa? In conclusion (preliminarily), since brevity is the soul of TWiP (apologies to Shakespeare), and as I’ve said before, the vita is brevis, I will spill the beans and leave any digressions to the endnotes.
Mucosal leishmaniasis. What is grandly termed American Tegumentary Leishmaniasis has several forms: cutaneous, mucocutaneous and mucosal. The usual culprit is Leishmania brazilienesis, but other members of the Leishmania Viannia species complex can be involved. Mucosal involvement is also described in Old World leishmaniasis though the mucosal lesions are usually contiguous with a cutaneous sore. Our patient is suffering from what is generally regarded as the ‘metastatic’ spread of a primary cutaneous lesion; mucosal disease can occur nearly contemporaneously with the skin lesion or occur many years after a healed or improperly treated primary cutaneous sore. Approximately 3% of cutaneous leishmaniasis cases are complicated by mucosal disease. The highest incidence occurs in Bolivia where as many as 20% of leishmania infections result in mucosal involvement. The affected mucosa, in order of observed frequency is: nose, pharynx, larynx, mouth. Pathogenesis is incompletely understood but generally agreed to be due to a dysregulated immune response to the parasite. In a coincidental collision of TWiV and TWiP topics, mucosal disease may be facilitated by the infection of the leishmania parasite with the dsRNA virus LRV-1. (see TWiV 128, Virologists in the Mist, April 10, 2011.) It has been observed that LRV-1 infected leishmania induce a greater chemokine/cytokine response which presumably augments the tissue destruction seen in mucocutaneous and mucosal leishmania infections.
Diagnosis: This patient should be tested for HIV since this is a risk factor for mucosal leishmaniasis. Strazulla’s review cites a case series of 100 co-infected HIV patients 68% of whom had mucosal involvement. Syphilis testing is warranted due to the well known but now very rare possibility of tertiary gummatous disease with tissue destruction of the nasal mucosa. Regarding definitive diagnosis of mucosal leishmaniasis, biopsy and histopathology is very low yield. tissue culture and NAAT, and immunohistochemistry/isoenzyme profiling have been attempted. PCR is currently regarded as the best diagnostic strategy.
Treatment: Pharmacotherapy of mucosal leishmaniasis is complex. The CDC website and PD6 list many different medications that are used, many of which are quite toxic and must be administered intravenously. In 2014, the FDA approved a 28 day course of oral medication for cutaneous, mucosal, and visceral leishmaniasis: IMPAVIDO (Miltefosine). As PD6 states, this drug is expensive: $16,712 for a 28 day supply according to Drugs.com. In summary, treatment must be individualized and is a rather specialized matter.
Surgical planning / reconstruction: It is reasonable to assume that complete cure of the infection should be undertaken prior to any surgical adventures. My PubMed and general internet search for a systematic review of plastic surgical reconstruction in mucosal leishmaniasis was unfulfilling. Surgery in these chronic patients is clearly a multidisciplinary affair in order to achieve a durable and successful outcome.
Thanks to all you TWiP professores
END NOTES AND REFERENCES:
- LEISHMANIASIS
Mucosal Leishmaniasis due to Leishmania (Viannia) Braziliensis L(V)b IN TR.S BRA.OS, BAHIA-BRAZIL, P. D. Marsden, Revista da Sociedade Brasileira de Medicina Tropical 27(2):93-101, abr-jun, 1994.
A classic paper. Has the same espundia photo that appears in PD6. Marsden is a good candidate for ‘hero’ status in future podcasts.
Palumbo, E., Treatment strategies for mucocutaneous leishmaniasis. J Glob Infect Dis 2010, 2 (2),
147-50.
Mucosal Leishmaniasis: An Underestimated Presentation of a Neglected Disease, Alessio Strazzulla, Biomed Res Int. 2013; 2013: 805108. Published online 2013 Jun 18. doi: 10.1155/2013/805108
Interventions for American Cutaneous and Mucocutaneous Leishmaniasis: A Systematic Review Update, Ludovic Reveiz, PLoS One. 2013; 8(4): e61843, OPEN ACCESS
Current diagnosis and treatment of cutaneous and mucocutaneous leishmaniasis. Goto H1, Lindoso JA.Expert Rev Anti Infect Ther. 2010 Apr;8(4):419-33. doi: 10.1586/eri.10.19.
