AJR 2003; 181:1433-1434
© American Roentgen Ray Society
Unique Adrenal Gland Imaging Features in Addison's Disease Caused by Paracoccidioidomycosis
Angela M. O. Leal,
Angela D. Bellucci,
Valdair F. Muglia and
Fabiano R. Lucchesi
School of Medicine of Ribeirão Preto University of São
Paulo São Paulo, 14049-900, Brazil
Note.—We thank Ayrton C. Moreira for providing the hormonal data on
the patient.
Paracoccidioidomycosis, or South American blastomycosis, is a chronic
infectious granulomatous disease caused by the dimorphic fungus
Paracoccidioides brasiliensis; it is the most common systemic mycosis
in South America. Imported cases have been reported in North America, Asia,
and Europe in individuals who had previously lived in areas in which the
disease is endemic, sometimes many years before the development of clinical
manifestations [1].
Paracoccidioidomycosis commonly involves the adrenal glands, as has been
proven in an autopsy series
[2]. Nevertheless, previous
reports of radiologic findings of paracoccidioidomycosis either have not
mentioned adrenal gland abnormalities or have described mainly normal adrenal
gland size and only contour irregularities and alterations in density
[3]. We describe unique imaging
features in a patient with Addison's disease who had proven disseminated
paracoccidioidomycosis. To our knowledge, our report is the first to describe
CT and MRI findings of this disease.
A 55-year-old man presented with a 7-month history of upper abdominal pain,
weight loss, nausea, vomiting, and anorexia. The physical examination revealed
dehydration and a cutaneous lesion on the upper eyelid. Results of laboratory
tests revealed hyponatremia and hypercalemia. The patient's basal cortisol
level was less than 40 nmol/L and did not rise after synthetic
adrenocorticotropic hormone injection, thus confirming the diagnosis of
Addison's disease or adrenal insufficiency. CT and MRI revealed that the
adrenal glands had irregular borders and were significantly enlarged, with the
right adrenal gland measuring 9.5 x 3.5 cm and the left measuring 7.5
x 3.8 cm. Predominant hypodensity was seen throughout the glands, and
contrast enhancement was seen mostly in the periphery, suggesting necrosis
(Figs. 4A and
4B).
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Fig. 4A. —55-year-old man with disseminated paracoccidioidomycosis and
Addison's disease. Contrast-enhanced CT scan shows enlarged adrenal glands
(arrows) with irregular borders. Right adrenal gland measured 9.5
x 3.5 cm and left gland measured 7.5 x 3.8 cm. Predominant
hypodensity is seen throughout glands, and contrast enhancement was seen
mostly in periphery, which is suggestive of necrosis.
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Fig. 4B. —55-year-old man with disseminated paracoccidioidomycosis and
Addison's disease. Coronal T1-weighted image shows enlarged adrenal glands
(arrows) with heterogeneous, predominately low signal intensity.
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The definitive diagnosis of active paracoccidioidomycosis was based on the
results of a serologic test (counterimmunoeletrophoresis revealed high
antibody titers against Paracoccidioides brasiliensis) and a biopsy
of the cutaneous lesion. Biopsy of the adrenal glands was not performed
because the clinical condition of the patient made it potentially dangerous.
The patient was placed on antifungal and glucocorticoid replacement therapy
and made an excellent symptomatic recovery. Five years later, imaging revealed
a significant decrease in the dimensions of the adrenal glands (Figs.
4C and
4D).
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Fig. 4C. —55-year-old man with disseminated paracoccidioidomycosis and
Addison's disease. Contrast-enhanced CT scan obtained 5 years after treatment
reveals marked decrease in dimensions of adrenal glands (arrows).
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Fig. 4D. —55-year-old man with disseminated paracoccidioidomycosis and
Addison's disease. Coronal T2-weighted image obtained 5 years after treatment
shows marked decrease in size of left adrenal gland (arrow). Right
adrenal gland is not visualized in this image.
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Clinical forms of paracoccidioidomycosis range from a benign self-limited
infection to a severe, progressive, and sometimes fatal disease involving
pulmonary and extrapulmonary tissues. The chronic form of the disease may
develop in patients many years after they have left the region in which the
disease is endemic [1]. An
autopsy series reported that the involvement of the adrenal glands ranges
between 44% and 80% of cases
[2]. However, adrenal
insufficiency is frequently not suspected clinically because clinical adrenal
insufficiency usually does not occur unless at least 90% of the adrenal cortex
has been destroyed and because the initial symptoms of adrenal insufficiency
are often nonspecific and may be attributed to the systemic mycosis
[1].
The differential diagnosis of bilateral adrenal gland enlargement must
include other granulomatous diseases, lymphoma, bilateral adrenal metastases,
bilateral hemorrhage, and the rare occurrence of bilateral primary adrenal
tumors [4]. The diagnosis of
paracoccidioidomycosis should be considered in any patient who has bilateral
adrenal gland enlargement and who has resided in an area in which the disease
is endemic, especially if there is evidence of adrenal insufficiency.
References
- Murray HW, Littman ML, Roberts RB. Disseminated
paracoccidioidomycosis (South American blastomycosis) in the United States.
Am J Med 1974;56:209
–220[Medline]
- Franco MF, Montenegro MRG. Anatomia patologica [in Portuguese]. In:
Negro G, Lacaz CS, Fiorillo AM, eds.
Paracoccidioidomicose. São Paulo, Brazil:
Sarvier-Edusp, 1982:97
–117
- Tendrich M, De Luca V, Tourinho EK, et al. Computed tomography and
ultra sonography of the adrenal glands in paracoccidioidomycosis: comparison
with cortisol and aldosterone responses to ACTH stimulation. Am J
Trop Med Hyg 1991; 44:83
–92
- Moulton JS, Moulton JS. CT of the adrenal glands. Semin
Roentgenol 1988;23:288
–303[Medline]
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