Tularaemia A21.90

Author: Prof. Dr. med. Peter Altmeyer

Co-Autor: Dr. med. Ouadie El Makhtoum

Last updated on: 30.03.2021

Synonym(s)

Allergized tularaemia; cutaneous-glandular tularaemas; Deer fly fever; Deer Fly Fever; Francisella tularensis infection; Hare Plague; Lemming Disease; Lemming Fever; muco-glandular tularaemia; oculo-glandular tularaemia; Ohara's disease; rabbit fever; Rabbit fever; Rodent disease; typhoid tularaemia; ulcero-glandular tularaemia

History
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Chapin and Mc Coy 1912; Wherry and Lamb 1914

Definition
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Rare, plague-like rodent disease in Europe with possible transmission to humans (zoonosis) and typical primary complex. Duty to report! S.a. tularemia.

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Pathogen
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Francisella tularensis; gram-negative, coccoid, difficult to cultivate rods.F. tularensis is considered a highly contagious pathogen that can be transmitted by skin or mucous membrane contact with infected animals or their remains (e.g. hunters), consumption of contaminated, insufficiently heated meat (rabbits). Transmission by mosquitoes (Aedes spp., Chrysops spp.) or ticks (Dermacentor spp.) is also described. Transmission is also possible by contact via contaminated dust (faeces of infected animals) or contaminated water. Hares, beavers and ticks (Dermacentor spp.) are the main reservoirs of the pathogen.

Occurrence/Epidemiology
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Rarely, in Germany 20 to 50 cases/year are registered. Occurrence has been described especially in the rural population in Scandinavia, Russia, Japan, China, USA, Canada.

Etiopathogenesis
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Penetration of the pathogen into the human skin through small skin injuries, as well as through tick and fly bites. Infections by eating raw infected meat or drinking water as well as laboratory infections are possible.

Manifestation
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Predominantly 5-10 LJ or occurring in older adults after the 60th LJ.

Localization
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Especially the hands are affected.

Clinical features
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Incubation period: 2-14 days. General symptoms with headache and muscle pain may occur. A distinction is made between:

Cutaneous-glandular tularemia (ulcero-glandular form): primary effect at the site of entry, small nodular, livid infiltrate, ulcerating pustule. Painful, purulent melting, regional lymph node swelling, fistula formation. Development of a typhoid-like picture and atypical pneumonia are possible (5th day of illness). Roseolae and splenomegaly may occur.
Muco-glandular tularemia: primary effect on the oral mucosa in the form of aphthae. Involvement of regional lymph nodes corresponding to the cutaneous-glandular form.
Oculo-glandular tularemia: conjunctivitis, eyelid edema, involvement of regional lymph nodes. Usually spontaneous healing. Pulmonary (lung infiltrates) and abdominal forms are also observed with appropriate ports of entry. Immunity against systemic tularemia is possible after the disease has been overcome. However, recurrence of skin ulceration and reinfection is possible.
Typhoid tularemia: Sickness and gastrointestinal symptoms. Complicating pneumonia with respiratory distress may occur.
Allergic tularaemia: The tularaemia may be accompanied by polymorphous exanthema, sometimes scarlatina-like, also papulo-pustular or papulo-ulcerous. Nodular rashes are also possible.

Diagnosis
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Microscopic or cultural pathogen detection. The serum agglutination test is positive from about the second week of illness. A skin test with Francisella tularensis antigen is already positive in the first week of illness.

Differential diagnosis
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Ecthyma, actinomycosis, anthrax of the skin, syphilis I, boils, sporotrichosis, plague, malleus, lymphogranuloma inguinale, tuberculosis of the skin.

Complication(s)
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Lung abscess, meningitis, mediastinitis.

External therapy
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Symptomatic therapy with moist compresses, e.g. with polihexanide (Serasept, Prontoderm), potassium permanganate solution(light pink), quinolinol (e.g. quinosol 1:1000 or R042 ) or lotio alba, possibly with the addition of 2% clioquinol R050.

Notice! Early therapy is important because 1-5% of patients die if not treated adequately!

Internal therapy
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Drug of the 1st choice is streptomycin (e.g. Streptomycin Grünenthal) 2 times/day 0.5-1.0 g/day i.m. over 10 days, if necessary longer, in the full picture up to 25-30 mg/kg bw, including normalization of fever within the first 3-7 days, decrease of skin changes and lymphadenopathy over weeks.

As alternative antibiotics (especially for streptomycin resistance) spiramycin (e.g. selectomycin) 2-3 g/day p.o. for 7-14 days, gentamicin (e.g. re-fobacin) 3 mg/kg bw/day as i.m. injection, tetracycline (e.g. tetracycline Wolff) 3 times/day 500 mg p.o., erythromycin (erythro-hefa) 1.5-2 g divided into 2-3(-4) ED or ciprofloxacin 2 times/day 500 mg p.o.

Note(s)
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The name tularemia comes from the place where the pathogen was first found, Tulare in California.

Literature
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Faber M et al. (2018) Tularemia in Germany-A Re-emerging Zoonosis.Front Cell Infect Microbiol 8:40.
Jensen WA, Kirsch CM (2003) Tularemia. Semin Respir Infect 18: 146-158
McCoy GW, Chapin CW (1912) Further observations on a plague-like disease of rodents with a preliminary note on the causative agent, Bacterium tularense. J Infect Dis 10: 61-72
McGinley-Smith DE, Tsao SS (2003) Dermatoses from ticks. J Am Acad Dermatol 49: 363-392
Singh-Behl D et al (2003) Tick-borne infections. Dermatol Clin 21: 237-244
Wherry WB, Lamb BH (1914) Infection of man with Bacterium tularense. J Infect Dis 15: 331-340