Mucocutaneous leishmaniasis masquerading as Wegener granulomatosis. Brahn E, et al J Clin Rheumatol. 2010; 16: 125-128.
Leishmania RNA Virus Controls the Severity of Mucocutaneous Leishmaniasis, Annette Ives, Science 11 Feb 2011: Vol. 331, Issue 6018, pp. 775-778
This paper was discussed in TWiV 128.
Mucosal leishmaniasis: epidemiological and clinical aspects, Marcus Miranda Lessa, Rev. Bras. Otorrinolaringol. vol.73 no.6 São Paulo Nov./Dec. 2007
A relatively recent and very thorough review from Brazil.
Progressive Perforation of the Nasal Septum Due to Leishmania major: A Case of Mucosal Leishmaniasis in a Traveler,Nicole Harrison,Am J Trop Med Hyg. 2017 Mar 8; 96(3): 653–655. OPEN ACCESS
Interesting case report with some surprises. Has great illustrations and a supplemental video….a gross-out rhinoscopic view of a destroyed nasal cavity….great reference list.
The origin of espundia. Transactions of the Royal Society of Tropical Medicine and Hygiene, Larson, E. E., & Marsden, P. D. (1987), 81(5), 880.
An etymological idyll on the origins of the word ‘espundia’. The word is derived from the Latin spongia (sponge)–and Spanish esponja. The term was used as early as the 13th century to describe an exuberant cutaneous excresence that afflicted horses.
Lesson of the week Mucocutaneous leishmaniasis: an imported infection among travelers to central and South America, Sukhbir Ahluwalia, BMJ Volume 329 9 October 2004
https://www.cdc.gov/parasites/leishmaniasis/health_professionals/index.html
Deforming Mucocutaneous Leishmaniasis of the Nose, Gian Luca Gatti, MD,The Journal of Craniofacial Surgery Volume 00, Number 00, Month 2017
Impressive photographs but no directives about the need to definitively treat the infection prior to surgery.
- SYPHILIS
Clinical Lecture on Syphilitic Gummata, reprinted from the Edinburgh Medical Journal for October 1874 (translated by Francis Cadell)
Wonderful essay from 1875 by the famous dermatologist M A Fournier (of the eponymous Fournier’s gangrene), director of the Lourcine Hospital in Paris. OPEN ACCESS at: https://wellcomelibrary.org/item/b21480904#?c=0&m=0&s=0&cv=0&z=0.2515%2C0.4463%2C0.7519%2C0.323
Tertiary Nasal Syphilis: Rare But Still a Reality, Bipin Kishore Prasad, Arch Otolaryngol Rhinol 2(1): 013-015. DOI: 10.17352/2455-1759.000014 OPEN ACCESS
Case report.. see figure 2, Look ma, no septum!
- AUTOIMMUNE
Autoimmune-related nasal septum perforation: A case report and systematic review, Lohitha Guntupalli, 2017 Mar; 8(1): OPEN ACCESS
Summary: “Overall, 140 cases of autoimmune-associated NSPs were reported. Granulomatosis with polyangiitis (48%), relapsing polychondritis (26%), and cocaine-induced midline lesions (15%) constituted 89.3% of the reported cases.”
Autoimmune-related nasal septum perforation: A case report and systematic review, Lohitha Guntupalli, Allergy Rhinol (Providence). 2017 Mar; 8(1): e40–e44. OPEN ACCESS
A note on Wegener: Frederich Wegener (1907-1990) German pathologist after whom the disease ‘Wegener’s granulomatosis’ was named. Wegener’s Nazi past and suspected complicity in war crimes in 2000 led to the disease being renamed ‘granulomatosis with polyangiitis’.
- COCAINE
Cocaine induced midline destructive lesions, M. Trimarchi, Rhinology 52: 104-111, 2014
Interesting review with many disturbing photographs. Shows the less glamorous side of ‘nose candy’. I get no kick from cocaine, especially when accompanied by a destructive midline lesion….
