(Chest. 1999;115:275-276.)
© 1999
American College of Chest Physicians
The "Fairy Ring"*
A New Radiographic Finding in Sarcoidosis
Troy J. Marlow, MD;
Pavel I. Krapiva, MD;
Stephen I. Schabel, MD and
Marc A. Judson, MD, FCCP
*
From the Departments of Radiology (Mr. Marlow, Mr. Krapiva, and Dr.
Schabel) and Pulmonary and Critical Care Medicine (Dr. Judson), Medical
University of South Carolina, Charleston, SC.
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Abstract
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A patient who had the "fairy ring" finding shows another new
radiographic presentation of pulmonary sarcoidosis that clinicians can
add to the list of signs of the disease.
Key Words: alveolar disease • computed tomography • sarcoidosis
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Introduction
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The
"fairy ring" radiographic finding is another radiographic
presentation of pulmonary sarcoidosis. The patient reported here had
this, as well as other symptoms. Such radiographic evidence should
prompt the clinician to suspect sarcoidosis and to treat the patient
appropriately.
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Case Report
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A 43-year-old black woman, a lifelong nonsmoker, presented with
a cough of 9 months' duration. Initially, her cough was nonproductive.
Later, she produced sputum and occasionally had bouts of hemoptysis.
Two months prior to presentation, she developed painful, red eyes,
which transiently responded to cold compresses. Later, she also noticed
a red spot on her lower right shin which was painful, but it resolved
spontaneously. One month prior to presentation, she noted dyspnea. She
went to the emergency department with the preceding complaints and was
referred to a pulmonologist because sarcoidosis was suspected.
Spirometry values revealed a FVC of 62% of predicted and a
FEV1 value of 59% of predicted. A chest CT scan (Fig 1
)
and chest radiograph (Fig 2
) were performed. Sputum specimens were
negative for tuberculosis. A transbronchial biopsy revealed
noncaseating granuloma with stains and cultures negative for fungi and
mycobacteria; therefore, the biopsies were consistent with sarcoidosis.
Therapy with corticosteroids was started. Her pulmonary symptoms and
spirometric abnormalities resolved within 6 months.
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Figure 1. CT chest (lung windows). There is bilateral
involvement of the posterior lung fields with rings of granulomatous
tissue, all of different sizes. The central areas of each lesion are
normally aerated. Note the vasculature in the normal central
parenchyma. This is the typical appearance of the "fairy ring"
finding in sarcoidosis.
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Figure 2. Frontal view of the chest. This film, taken prior
to the CT scan, shows the classic "pawnbroker's sign" of right
paratracheal and bilateral hilar adenopathy. No evidence of the
"fairy ring" is found on the plain film.
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Discussion
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Sarcoidosis is an idiopathic systemic granulomatous disease which
is more common in black women, although men and nonblacks can be
affected. Radiographic manifestations have been documented in virtually
every organ system. The most common sites of involvement are the lungs,
skin, eyes, and lymph nodes.
Radiographic findings in sarcoidosis are diverse. Indeed, many
physicians have called sarcoidosis "the great imitator" because it
mimics many lung diseases, including tuberculosis, asbestosis,
carcinoma, and fungal disease. Lung involvement in sarcoidosis is
definitively diagnosed by the presence of noncaseating granulomas in
the parenchyma. Depending on their arrangement, they can create lesions
which are either nodular, reticular, or alveolar in appearance (Fig 2
).
With the exception of adenopathy, nodules are the most frequent finding
in pulmonary sarcoidosis.1
These are caused by the
accumulation of many granulomas which are a reaction to the initial
lesion of alveolitis.2
They may reach 1 cm in size and
usually are found around the bronchovascular tree or abutting pleura or
septae in the lung. Ground-glass attenuation is less common, tends to
follow the bronchovascular tree, and probably also is the result of
accumulation of many granulomas.3
Alveolar sarcoid occurs
when confluent granulomas involve the alveolar space. This typically
results in large opacities with air bronchograms,4
but
central necrosis often is observed.5
The patient in this report appeared to have alveolar sarcoid with
central necrosis on CT mediastinal windows. On lung windows, however,
it was noted that the central areas of the lesions were comprised of
normal lung tissue. This finding may be a distinct new type of alveolar
sarcoidosis with a ring of granulomatous tissue extending
concentrically from a given point in the lung. Strangely, the lung
tissue inside the ring appears undisturbed and without disease. No air
bronchograms are appreciated, and the process appears to behave in an
organized, circumferential fashion. Unlike the airspace distortion in
some focal sarcoid lesions, this entity shows no evidence of abnormal
air distribution. The center of the ring comprises normally aerated
lung tissue. We have called these lesions the "fairy ring" sign.
Fairy rings are well described in Celtic mythology. According to
legend, fairies would come out at night and dance in small circles in
the grass. Their tiny feet would beat a bare circular path at the edge
of the ring. When tired, the fairies would sit and rest on the
toadstools that defined the circle's periphery. The next day, passing
travelers saw only a circle in the grass with a ring of mushrooms. The
fairy ring pattern is actually created by the Marasmus
oreades mushroom, a fungus which may live to be more than 600
years old. Some botanists believed it to be the largest organism on the
planet.6
The granulomatous inflammation of sarcoidosis can resolve spontaneously
without therapy.7
Perhaps fairy ring lesions are formed by
central granulomatous inflammation which has spontaneously resolved
while new granulomatous inflammation develops at the periphery. The
fairy ring sign should be added to the list of radiographic
presentations of pulmonary sarcoidosis.
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Footnotes
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Correspondence to: Stephen I. Schabel, MD, Professor of
Radiology, Department of Radiology, Medical University of South
Carolina, 171 Ashley Ave, Charleston, SC 29425
Received for publication May 19, 1998.
Accepted for publication May 22, 1998.
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References
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Muller, NL, Kullnig, P, Miller, RR (1989) The CT findings of pulmonary sarcoidosis: analysis of 25 patients. AJR Am J Roentgenol 152,1179-1182[Abstract/Free Full Text]
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Dawson, WB, Muller, NL (1990) High-resolution computed tomography in pulmonary sarcoidosis. Sem Ultrasound CTMR 11,423-429
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Nishimura, K, Itoh, H, Kitaichi, M, et al (1993) Pulmonary sarcoidosis: correlation of CT and histopathologic findings. Radiology 189,105-109[Abstract/Free Full Text]
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Battesti, JP, Saumon, G, Valeyre, D, et al (1992) Pulmonary sarcoidosis with an alveolar radiographic pattern. Thorax 37,448-452[Abstract]
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Ichikawa, Y, Fujimoto, K, Shiraishi, T, et al (1994) Primary cavitary sarcoidosis: high-resolution CT findings [letter]. AJR Am J Roentgenol 163,745[ISI][Medline]
-
Lincoff, GH (1981) The Audubon Society field guide to North American mushrooms. ,772-773 Alfred A Knopf New York, NY.
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Neville, E, Walker, AN, James, DG (1983) Prognostic factors predicting the outcome of sarcoidosis: an analysis of 818 patients. QJM 208,525-533
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Abstract
Introduction
