Oroya fever A44.0 - A44.1

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 31.10.2023

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Synonym(s)

Carrión disease; Carrión fever; Peru wart; Verruga peruana

History
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Carrion 1886; Barton 1909;

Definition
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Biphasic disease caused by Bartonella bacilliformis and occurring only in South America, initially manifesting as acute, severe febrile hemolytic anemia (so-called orya fever), in a later stage as chronic localized or disseminated skin disease with wart-like angiomatous efflorescences reminiscent of Kaposi's sarcoma (verruga peruana or Peru warts). See also Bartonellosis.

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Pathogen
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Bartonella bacilliformis is a small, pleomorphic, peritrichous flagellated rod bacterium with marked tropism for human erythrocytes. Transmission by the sand fly Lutzomya verrucarum. Since the sand fly is found exclusively in mountain valleys above 800-3000 m in Peru, Ecuador and Colombia, the disease is also only widespread there.

Occurrence/Epidemiology
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Occurrence in Peru, Ecuador, Colombia (in mountain valleys of the Andes located at over 800 m altitude). In endemic areas in Peru 30-45% of the healthy population show antibodies against B. bacilliformis. 40% of the population in endemic areas is affected by either the acute febrile form (Oroya fever) or the chronic verrucous form of the disease (Verruga peruana).

Manifestation
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2/3 of the diseases occur before the 10th LJ. The majority of the diseases (90%) occur seasonally between January and June.

Clinical features
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Oroya fever: After an incubation period of 15-40 days, high intermittent fever, severe feeling of illness, swelling of lymph nodes, liver and spleen, rapidly progressive hemolytic anemia (due to the decomposition of bacteria-infected erythrocytes).

Verruga peruana: In the second stage of the disease, after overcoming Oroya fever, an eruption of multiple, coarse, red plaques and papules and soft, angiomatous, subcutaneous, 1-2 cm large nodules on the extensor sides of the extremities and on the face appears. These ulcerate easily, transform into papules and finally heal without scarring.

Notice! Oroya fever and Verruga peruana can also occur in isolation.

Histology
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Verruga peruana belongs to the bacterially induced lobular hemangiomas. In the late stages it resembles a granuloma pyogenicum with loose stroma and lobularly grouped capillary vessels.

Diagnosis
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Pathogen detection in the Giemsa-stained blood smear: Numerous bacteria, mostly intraerythrocytic. Cultural cultivation from the blood culture. Pathogen detection in histological sections from skin biopsy.

Complication(s)
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The mortality rate in untreated cases is 5-10%. Complications in untreated cases due to opportunistic pathogens (Mycobacterium tuberculosis, Toxoplasma gondii, Salmonella, Shigella) are occasionally observed.

Internal therapy
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Start antibiotic therapy immediately if suspected. Treatment with tetracycline (e.g. tetracycline Wolff) 4 times/day 500 mg p.o. or ampicillin (e.g. ampicillin ratiopharm) 3 times/day 0.5-5.0 g i.v. as a short infusion, also penicillin G 4 times/day 1 million IU as a short infusion or streptomycin (e.g. streptomycin Grünenthal) 1 times/day 1 g as a short infusion are effective.

Alternative: Erythromycin (e.g. Erythromycin ratiopharm): 4 times/day 500-1000 mg p.o. or i.v.

Operative therapie
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In the late stage, operative therapy of the skin changes in order to accelerate the healing process.

Prophylaxis
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Insect screen.

Aftercare
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After therapy and clinical freedom from symptoms, 10-15% of patients with acute Oroya fever peruana show bacteremia with B. bacilliformis. Herein lies the reservoir for B. bacilliformis.

Note(s)
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Between 1870 and 1890 an unknown epidemic occurred in Peru, affecting mainly railway workers. They suffered from high fever, weakness and anaemia. The disease spread mainly along the new railway line between the capital Lima and the town of La Oroya, which gave it its name. In 1881 a young Peruvian medical student died of the Oroya fever. At the same time he suffered from wartlike skin rashes. A study friend named Alcides Carrion suspected a connection between the fever and the warts. Carrion had the blood of a woman suffering from the rashes inoculated. 22 days later he fell ill with pain, nausea, fever and died some time later from the infection. To this day he is celebrated for his courage as Peru's national hero. The pathogen was first discovered in 1909 by Alberto Barton and was named after him as "Bartonella bacilliformis".

Literature
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Alexander B (1995) A review of bartonellosis in Ecuador and Colombia. At J Trop Med Hyg 52: 354-359
Barton AL (1909) Descripción de elementos endo-globulares hallados en las enfermos de fiebre verrucosa La Crónica médica de Lima 26: 7-10
Bravo F et al (2003) New and re-emerging cutaneous infectious diseases in Latin America and other geographic areas. Dermatol Clin 21: 655-668
Carrion DA (1886) Apuntes sobre la verruga peruana. Posthumous publication, 1886, Clinical studies at the Dos de Mayo hospital in Lima, based on nine cases.
Eremeeva ME et al (2007) Bacteremia, fever, and splenomegaly caused by a newly recognized bartonella species. N Engl J Med 356: 2381-2387
Garcia-Caceres V et al (1991) Bartnellosis. An immunodepressive disease and the life of Daniel Alcides Carrion. At J Clin Pathol 95: 58-66
Minnick MF et al. (2014) Oroya fever and verruga peruana: bar toneless unique to South America.
PLoS Negl Trop Dis8:e2919.
Vassallo C et al (2007) Bartonella-related pseudomembranous angiomatous papillomatosis of the oral cavity associated with allogeneic bone marrow transplantation and oral graft-versus-host disease. Br J Dermatol 157: 174-178
Wormser GP (2007) Discovery of new infectious diseases - bartonella species. N Engl J Med 356: 2346-2347