- MYIASIS
Nasal myiasis by Oestrus ovis second stage larva in an immunocompetent man: case report and
literature review, H Einer, E Ellegård, The Journal of Laryngology & Otology (2011), 125, 745–746.
No nasal septum destruction described in the four reported cases.
Nasal myiasis with orbital and palatal complications, Shaji Thomas, BMJ Case Rep. 2010; 2010 OPEN ACCESS
Some rather unsettling photographs. The culprits were Chrysomia bezziana. In addition to ivermectin, the following was applied: “A cotton bud impregnated with turpentine was placed in the right nostril for approximately 10 min and 20 maggots were manually removed. The same procedure was repeated for two more days. (!)
Nasal myiasis: Review of 10 years experience, H. Sharma,The Journal of Laryngology and Otology May 1989. Vol. 103. pp. 489-491
Article is a series of 252 cases of what the authors describe as a “demoralizing condition”. Septal perforation was seen in 16% of their patients.”Conservative management by packing the nose with a chloroform and turpentine (1:4) mixture followed by manual removal of the dead maggots is an effective method.”
DIFFERENTIAL DIAGNOSIS OF NASAL SEPTAL PERFORATION:
ETIOLOGY OF NASAL SEPTAL PERFORATIONS. [FROM S. MOCELLA]
TRAUMATIC CAUSES
Previous surgery
Cauterization or embolization for epistaxis
Nasal packing
Nasogastric tube placement
Septal haematoma from blunt trauma
Battery or other foreign body in nose
Chronic nasal cannula use
Turbulent airflow
Inflammatory or infectious causes
Sarcoidosis
Wegener granulomatosis
Systemic lupus erythematous
Tuberculosis
AIDS
Crohn’s disease
Autoimmune diseases
Leishmaniasis
Cryoglobulinaemia
Celiac disease
Invasive fungal sinusitis
Mycobacterium Kansas infection
Neoplastic causes
Carcinoma
T-cell lymphomas
Other causes
Inhaled substances (e.g., cocaine, topical corticosteroids, long-term oxymetazoline or phenylephrine use)
Chromic acid fumes
Renal failure
Use of targeted/biologic therapies in the treatment of malignant and nonmalignant diseases (bevacizumab)
Use of methotrexate or docetaxel in the treatment of malignant disease
David writes:
Dear Hosts,
I hope you all had a wonderful holiday season and a happy New Year. Like this week’s patient, I spent my holiday break in beautiful, sunny Costa Rica – covering a mere 0.03% of the planet’s landmass, but sustaining an incredible 5% of Earth’s biodiversity. I learned Costa Rica is home to more species of butterfly than the whole continent of Africa! While there, I was highly cognizant of the many parasitic species one could acquire if not careful, and took precautions as to not end up as the next patient in one of Dr. Griffin’s case studies.
As for this week’s case guess, I believe the patient has developed a case of cutaneous leishmaniasis caused by protozoans in the genus Leishmania. It is likely he contracted the parasites from sandflies on the beach in Costa Rica. It seems he had a previous exposure more than 20 years ago to something which gave him similar lesions on his hand, so if he has been returning to the same surfing spot over the years, its possible this beach is likely highly contaminated with parasite-ridden flies.
Thank you for the informative and entertaining podcasts,
Sincerely, David P.
Carrie writes: won
Dear TWiP trio,
In a parasitic context, a skin ulcer acquired in a tropical location immediately brings to mind cutaneous leishmaniasis – caused by one of the many Leishmania species, transmitted by sand fly bites – and that other one with ulcers that I never remember. The combination with a lesion in the nose many years later refines this to mucocutaneous leishmaniasis, caused by one of a smaller subset of Leishmania species. The fact that a parasitologist, upon seeing the nasal lesion, recognised it and deduced the existence of the past ulcer, leaves little room for doubt in this diagnosis.
PCR would provide a definitive test, although the diagnosis doesn’t seem to have been in question. However it could also confirm the species of Leishmania, which may have a bearing on the treatment options – although in this case it’s already narrowed down considerably. Whether the patient is at risk of mucosal recurrence, is also not in question.
