Tularemia Clinical Presentation
- Author: Kerry O Cleveland, MD; Chief Editor: Burke A Cunha, MD more...
History
Most patients with tularemia experience an abrupt onset of fever, chills, malaise, and fatigue and develop 1 of 6 well-recognized clinical forms: ulceroglandular tularemia, glandular tularemia, oculoglandular tularemia, oropharyngeal tularemia, pneumonic tularemia, and typhoidal (septicemic) tularemia.
- Ulceroglandular tularemia
- This form accounts for approximately 80% of tularemia cases.
- F tularensis usually gains entry into the body via a scratch or abrasion and then spreads lymphatically, usually causing painful regional lymphadenopathy and an ulcerated skin lesion. Rarely, lymphangitis or nodular sporotrichoid lesions develop proximal to the ulcer.
- In rabbit-associated disease, the ulcer is located on a finger or hand in more than 90% of patients. See the image below.
Eschar on thumb and under thumbnail at the site of a rabbit bite in a patient with tularemia. - In tick-borne tularemia, the ulcer is found on a lower extremity or the perineal area in 50% of patients, the trunk in 30%, and the head in 5-10%.
- Glandular tularemia
- Oculoglandular tularemia
- In this form (1-2% of patients), F tularensis enters via the conjunctivae after inoculation from either splashing of blood or rubbing of eyes after contact with contaminated tissue fluids.
- Clinical manifestations are usually unilateral.
- Painful purulent conjunctivitis with preauricular or cervical lymphadenopathy may develop. Some patients experience chemosis, periorbital edema, and small nodular or ulcerative lesions of the palpebral conjunctivae.
- Oropharyngeal tularemia
- This is a rare form that may occur after consumption of poorly cooked meat of an infected rabbit.
- Patients with oropharyngeal tularemia usually report a sore throat, abdominal pain (due to mesenteric lymphadenopathy), nausea, vomiting, diarrhea, and, occasionally, frank gastrointestinal bleeding (caused by intestinal ulcerations).
- Pneumonic tularemia
- Primary tularemia pneumonia is uncommon and occurs after inhalation of the F tularensis.[6]
- Rarely acquired naturally, pneumonic tularemia may develop in laboratory workers.
- Pneumonia develops after hematogenous spread in 10-15% of patients with ulceroglandular tularemia and in 30-80% of those with typhoidal tularemia.
- Patients with this form of tularemia usually report a dry cough, dyspnea, and pleuritic-type chest pain.
- Chest radiography may reveal patchy ill-defined infiltrates in one or more lobes. Frank lobar pneumonia may also develop. Bilateral hilar adenopathy may be present. Bloody pleural effusions are characteristic and demonstrate a mononuclear cellular response.
- Adult respiratory distress syndrome (ARDS) develops in some patients.
- Typhoidal (septicemic) tularemia
- This form accounts for 10-15% of tularemia cases.
- It is more severe and probably represents F tularensis bacteremia.
- Patients with this form of tularemia present with fever, chills, myalgias, malaise, and weight loss. They often have pneumonia.
- Diagnosis is difficult because ulcers and lymphadenopathy are usually absent.
- Clinical symptoms
- Clinical symptoms correspond to the type of tularemia.
- As many as 20% of patients with tularemia have a blotchy, macular, maculopapular, or pustular rash.
- Erythema nodosum and erythema multiforme are rare.
- Other forms
- Rare manifestations of tularemia include osteomyelitis, pericarditis, peritonitis, endocarditis, and nervous system abnormalities, including meningitis, abscesses,[7] and optic neuritis.[8]
- Other possible manifestations include acute renal failure, hepatomegaly, abnormal liver function, and rhabdomyolysis.
Physical
Physical findings of tularemia vary based on the clinical form disease presentation. Patients have fever and possibly tender hepatosplenomegaly.
- Ulceroglandular tularemia
- This form is characterized by an ulcer at the site of F tularensis entry through the skin. The ulcer varies with the vector. It usually begins as a tender papule that eventually ulcerates and has a sharply demarcated border with a yellowish exudate. Initially, the base of the ulcer also has a yellowish exudate that turns to black.
- Regional lymphadenopathy develops. The lymph nodes are usually edematous and tender. They can become fluctuant and may drain spontaneously.
- Oculoglandular tularemia
- Ocular findings are usually unilateral.
- Painful conjunctivitis with purulent exudate may be present.
- Nodules or ulcerations may develop on the palpebral conjunctivae.
- Submandibular, preauricular, and cervical adenopathy are common.
- Corneal ulcerations may develop.
- Oropharyngeal tularemia: Exudative or membranous pharyngotonsillitis with regional adenopathy may be observed.
- Pneumonic tularemia: Chest examination findings may be normal in tularemic pneumonia, or rales may be present in the affected lung fields.
- Clinical symptoms: As many as 20% of patients with tularemia have a rash that may begin as blotchy, macular, or maculopapular and that may progress to pustular lesions. Erythema nodosum and erythema multiforme rarely occur.
- Less-common clinical forms of tularemia: In these forms of the disease (eg, meningitis, pericarditis, peritonitis, osteomyelitis), physical findings are the same as those commonly found in the clinical forms described above.
Causes
- Tularemia is caused by infection with the bacterium F tularensis.
Trevisanato SI. The 'Hittite plague', an epidemic of tularemia and the first record of biological warfare. Med Hypotheses. May 11 2007;[Medline].
