Mediterranean spotted fever (Boutonneuse fever) is found in Africa, India, and Europe/Middle East adjacent to Mediterranean, Black, and Caspian seas. Patients have fever, eschar at site of tick bite, regional lymphadenopathy, and rash starting on the extremities--affects palms/soles.
CASES/YEAR
0 (US); 30,000 (Global)
OTHER NAMES
Boutonneuse fever; Mediterranean tick fever; Marseilles fever; Kenya tick typhus; India tick typhus; Israeli tick typhus; Astrakhan fever; Rickettsia conorii infection;
INCUBATION
7 days (mean); [PPID, p. 2355] 2-21 days; [CCDM, p. 518]
INITIAL SYMPTOMS
Fever, eschar at site of tick bite, regional lymphadenopathy, and rash starting on the extremities--affects palms/soles; rash may be petechial; [ID, p. 59, p. 1490]
PRECAUTIONS
Not directly transmitted from person to person. [CCDM, p. 518]
COMMENTS
FINDINGS:
The eschar begins as a 2-5 mm ulcer and is usually present with the onset of fever. The maculopapular rash follows on days 4-5. The rickettsial infections are characterized by a vasculitis that can lead to organ failure (heart, renal, and respiratory), bleeding, and neurological complications. [CCDM, p. 514-20] The disease is similar to Rocky Mountain spotted fever, but is less severe. [ID, p. 1490] About 10% of patients develop deep venous thromboses late in the course of the illness. In fatal cases, the infection causes vascular injury to many organs including the kidneys, liver, lungs, GI tract, heart, and brain (meningoencephalitis). The percentage of findings in cases series are: tache noire (72%), myalgia (36%), petechial rash (10%), conjunctivitis (9%), stupor (10%), meningismus (11%), splenomegaly (6%), hepatomegaly (13%), jaundice (2%), cough (10%), dyspnea (21%), myocarditis (11%), increased ALT (39%), thrombocytopenia (35%), and azotemia (6%). [PPID, p. 2352t, 2353] CNS complications of Rocky Mountain spotted fever and other severe spotted fever infections include meningoencephalitis, seizures, and coma. Signs of pulmonary complications are cough, infiltrates, pleural effusions, and ARDS. [PPID, p. 2352] Direct inoculation of the eye with the blood of an infected tick can cause Parinaud's oculoglandular syndrome. [Guerrant, p. 1005] Lymphadenopathy is present in a minority of cases. Also present are rash (97%) and eschar (50%). [Harrison ID, p. 689t] Regional lymphadenopathy is rare. [Cohen, p. 1667t] A malignant form of the disease (purpuric rash, shock, and multiple organ dysfunction) may occur in susceptible patients (alcoholic, diabetic, AIDS, debilitated). [Cecil, p. 2020] Nonspecific laboratory findings for rickettsial species infections include elevated transaminases, thrombocytopenia, and leukopenia or leukocytosis. [ABX Guide: Rickettsia species] Fatality rates without treatment: Rocky Mountain (20-25%); Mediterranean spotted fever (1-2.5%); [Cohen, p. 1667t]
DIAGNOSTIC
Serology (Indirect immunofluorescence assay) becomes positive 2nd or 3rd week of illness; Older Weil-Felix test not recommended; PCR of blood; Biopsy immunostaining or PCR; Culture for confirmation; [CCDM]
SCOPE
Africa and India; Europe and Middle East adjacent to Mediterranean, Black, and Caspian seas; [CCDM] India, Pakistan, Israel, Russia, Georgia, Bulgaria, Turkey, Ukraine, Ethiopia, Kenya, South Africa, Morocco & southern Europe; [PPID, p. 2355]
SIGNS & SYMPTOMS
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>fatigue, weakness
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>fever
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>myalgia
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G hepatomegaly
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G jaundice
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G liver function test, abnormal
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G nausea, vomiting
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H leukocytosis
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H leukopenia
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H lymphadenopathy
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H thrombocytopenia
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N headache
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N lethargy
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O conjunctivitis, acute
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O oculoglandular syndrome
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R cough
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R dyspnea
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S entry wound with lymph nodes
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S papules or plaques
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S petechiae and ecchymoses
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S rash (exanthem)
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S rash on palms
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S ulcer of skin
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*acute renal failure
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*bleeding tendency
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*encephalitis
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*meningitis
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*myocarditis
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*peripheral neuropathy
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*shock
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*stupor, coma
RESERVOIR
Cattle, Goats and Sheep, Dogs, Rabbits, Rodents, Wild Animals
RISK FACTORS
- Travel to endemic area
- Work in a medical or research lab
- Work or play in tick-infested area
REFERENCES FOR CASES/YEAR
1.
2. (Global) Guesstimate: assume 1/2 as common as African tick bite fever;