For the sake of thoroughness, however, I consulted Parasitic Diseases 6th edition and found and ruled out Dracunculus medinensis, or Guinea worm, which can be found in Latin America but normally causes ulcers on the legs and feet, typically leads to multiple blisters and ulcers, and doesn’t as far as I know recur in the nose two decades later.
One could certainly pick up cutaneous leishmaniasis in Costa Rica, but it is a little north of the so-called mucosal belt – Brazil, Bolivia, and Peru where the beasties that cause mucocutaneous infection reside. The typical culprit in this location is L. panamensis, which only causes cutaneous disease. However, I was able to find reports of a study from 1998 – the right approximate timeframe – which isolated L. braziliensis from two out of thirty-four leishmaniasis patients in Costa Rica. More commonly found further south as its name suggests, braziliensis is one of the species responsible for mucocutaneous leishmaniasis, and I would put it forward as the most likely offender in this case.
The atypical infection for the location may possibly explain why the infection has recurred in spite of treatment at the time of infection: since some treatments for leishmaniasis are more or less effective against different species of the parasite, it’s possible that an inappropriate one was given, on the assumption that this was a panamensis infection.
Mucosal leishmaniasis lesions will not normally heal on their own, and left untreated can result in extensive tissue erosion with severe disfigurement and, in the case of oral cavity lesions, has the potential to lead to fatal infections of the lungs. Treatment would be systemic rather than local, and quite an array of different drugs are available with different pros and cons: I will leave selection to the more qualified.
I can’t find any specific recommendations in the literature regarding reconstructive surgery in the treatment of mucosal leishmaniasis, but in spite of a complete lack of medical training I will hazard a common-sense guess. I can’t imagine any reason this chap shouldn’t have his septum reconstructed, but I would think it should wait until after the ongoing infection has been dealt with (and of course, the surgeon should be apprised of the actual nature of the lesion.)
Thank you for another fascinating podcast.
From Carrie, in Newcastle upon Tyne, England.
References:
Zymodeme and Serodeme Characterization of Leishmania Isolates Obtained from Costa Rican Patients, 1998
Alexey writes:
Dear TWIPsters,
I enjoy listening to your podcasts very much, along with the rest of Microbe.tv series.
I am sending you a link to a strange klezmer song, “life as a parasite” that i’ve stumbled upon recently, hope you like it. It is almost an illustration to some of your recent stories –
https://www.youtube.com/watch?v=UM6wrV0VLL4 .
Sincerely,
Alexey M. Chumakov PhD
Sara writes:
Podfessors!
As always it’s a delight to listen to you, I’m sad I’ve not been keeping up with the cases – I’ll try my best in the future (winning a book in time for Christmas wouldn’t be half bad!). I thought I’d jump on the recently discussed topic of heroes/heroines. I love that new interesting heroines have been sent in and will be included in your book and podcast and I’m shamelessly going to plug one of my own efforts to shine a light on women in science here – hoping for TWiP-bump! Me and the scicomm keen ScienceGrrl Glasgow volunteer crew have started a new blog series called ScienceGrrl of the Moment where we feature profiles/interviews of women out there doing some cool science on our blog and Twitter. We hope to inspire others to learn more about science and perhaps even pursue some equally cool careers. They might not be suitable for your hero segment (the first one we featured isn’t even a parasitologist – GASP!) but are for sure worthy hearing from and about none the less. Feel free to share with hosts and audiences on your other TWi-series podcasts!
https://sciencegrrlglasgow.wordpress.com/2018/11/28/sciencegrrl-of-the-moment-margaret-stanley/
Forever your faithful listener,
Sara from (right now) New Zealand
P.S. If this is read on TWiV – I love the weather segment, it contributes to the chill conversational atmosphere and is genuinely interesting! Just skip it if you don’t like it, or just “suffer” through – it’s really not that long. It’s currently 21°C and very sunny here in Auckland (sunscreen absolutely needed), but yikes the weather here is changeable – we have rain rivalling Glasgow some days and last week it hailed! Really spoiled our avocado picking. It helps with my Christmas feeling though, a sunny BBQ Christmas dinner just doesn’t seem right when you’re from Sweden…