Francis E. A summary of present knowledge of Tularaemia. Medicine. 1928;7:411-32.
Hauri AM, Hofstetter I, Seibold E, Kaysser P, Eckert J, Neubauer H, et al. Investigating an airborne tularemia outbreak, Germany. Emerg Infect Dis. Feb 2010;16(2):238-43. [Medline]. [Full Text].
Rydén P, Björk R, Schäfer ML, Lundström JO, Petersén B, Lindblom A, et al. Outbreaks of tularemia in a boreal forest region depends on mosquito prevalence. J Infect Dis. Jan 2012;205(2):297-304. [Medline]. [Full Text].
Hansen CM, Vogler AJ, Keim P, Wagner DM, Hueffer K. Tularemia in alaska, 1938 - 2010. Acta Vet Scand. Nov 18 2011;53:61. [Medline]. [Full Text].
Thomas LD, Schaffner W. Tularemia pneumonia. Infect Dis Clin North Am. Mar 2010;24(1):43-55. [Medline].
van de Beek D, Steckelberg JM, Marshall WF, Kijpittayarit S, Wijdicks EF. Tularemia with brain abscesses. Neurology. Feb 13 2007;68(7):531. [Medline].
Mitchell LA, Bradsher RW Jr, Paden TC, Malak SF, Warmack TS, Nazarian SM. Tularemia induced bilateral optic neuritis. J Ark Med Soc. Mar 2006;102(9):246-9. [Medline].
Gelfand MS, Slade W, Abolnik IZ. Tularemia serology: Differentiating true-positive and false-positive titers. Inf Dis Clin Pract. 1992;1:105-8.
Tärnvik A, Chu MC. New approaches to diagnosis and therapy of tularemia. Ann N Y Acad Sci. Apr 27 2007;[Medline].
Cronquist SD. Tularemia: the disease and the weapon. Dermatol Clin. Jul 2004;22(3):313-20, vi-vii. [Medline].
Gallagher-Smith M, Kim J, Al-Bawardy R, Josko D. Francisella tularensis: possible agent in bioterrorism. Clin Lab Sci. 2004;17(1):35-9. [Medline].
Choi E. Tularemia and Q fever. Med Clin North Am. Mar 2002;86(2):393-416. [Medline].
Cunha BA. Bioterrorism in the Emergency Room: Anthrax, Tularemia, Plague, Ebola, and Smallpox. Clin Microbiol Infect. 2002;8:489-503.
Cunha BA. Tularemia. In: Tickborne Infectious Diseases: Diagnosis and Management. Marcel Dekker; 2000:251-68.
Dienst FT. Tularemia: a perusal of three hundred thirty-nine cases. J La State Med Soc. Apr 1963;115:114-27. [Medline].
Ellis J, Oyston PC, Green M, Titball RW. Tularemia. Clin Microbiol Rev. Oct 2002;15(4):631-46. [Medline].
Evans ME, Gregory DW, Schaffner W, McGee ZA. Tularemia: a 30-year experience with 88 cases. Medicine (Baltimore). Jul 1985;64(4):251-69. [Medline].
Farlow J, Wagner DM, Dukerich M, Stanley M, Chu M, Kubota K. Francisella tularensis in the United States. Emerg Infect Dis. Dec 2005;11(12):1835-41. [Medline].
Gill MV, Cunha BA. Tularemia Pneumonia. Sem Respir Infect. 1997;13:61-67.
Guffey MB, Dalzell A, Kelly DR, Cassady KA. Ulceroglandular tularemia in a nonendemic area. South Med J. Mar 2007;100(3):304-8. [Medline].
Kandemir B, Erayman I, Bitirgen M, Aribas ET, Guler S. Tularemia presenting with tonsillopharyngitis and cervical lymphadenitis: report of two cases. Scand J Infect Dis. 2007;39(6-7):620-2. [Medline].
Leblebicioglu H, Esen S, Turan D, Tanyeri Y, Karadenizli A, Ziyagil F, et al. Outbreak of tularemia: a case-control study and environmental investigation in Turkey. Int J Infect Dis. May 2008;12(3):265-9. [Medline].
Markowitz LE, Hynes NA, de la Cruz P, et al. Tick-borne tularemia. An outbreak of lymphadenopathy in children. JAMA. Nov 22-29 1985;254(20):2922-5. [Medline].
Matyas BT, Nieder HS, Telford SR 3rd. Pneumonic tularemia on Martha's Vineyard: clinical, epidemiologic, and ecological characteristics. Ann N Y Acad Sci. Jun 2007;1105:351-77. [Medline].
Nigrovic LE, Wingerter SL. Tularemia. Infect Dis Clin North Am. Sep 2008;22(3):489-504. [Medline].
Penn RL, Kinasewitz GT. Factors associated with a poor outcome in tularemia. Arch Intern Med. Feb 1987;147(2):265-8. [Medline].
Sinclair JR, Newton A, Hinshaw K, Fraser G, Ross P, Chernak E, et al. Tularemia in a park, Philadelphia, Pennsylvania. Emerg Infect Dis. Sep 2008;14(9):1482-3. [Medline].
Sjöstedt A. Tularemia: history, epidemiology, pathogen physiology, and clinical manifestations. Ann N Y Acad Sci. Jun 2007;1105:1-29. [Medline].


