The “Fairy Ring”: A New Radiographic Finding in Sarcoidosis

doi:10.1378/chest.115.1.275

Chest

Volume 115, Issue 1, January 1999, Pages 275-276

https://doi.org/10.1378/chest.115.1.275 Get rights and content

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.

Section snippets

Case Report

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

Discussion

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,

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NL Muller et al.
The CT findings of pulmonary sarcoidosis: analysis of 25 patients
AJR Am J Roentgenol
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WB Dawson et al.
High-resolution computed tomography in pulmonary sarcoidosis
Sem Ultrasound CTMR
(1990)
K Nishimura et al.
Pulmonary sarcoidosis: correlation of CT and histopathologic findings
Radiology
(1993)

There are more references available in the full text version of this article.

Cited by (29)

Update thoracic sarcoidosis
2011, Radiologia
La sarcoidosis es una enfermedad granulomatosa multisistémica de causa desconocida, que afecta principalmente a los ganglios linfáticos torácicos y a los pulmones. La estadificación, que agrupa a los pacientes con similar probabilidad de remisión espontánea, se basa en los hallazgos de la radiografía de tórax, que tiene menor sensibilidad que la tomografía computarizada de alta resolución (TCAR) para detectar la afectación ganglionar, pulmonar y bronquial. Los hallazgos en TCAR pueden ser típicos, prácticamente patognomónicos, o atípicos. La TCAR aporta información sobre la actividad de la enfermedad y detecta incipientes signos de fibrosis y otras complicaciones. Para realizar el diagnóstico es precisa la correlación clínica, radiológica, y en muchos casos anatomopatológica.
La afectación cardiaca puede provocar muerte súbita; el diagnóstico, difícil, se basa en varias pruebas de imagen, como la resonancia magnética, que tiene mayor especificidad, y la tomografía por emisión de positrones; la confirmación mediante biopsia endomiocárdica se obtiene en pocos pacientes.
Sarcoidosis is a multisystemic granulomatous disease of unknown etiology. It mainly affects the thoracic lymph nodes and the lungs. The staging of sarcoidosis, which classifies patients according to their probability of spontaneous remission, is based on the plain chest film findings. Plain chest films are not as sensitive as high resolution computed tomography (HRCT) at detecting involvement of the lymph nodes, lungs, or bronchi. The high resolution CT findings can be typical, practically pathognomic, or atypical. High resolution CT provides information about the activity of the disease and detects incipient signs of fibrosis and other complications. To reach the diagnosis, it is necessary to correlate the clinical and radiological findings (and often the histological findings).
Cardiac involvement can cause sudden death. The diagnosis of cardiac involvement is difficult; it is based on various imaging tests, like magnetic resonance imaging, which is more specific, and positron emission tomography. Diagnostic confirmation by endomyocardial biopsy is obtained in few patients.
Pulmonary histoplasmosis presenting with the reversed halo sign on high-resolution CT scan
2011, Chest
Citation Excerpt :
The reports were published between 1996 and 2010 in the English literature as full-length articles or letters. We also searched using the keywords “atoll sign”13,14 and “fairy ring,”21 which were initially used to describe the RHS. We determined from each study the patient's age, sex, and diagnosis, and the number of RHSs and their lung distribution.
We describe the case of a 23-year-old man with pulmonary histoplasmosis whose high-resolution CT scan demonstrated the reversed halo sign. We also extensively review the literature about this CT scan sign. The reversed halo sign has been described in a number of diseases, both infectious and noninfectious. However, to our knowledge, this is the first reported case of pulmonary histoplasmosis presenting with this radiologic finding.
Atypical distribution of small nodules on high resolution CT studies: Patterns and differentials
2011, Respiratory Medicine
Citation Excerpt :
The authors named these lesions the “fairy ring” sign, after rings from Celtic mythology. According to legend, fairies come out at night and dance in small circles in the grass, and tiny feet beat a bare circular path at the edge of a ring.11 Kumazoe et al.12 reported the RHS in a case of sarcoidosis in which the HRCT images showed multiple, central ground-glass opacities surrounded by crescent or ring-shaped areas of consolidation in both lungs.
Accurate diagnosis of lung disease with high resolution CT is challenging and relies on a pattern-based approach coupled with knowledge of the distribution of the abnormalities in the lung parenchyma. Some findings and distributions of small nodules are specific for certain diseases, but atypical patterns have been described, especially for granulomatous diseases such as sarcoidosis and tuberculosis. Unusual HRCT aspects that involve the coalescence of small nodules have been termed the “sarcoid galaxy sign” and the “sarcoid cluster sign”. Other imaging findings such as the “reversed halo sign” and the “fairy ring sign” can also be composed of small nodules. The aim of this review was to describe and illustrate a range of conditions that manifest with atypical distribution of small nodules on HRCT. We discuss the various aspects, associated findings, and differential diagnosis particularly in sarcoidosis and tuberculosis.
