A Rash Decision - A case study on Lyme Disease

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Rebecca Dew, Medical Laboratory Scientist at Canterbury Health Laboratories presented this case study on Lyme Disease at the NZIMLS South Island Seminar in Hokitika in April 2013

Rebecca Dew, Medical Laboratory Scientist at Canterbury Health Laboratories presented this case study on Lyme Disease at the NZIMLS South Island Seminar in Hokitika in April 2013

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  • Here we have Mr B, a 60 year old man who moved from Scotland to Nelson in 2002 . He presented with a 5-6 year history of a slowly extending rash on the dorsal aspect of his right arm with no other symptoms that he could recall . He also had no history of any foreign travel other than the occasional trip back to Scotland where he spent time in the country hunting and fishing. Upon presentation, his G.P immediately referred Mr B to a dermatologist ………Any ideas????? These are the actual patient photographs .
  • You can see here the extent of the purplish rash on his arm and the thickening of the skin at the elbow and you can also see where the dermatologist took the biopsies . The dermatologist originally thought it may have been the result of a trauma to the elbow about 4-5 years ago since the skin was, apparently, quite normal until that point. The elbow became very swollen and erythematous (ear-a-them-a-tis) and the blotchy condition seemed to develop from there .
  • Early in the infection there is, in general, a bluish/red rash and swelling of the extensor surfaces of the hands and feet (the outside surfaces of a joint). This rash and swelling is unilateral and some will have flu-like symptoms with or without the rash . (not our patient photos) In Mr B’s case, the blotchy discolouration on his upper arm gradually developed and slowly extended but had a fairly sharp cut-off margin around the upper deltoid area. The rash extended more imperceptibly down the dorsal forearm . Mr B’s rash was largely asymptomatic, although a little itchy and “burny ” at times, the blotches were not particularly swollen or indurated (hardened). There were also some atrophic looking macules which had thin wrinkled skin over them. The dermatologist felt certain that it was the result of some sort of inflammatory condition and, due to the fact that it had a chronological association with the elbow injury , it must be something to do with that, perhaps an unusual post-inflammatory reaction .
  • In general, this type of rash may extend for months to years and obviously in Mr B’s case it was years. Chronic infection may result in recurring episodes of oedema, swelling and redness in one or more of the joints which may last for months at a time. The oedema resolves as the atrophy develops and the skin becomes more parchment-like with prominent veins. Other chronic symptoms may include numbness and tingling in hands or feet, severe fatigue, partial facial nerve paralysis or problems with memory, mood or sleep .
  • Later in the infection, some patients will develop hard nodules over bony prominences which can cause peripheral nerve or joint damage . Arthritis is also quite common and usually involves one or a few joints, especially the large, weight-bearing joints such as the knees . Swelling may or may not be present and the arthritis may be recurring or long lasting .
  • This slide shows the Histological appearance of the rash biopsy . It shows the stage of the infection called “Acrodermatitis chronica atrophicans” . This is the late stage of infection most often associated with Europeans with this infection and shows unusual, progressive, hardening of the skin and is due to the effect of continuing active infection . It is usually evident on the extremities , and begins with an inflammatory stage with bluish red discolouration and swelling and concludes months to years later with an atrophic phase. This is how Mr B was diagnosed. His dermatologist was not fully convinced of what he saw in the slides of the biopsies, so he sent them to another specialist in Auckland who determined the appearance to be “Acrodermatitis chronica atrophicans” and thus led to the diagnosis of…….
  • To recap, this particular patient Mr B was a deer farmer in the Highlands of Scotland prior to his move to New Zealand and as previously mentioned, he travelled back to Scotland from time to time where his family lived in the country. During his visits he spent time in the outdoors fishing and hunting. LYME DISEASE…….
  • Lyme disease is a bacterial infection caused by a Spirochaete of the genus Borrelia. There are several species which cause infection, however, the three main ones are B.burgdorferi, B.garinii and B.afzelii. The bacteria are helical or coiled hence the name Spirochaete. They are transmitted to humans from animals so we call it a zoonosis Transmission is by a vector which is usually an arthropod or insect which feeds on both sets of hosts. The vector in Lyme Disease is the Ixodes Tick. Lyme Disease was named after a town in Southeastern Connecticutt called Old Lyme where the first officially recognised case was found in 1979. Mr B had a positive Lyme ELISA which was followed up for confirmation by Western Blot analysis . This was performed at Westmead Hospital in Sydney. Confirmation is required because false positive ELISA results can occur if the patient has been exposed to other agents which may share proteins with Borrelia sp . The WB results for Mr B showed 3 bands to B.burgdorferi and 7 bands to B.afzelii , confirming the positive ELISA results and giving a diagnosis of LYME DISEASE. 5 bands are required to confirm true Lyme Disease . Mr B was absolutely sure he had no rash or obvious tick bite reaction at the time, however, he recalls getting tick bites which were not uncommon in the area where he lived in Scotland. He confirmed with a friend in that area that another case had been confirmed and it is obviously a recognised infection in the area.
