51 Uysal Etal
51 Uysal Etal
51 Uysal Etal
Uysal et al., Int J Med Res Health Sci. 2014; 3(4): 1047-1050
International Journal of Medical Research
&
Health Sciences
www.ijmrhs.com Volume 3 Issue 4 Coden: IJMRHS Copyright @2014 ISSN: 2319-5886
Received: 1
st
Aug 2014 Revised: 1
st
Sep 2014 Accepted: 9
th
Sep 2014
Case report
PONTIAC FEVER ASSOCIATED WITH ERYTHEMA NODOSUM
*
Hilal Bektas Uysal
1
, Hulki Meltem Snmez
2
, Sertan Bulut
3
, Murat Telli
4
1
Assistant Professor,
2
Professor Department of Internal Medicine, Adnan Menderes University School of
Medicine, Aytepemevkii Merkez/ Aydin 09100, Turkey
3
Doctor in Pulmoner Diseases Department, Adnan Menderes University School of Medicine, Aytepemevkii
Merkez/ Aydin 09100, Turkey
4
Assistance Professor in Department of Microbiology, Adnan Menderes University School of Medicine,
Aytepemevkii Merkez/ Aydin 09100, Turkey
*Corresponding author email: hilalbektasuysal@yahoo.com
ABSTRACT
Legionellae are gram-negative bacteria found in clean water sources worldwide. Infection with Legionella
species presents as two distinct clinical forms; Legionnaires' Disease; characterized by pneumonia and Pontiac
Fever; a flu-like illness, characterised by a high attack rate and absence of fatal complications. Cutaneous
manifestations are very uncommon during Legionella infections. To our knowledge; only nine cases of
legionellosis, presenting with skin rash have been reported in the literature. The striking point is, only two men in
this nine cases of Legionella infections, had had Pontiac Fever. Here we present the third case of skin rash
associated with Pontiac fever, reported in the literature to date.
Keywords: Pontiac Fever, Erythema Nodosum, Legionella
INTRODUCTION
Legionellae are gram-negative bacteria found in clean
water sources worldwide. Water is the major
reservoir of Legionella species.
1
Most aerosolized
sources of bacterial-contaminated warm water,
including whirlpool spas, warm spring pools, garden
watering systems, decorative fountains, cooling
towers, and industrial cleaning systems that use high-
pressure water, have been linked to outbreaks of
legionellosis.
2,3
Infection with Legionella species presents as two
distinct clinical forms; Legionnaires' Disease (LD), a
multisystem illness characterized by pneumonia and
Pontiac Fever (PF), a self-limiting flu-like illness.
4
It
is not known why these two different clinical forms
occur, but organism inoculation, transmission modes ,
and host factors seems to be important.
5
Pontiac fever, occurs after exposure to aerosols of
water colonized with Legionella species. Disease
mainly diagnosed in outbreaks, rarely seen
simultaneously.
6
The first outbreak has been reported
in the United States in 1967 in Pontiac, Michigan,
that resulted from the contamination of the central
air-conditioning system from an evaporating
condenser. PF takes its name from this outbreak.
7
LD, is characterized by an acute severe pneumonia
with an average incubation time of 2 to 10 days, and
a low attack rate (0.1 to 5%) in general population.
PF, is the milder form characterised by a short
incubation time of 30-90 h, nearly 70-90% of high
attack rates
8
and absence of fatal or long term
complications
9
. Patients with PF have influenza like
symptoms. After a typical asymptomatic interval of
1248 h after exposure; fever, chills, headache,
DOI: 10.5958/2319-5886.2014.00051.4
1048
Uysal et al., Int J Med Res Health Sci. 2014; 3(4): 1047-1050
myalgia, arthralgia, malaise, sore troat, nonproductive
cough, abdominal pain, nausea occurs in most of the
cases.
7-9
There is no evidence of pneumonia on chest
radiography or physical examination of the patients.
Slight leukocytosis with neutrophilic predominance
may sometimes be detected.
10
Multisystemic extrapulmonary involvement is
observed during Legionella infections, however,
cutaneous manifestations is very uncommon. Only a
few cases of cutaneous involvement have been
described to date, according to our present
knowledge. Here we are reporting a Pontiac Fever
case, associated with ertythema nodosum.
CASE REPORT
A 39 year old woman with no underlying disease,
was admitted to our clinic with malaise, non-
productive cough, sore throat, hoarseness, myalgia,
arthralgia and fever for 3 days followed by warm,
painful, erythematous, non-pruriginous nodules on
bilateral pretibial areas and swelling of the right
ankle.