The "sarcoid cluster sign". A new sign in high resolution chest CT
2009, Radiologia
Valorar la presencia de múltiples nodulillos puntiformes formando un grupo en el pulmón periférico en pacientes con sarcoidosis y describir este hallazgo como el nuevo “signo del cúmulo sarcoideo” en tomografía computarizada de alta resolución (TCAR).
Estudio retrospectivo de las TCAR de 91 pacientes diagnosticados de sarcoidosis torácica en el Hospital de León. Se valoró la presencia de grupos de múltiples nodulillos puntiformes en el pulmón periférico (“signo del cúmulo sarcoideo”), su morfología redonda o alargada y su distribución en los campos pulmonares. Se utilizaron reconstrucciones de máxima intensidad de proyección. Se realizó correlación radiopatológica en 3 pacientes.
Posteriormente, se valoró la presencia de este signo en TCAR en 120 pacientes consecutivos estudiados por sospecha de enfermedad pulmonar infiltrativa difusa (EPID).
De los 91 pacientes con sarcoidosis, 9 (6 varones y 3 mujeres, con edad media de 29 años) presentaban el “signo del cúmulo sarcoideo” en TCAR. La mayoría de los cúmulos mostraban morfología redondeada y su distribución fue predominante en la zona periférica no subpleural de los campos pulmonares superiores y medios; cuando los cúmulos tenían distribución periférica subpleural, nodulillos subpleurales formaban parte del cúmulo. Los cúmulos correspondían a granulomas no caseosos, no coalescentes, con predominio de linfocitos CD4+, sin fibrosis y con distribución linfangítica.
Únicamente 2 de los 120 pacientes con sospecha de EPID mostraban grupos periféricos de múltiples nodulillos puntiformes (“signo del cúmulo sarcoideo”); los 2 correspondían a sarcoidosis. El total de sarcoidosis diagnosticadas en este grupo fue de 1.
El nuevo “signo del cúmulo sarcoideo” en TCAR solamente se ha observado en pacientes jóvenes con sarcoidosis pulmonar. Puede ser un hallazgo radiológico útil en el diagnóstico de esta enfermedad.
To evaluate the presence of clusters of multiple small punctiform nodules in the peripheral regions of the lung in patients with sarcoidosis, and to report this finding as a new sign “the sarcoid cluster sign” in high-resolution chest CT (HRCT).
This is a retrospective study of the HRCT findings in 91 patients diagnosed with lung sarcoidosis at our hospital. We evaluated the presence of clusters of multiple small punctiform nodules in the periphery of the lungs (the “sarcoid cluster sign”), whether these were rounded or long in shape, and their distribution in the lung fields. We used reconstructions of maximum intensity projections (MIP). We correlated the imaging and histopathological results in three patients.
Afterward, we evaluated the presence of the “sarcoid cluster sign” on high resolution CT findings in 120 consecutive patients studied for suspected diffuse lung disease.
The “sarcoid cluster sign” was present at HRCT in 9 of the 91 patients with sarcoidosis (6 men and 3 women; mean age 29 years). Most clusters were rounded and most were located in the non-subpleural peripheral regions of the upper and middle fields of the lungs. When the clusters were located in the subpleural peripheral regions, the clusters contained small subpleural nodules. The correlation between the imaging and histopathological results revealed that the clusters corresponded to noncaseous, noncoalescing granulomas with a predominance of CD4+ lymphocytes, without fibrosis, that were distributed in the lymph vessels.
Only 2 of the 120 patients with suspected diffuse lung disease had peripheral clusters of multiple small punctiform nodules (the “sarcoid cluster sign”); these two patients had sarcoidosis, and a total of 21 cases of sarcoidosis were diagnosed in this group.
The “sarcoid cluster sign” on HRCT has been observed only in young patients with lung sarcoidosis. This finding could be useful in the diagnosis in the diagnosis of this disease.
Lipoid pneumonia showing multiple pulmonary nodules and reversed halo sign
2007, Respiratory Medicine Extra
We describe a 50-year-old man diagnosed with chronic exogenous lipoid pneumonia due to inhalation of paint spray. CT scan revealed multiple pulmonary nodules in both lungs. Resected specimens showed clusters of foamy macrophages in alveolar spaces, suggesting lipoid pneumonia. Long time inhalation of a small amount of paint oil may lead to lipoid pneumonia showing rare patterns such as multiple pulmonary nodules without surrounding parenchymal abnormal shadows. Five months later, some nodules increased still more in size, and some revealed reversed halo sign on CT. This case also suggests that reversed halo sign can be shown in lipoid pneumonia.
Imaging features of thoracic sarcoidosis
2002, Seminars in Roentgenology

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Chest

Selected ReportsThe “Fairy Ring”: A New Radiographic Finding in Sarcoidosis

Section snippets

Case Report

Discussion

The CT findings of pulmonary sarcoidosis: analysis of 25 patients

AJR Am J Roentgenol

High-resolution computed tomography in pulmonary sarcoidosis

Sem Ultrasound CTMR

Pulmonary sarcoidosis: correlation of CT and histopathologic findings

Radiology

Selected Reports
The “Fairy Ring”: A New Radiographic Finding in Sarcoidosis