  • This slide shows a picture of the Ixodes (IK-SO-DES) ricinus or the  Castor Bean Tick. This is the species most commonly found in Europe. Here we have the tick in the starved state and here the tick is fully engorged with a blood meal. The map shows the global distribution of the tick species including Ixodes scapularis which is the most common vector in the northeastern and north-central United States. Ixodes pacificus is the most common species spreading Lyme Disease on the Pacific Coast. This is the nymph stage of the tick, this is the form of the tick which usually transmits the infection because they are so small, only about 2mm in size, that they are not noticed when they are biting and feeding.
  • Lyme Disease is a systemic illness that can affect nearly any part of the body. The signs and symptoms are variable depending on the stage of disease. It has been referred to as a “Great Imitator” because the symptoms can mimic many other diseases including MS and ADHD . Fewer than 50% recall either a tick bite or rash. The Erythema migrans or “Bulls-eye rash” is considered a classic sign of LD although it is not always present. Without the tick bite, rash or Erythema migrans diagnosis can be quite difficult and the patient may develop multiple and chronic multisystem complications , including fever, sweats, chills, twitching, headache, shortness of breath, heart palpitations , the list goes on. Mr B is in some ways quite lucky because he doesn’t remember ever being unwell, he just had the rash. As mentioned, Lyme arthritis usually affects the knees but in a minority of patients, arthritis can occur in other joints, including the ankles, elbows, wrists, hips, and shoulders and joint erosion can occur . Neuroborreliosis is a disorder of the central nervous system caused by Lyme Disease infection. It progresses similarly to neurosyphilis but is a rare manifestation of late Lyme Disease . Neuroboreliosis is often preceded by the typical symptoms of Lyme Disease, including Erythema migrans and flu-like symptoms. Neurologic symptoms include the inflammation of the meninges, cranial nerve abnormalities, and altered mental status can occur and sensory findings may also be present.
  • It has been quite challenging getting actual figures on Lyme Disease since there seems to be many conflicting reports. The Centre for Disease Control in the US, however, indicates that there are approximately 25-30,000 new cases reported each year in the United States. Lyme Disease is a notifiable disease in North America so I expect this figure to be fairly accurate. According to the Health Protection Agency in the UK, there has been a 90% increase in cases across the UK since 2006 . In England and Wales in 2011 there were 972 cases confirmed . Some reports say that these figures should be multiplied by ten because the infection presents in a similar way to so many other infections that it may well be under-diagnosed and under-reported. In New Zealand the cases that we see are those where the patient has been bitten by ticks overseas but diagnosed here. One report I read, however, suggested that there are approximately 1400 new cases of Lyme Disease in New Zealand each year ……..NO WAY! In my experience we would be lucky to get 100 new cases. Officially, Lyme Disease is not present in Australia but there have been reports to suggest otherwise.
  • Antibiotics are used to treat Lyme disease. In general, recovery will be quicker and more complete the sooner treatment begins. Oral antibiotics are the standard treatment for early-stage Lyme disease. These usually include doxycycline for adults and children older than 8 , or amoxicillin or cefuroxime for adults, younger children, and pregnant or breast-feeding women . A 14- to 21-day course is usually recommended, but some studies suggest that courses lasting 10 to 14 days are equally effective. Intravenous antibiotics may be required if the central nervous system is involved, usually a 14 to 28 day course . This is effective in eliminating infection, although it may take some time to recover from the symptoms. IV antibiotics can cause various side effects, including a lower white blood cell count, mild to severe diarrhoea, or colonisation or infection with other antibiotic resistant organisms unrelated to Lyme. Mr B was placed on antibiotics and within 4 weeks he noted a distinct improvement in the rash and much less itching and burning which is always good.
  • Know where to expect ticks. Lyme Disease is found throughout the world so the next time you are on an overseas jaunt remember, you may come into contact with ticks during outdoor activities, around your accommodation or when walking through the bush, and of course hunting and fishing. Use a repellent with DEET (on skin and clothing) and wear long sleeves and long pants and if possible, tuck them into your socks or boots. Check your body and clothes for ticks after being outdoors. Ideally you should use a mirror to make sure all parts of your body are thoroughly checked but, I mean, really, who wants to do that ?!?!?!?!?!? Check your pets for ticks. Ticks may be carried indoors on your pet. Remove an attached tick from the skin by using fine-tipped tweezers as soon as you notice it. If a tick is attached to your skin for less than 24 hours, your chance of getting Lyme disease is extremely small. In 2002 Smithkline Beecham produced a vaccine called LYMErix but it displayed a limited efficacy, high price and a possible link to autoimmune arthritis and the development of LD itself . It has been removed from the market and law suits are in progress. Work is in progress for a better vaccine although much care is being taken.
  • Finally, Thanks to Dr Richard Clinghan for giving me a huge amount of information in the beginning and Dr Sophie Wen for doing some further background work on my behalf which I really appreciate and remember “FLICK THE TICK”. Thank you. Questions?????