She did not use alcohol or tobacco. She was living in
a farm and working in the garden care and in the care
of farm animals. She had not travelled recently and
any one of the household had had an upper
respiratory infection within the preceding week.
Upon admission she had a body temperature of 37.5
C, blood pressure of 110/60 mmHg and a pulse rate
of 93 per minute. On physical examination, she had
pale skin and mucous membranes, apical systolic
murmur, unremarkable respiratory system and
abdominal examinations. Arthritis identified on the
right ankle with swelling and pain. She had red
coloured, rounded, approximately 1cm in diameter,
maculer, painful lesions on bilateral forearms and
bilateral pretibial rounded, slightly raised,
nonulcerative, painful, warm, red nodules compatible
with erythema nodosum (Fig-1, Fig-2)
Fig1: In a PF patient the cutaneous involvement of legs
with erythema nodosum
Fig- 2: In a PF patient the cutaneous involvement
of forearms.
Laboratory work-up demonstrated dimorphic anemia
of both iron and vitamin B12 deficiency. Her blood
test results showed the following: white blood cell
count at 9,97x 10
3
/L with remarkable neutrophilia (
82,4%), mild hyponatraemia at 135 mmol/L (normal
range 136-145 mmol/L), an erythrocyte
sedimentation rate during the first hour 64 mm
(normal < 20 mm) and C-reactive protein rate at 65,3
mg/L (Normal range 0-6 mg/L). Chest X-ray was
unremarkable.
Erythema nodosum has been associated with a wide
spectrum of infections, drugs and systemic diseases,
and may also be idiopathic. To consider all possible
etiologies of erythema nodosum a few tests
conducted. Throat swab, nasopharingeal swab, urine
cultures were negative. Antistreptolysin-O (ASO)
titer was in normal range. PPD skin test for
tuberculosis was 7 mm (negative for active infection
in Turkey). Contrast enhanced chest CT scan was
unremarkable. Pathergy test for Behcet disease was
negative. Anti-ds DNA, anti-nuclear antibody and
rheumatoid factor were all negative. There was no
clinical or laboratory alarm sign for malignancy. The
multiplex RT-PCR assay of nasopharingeal swab for
bacteria was positive for Legionella spp. Multiplex
RT-PCR assay of nasopharingeal swab for viruses
was negative. Legionella Urine Antigen Test (UAT)
was negative.
Immediately, bed rest, elevation of the legs and
supportive therapy started. For pain management oral
indomethacin 25 mg, twice a day started.
Two days after the admission, malaise, non-
productive cough, sore troat, hoarseness and myalgia
disappeared. Arthritis on the right ankle regressed and
erythema nodosum nodules evolved from bright red
to a brownish yellow discoloration resembling bruises
on the fifth day of admission.
1049
Uysal et al., Int J Med Res Health Sci. 2014; 3(4): 1047-1050
The patient became asymptomatic on the ninth day of
hospital admission, laboratory examination results
were all normal except the persisting increased
erythrocyte sedimentation rate (43 mm/h). She
discharged with a control plan after 3 weeks .
DISCUSSION
Cutaneous involvement during legionella infections is
very rare, and to our knowledge; only nine cases of
legionellosis, presenting with skin rash have been
reported in the literature
11-15
.The striking point is,
only two men in this nine cases of Legionella
diseases, had had PF.
16
Here we present the third
case of skin rash associated with PF.
The pathogenesis of skin involvement in the progress
of PF is unknown. It was considered to be mediated
either by a toxin produced by the organism or by an
immunological reaction formed by the host to the
bacteria, or other unidentified additional
mechanisms.
13,16
The clinical diagnosis of PF is usually very difficult
in most patients because of its nonspecific
presentation. Thus, the door to successful diagnosis
is, making proper microbiological tests. Serologic
and urinary antigen tests (UAT) are the most useful
routine tests for the diagnosis of PF.
Approximately 50% of the 48 species of Legionella
and 70 distinct serogroups identified have been
associated with human disease. Validated serologic
testing fort he majority of the 70 serogroups have not
been fully developed, only Legionella pneumophilia
serogroup 1 (LP1) can be reliably assayed by urinary
antigen tests.
17
Nonpneumophilia causes of the
disease are particularly difficult to diagnose. The
sensitivity of UAT is, proportionally increased with
the clinical severity of the disease
4
. Thus, patients
with PF may have been undiagnosed because of the
milder clinical course, if only UAT were used.
Previous studies about UATs in PF are very limited
18
.