Transcript

  • 1. A RASH DECISION Canterbury Health LaboratoriesRebecca Dew BSc, Grad Dip SciSerology/Virology
  • 2. Presenting Complaint Canterbury Health Laboratories• 60 year old man• Originally from Scotland• 5-6 year history of a slowly extending rash• No foreign travel
  • 3. Presenting Complaint Canterbury Health Laboratories• Right arm• Thickening over elbow
  • 4. Early Infection Canterbury Health Laboratories• Bluish/red discolouration with swelling• Extensor surfaces hands and feet• Unilateral
  • 5. Over the Years Canterbury Health Laboratories• Extends over months to years• Oedema resolves as atrophy develops• Prominent underlying veins
  • 6. Late Infection Canterbury Health Laboratories• Fibrous induration/ nodules over bony prominences• Sclerotic lesions can cause peripheral nerve or joint damage
  • 7. Histology Canterbury Health Laboratories• Acrodermatitis chronica atrophicans
  • 8. To Recap…… Canterbury Health Laboratories• Deer farmer in the Highlands of Scotland• What’s your answer?
  • 9. Lyme Disease Canterbury Health Laboratories• Positive Lyme serology• Western Blot – 3 bands to Borrelia burgdorferi – 7 bands to B. afzelii• No identified tick bite• Known cases among co-workers
  • 10. Castor Bean Tick Canterbury Health Laboratories• Ixodes ricinus – B. burgdorferi – B. afzelii – B. garinii
  • 11. Symptoms and ClinicalFeatures Canterbury Health Laboratories• Tick bite• “Bulls-eye” rash• Multiple and chronic multisystem complications – Arthritis• Neuroboreliosis
  • 12. Canterbury Health LaboratoriesReported Cases of Lyme Disease by Year,United States, 2002-2011 Centre for Disease Control and Prevention
  • 13. Treatment Canterbury Health Laboratories• Antibiotics - Generally, recovery will be quicker and more complete the sooner treatment begins• Oral antibiotics - early-stage Lyme disease – Doxycycline for adults and children older than 8, or amoxicillin or cefuroxime for adults, younger children, and pregnant or breast-feeding women• Intravenous antibiotics - CNS involvement.
  • 14. Prevention Canterbury Health Laboratories• Long sleeves and long pants• DEET• Check, check and check again…….• Vaccination???
  • 15. And to finish…. Canterbury Health Laboratories• Dr Richard Clinghan• Dr Sophie Wen