This may be because these tests were primarily used
for diagnosis of hospitalised patients. Thus, the
validity of these laboratory tests for patients with
mild clinical illness and not demanding hospital
admission, is not clear. In our case the negative result
of UAT, demonstrates the cause of the PF is, an
another species, or serogroup of Legionella, rather
than LP1.
There is no agreed-upon definition of Pontiac fever.
The diagnosis is usually made on the basis of
epidemiologic, clinical, laboratory, and
environmental microbiology findings. Because of its
benign course and the absence of specific findings,
the occurrence of PF is often undiagnosed. Although,
the disease is self-limiting and patients recover
without treatment, the diagnosis is very important.
CONCLUSION
The diagnosis of PF is a marker of patients
environmental contamination by Legionella and
thereby should be a sign for taking all prevention
measures.
The case reported here demonstrates the importance
of using additional diagnostic methods (RT-PCR),
besides the fast and easy to perform urinary antigen
tests, to obtain a more accurate diagnosis, if
suspected. Furthermore, this case shows that PF can
be associated with cutaneous involvement.
Conflict of Interest :Nil
REFERENCES
1. Fliermans CB, Cherry WB, Orrison LH,
Smith SJ, Tison DL, Pope HD. Ecological
distribution of Legionella pneumophila.
Appl. Environ. Microbiol.1981; 41:916.
2. Fraser DW, Deubner DC, Hill DL, Gilliam
DK. Non pneumonic, short-incubation-period
legionellosis (Pontiac fever) in men who
cleaned a steam turbine condenser. Science
1979; 205:6901
3. Jones TF, Benson RF, Brown EW, Rowland
JR, Crosier SC, Schaffner W. Epidemiologic
investigation of a restaurant-associated
outbreak of Pontiac fever. Clin Infect Dis
2003; 371:2927
4. Diederen B. Legionella species and
Legionnaires Disease, Journal of Infection.
2008; 56:1-12
5. Mandell GL, Bennett JE, Dolin R Eds,
Principles and Practice of Infectious
Diseases, 4th edition, Churchill Livingstone,
1995: 2087.
6. Fenstersheib M, Miller M, Diggins C.
Outbreak of Pontiac fever due to Legionella
anisa. Lancet 1990; 336:3537
1050
Uysal et al., Int J Med Res Health Sci. 2014; 3(4): 1047-1050
7. Glick TH, Gregg MB, Berman B, Mallison
G, Rhodes WW Jr, Kassanoff I. Pontiac
fever: an epidemic of unknown etiology in a
health department.I. Clinical and
epidemiologic aspects. Am J Epidemiol
1978; 107:14960
8. Pancer K, Stypulkowska-Misiurewicz H:
Pontiac Fever non-pneumonic legionellosis.
Przegl Epidemiol 2003; 576:7-12
9. Fields BS, Haupt T, Davis JP, Arduino MJ,
Miller HP, Butler JC: Pontiac fever due to
Legionella micdadei from whirlpool Spa:
possible role of bacterial endotoxin. J Infect
Dis 2001; 184:1289-92
10. Luttichau HR, Vinther C, Uldum SA. An
outbreak of Pontiac fever among children
following use of a whirlpool. Clin Infect
Dis.1998; 26: 1374-78
11. Randall TW, Naidoo P, Newton KA.
Legionnaires disease in Port Elisabeth. S Af
Med J 1980; 581:7-23.
12. Helms CM, Johnson W, Donaldson MF.
Pretibial rash in Legionella pneumophila
pneumonia. JAMA 1981; 2451:758-59
13. Allen TP, Fried JS, Wiegmann TB.
Legionnaires disease associated with rash
and renal failure. Arch Intern Med 1985;
145:729-30
14. Calza L, Briganti E, Casolari S.
Legionnaires disease associated with
macular rash: two cases. Acta Derm Venereol
2005; 85:342-44
15. Ziemer M , Ebert K, Schreiber G, Voigt R.
Exanthema in Legionnaires disease
mimicking a severe cutaneous drug reaction.
Clin Exp Derm 2009; 34:72-74
16. Spitalny KC, Vogt RL, Witherell LE.
National survey on outbreaks associated with
whirlpool spas. Am J Public Health 1984; 74:
725-6
17. Fields BS, Benson RF, Besser RE.
Legionella and Legionnaires disease: 25
years of investigation. Clin Microbial Rev
2002; 15:506-26
18. Edelstein PH. Urine antigen tests positive for
Pontiac fever: implications for diagnosisand
pathogenesis. Clin. Infect. Diseases 2002;
44:229-31