A Unique, Histopathologic Lesion in a Subset of Patients
With Spontaneous Pneumothorax
Deborah A. Belchis, MD; Kris Shekitka, MD; Christopher D. Gocke, MD
 Context.—Spontaneous pneumothorax can be idiopathic
(primary), or it can occur in association with an underlying
predisposing condition (secondary). Spontaneous pneumo-
thorax may be a harbinger of an undiagnosed clinical
condition, which may be associated with serious systemic
abnormalities, making early recognition and diagnosis
important. The pulmonary pathology of some of these
disorders has not been fully elucidated.
Objective.—To review cases of pneumothorax in the
hope of identifying pathologic features that might correlate
to specific clinical syndromes.
Design.—The pathology computer files at 3 hospitals
were searched for all cases of spontaneous pneumothorax,
primary and secondary, regardless of etiology during a 11-
year period. Ninety-two cases were retrieved. Each of the
cases was evaluated for reactive eosinophilic pleuritis,
elastosis, pleural fibrosis, emphysema, intra-alveolar mac-
rophages, cholesterol clefts, vasculopathy, and intraparen-
chymal or intrapleural cysts. Clinical information
S pontaneous pneumothorax (SP) is a well-recognized
pulmonary complication of a wide variety of disorders,
ranging from endometriosis to metastatic malignancy. It has
been separated into primary and secondary types, where the
secondary cases are associated with a clear etiology. In
primary or idiopathic cases of SP, a clear etiology is not
apparent.1 Pathologic examination of cases is important
because SP may be the initial presentation in a variety of
conditions.2–4 Some of those diseases are well described and
have clearly recognizable pathologic features, such as
lymphangioleiomyomatosis or malignancy. Others are just
recently being recognized, both clinically and pathologically,
for example Birt-Hogg-Dubé syndrome.5,6 For other pneu-
mothorax-associated syndromes, clearly identifiable patho-
Accepted for publication July 19, 2012.
From the Department of Pathology, Johns Hopkins University
School of Medicine, Baltimore, Maryland (Drs Belchis and Gocke);
and the Department of Pathology, St Agnes Hospital, Baltimore (Dr
Shekitka).
The authors have no relevant financial interest in the products or
companies described in this article.
Presented in part at the annual meeting of the United States and
Canadian Academy of Pathology, Vancouver, British Columbia,
Canada, March 21, 2012.
Reprints: Deborah A. Belchis, MD, Department of Pathology, Johns
Hopkins University School of Medicine, 1st floor, Room AA158,
4940 Eastern Ave, Baltimore, MD 21224 (e-mail: dbelchi1@jhmi.
edu).
1522 Arch Pathol Lab Med—Vol 136, December 2012
regarding asthma and smoking history, site of the
pneumothorax, family history, radiographic findings, pre-
disposing conditions, recurrence, age, and sex were
extracted from the medical records.
Results.—In 11 patients (12% of all the patients with
spontaneous pneumothorax), a distinctive pattern of
pleural fibrosis with islands of fibroblastic foci within a
myxoid stroma was noted at the pleural-parenchymal
interface or leading edge. These lesions correlated with a
select subset of patients, consisting predominantly of
young men.
Conclusions.—Our review identified a distinct pattern of
pneumothorax–associated fibroblastic lesions in a subset of
cases of spontaneous pneumothorax. Whether this is
related to the pathogenesis of the pneumothorax remains
to be elucidated.
(Arch Pathol Lab Med. 2012;136:1522–1527; doi:
10.5858/arpa.2012-0330-OA)
logic features have been elusive, despite careful analysis. For
example, a recent article evaluating known cases of Marfan
syndrome7 describes distal acinar (paraseptal) emphysema
as a feature of Marfan syndrome. However, that is also a
feature believed to represent a nonspecific reaction to a
pneumothorax.7,8 Identifying the underlying disorder may
carry important clinical ramifications, exemplified by the
development of renal tumors in Birt-Hogg-Dubé syndrome
and aortic root dissection in patients with Marfan syndrome.
With this in mind, we conducted a multicenter, 10-year,
retrospective review of all cases of SP with the hope of
identifying and correlating histopathologic patterns with
specific clinical entities. During this review, we identified a
clinicopathologic entity recognizable based on its histologic
features. Early reports of SP8 seem to include a subset of
similar cases that were identified based on clinical features,
not histopathology. This report describes the histologic
entity, which we have termed pneumothorax-associated
fibroblastic lesion (PAFL).
MATERIALS AND METHODS
Cases of SP were retrieved from the pathology files of 3
institutions (St Agnes Hospital, Baltimore, Maryland; Northwest
Hospital, Randallstown, Maryland; and Sinai Hospital, Baltimore)
for a 11-year period (2001–2011). The pathology computer files
were searched for cases with the term spontaneous pneumothorax
listed anywhere in the report. All cases of SP were reviewed,
regardless of clinical impression of etiology (primary or secondary).
Pneumothorax-Associated Fibroblastic Lesion––Belchis et al
 Table 1. Clinical Characteristics of Spontaneous Pneumothorax With Pneumothorax-Associated Fibroblastic Lesion
Biopsy Age,
No. y/Sex Location/Recurrent Smoker Asthma Radiology Family History Other
1 16/M Apex/yes Nonsmoker No Blebs LUL Possible Marfan
syndrome, father’s
family all thin
and tall
2a 16/M LUL/yes Yes No
3 22/M Left anterior lobe/no Yes No Normal
4 17/M RUL and basal Nonsmoker Yes
segment of left
lung/yes
5 16/M Left apex/Unk Unk Yes
6 53/M RUL/yes Nonsmoker Unk
7 25/M Apex, left and left Yes Unk Bilateral interstitial
lower lobe/yes infiltrates on
chest x-ray
8a 18/M RUL/yes prior LUL Yes No
9 24/M Right apex/Unk Yes Unk Uncle with
pneumothorax
10 17/F LUL/yes Nonsmoker Yes Asthma
11 17/M LUL/yes Yes Unk
12 20/M LUL/yes Yes Unk Apical fibrosis with
biapical bullous
changes
Abbreviations: LUL, left upper lobe; RUL, right upper lobe; Unk, unknown.
a
Biopsies 2 and 8 are from the same patient.

Using as a guideline the morphologic features found in previous
reviews of SP,8–12 each case was evaluated for reactive eosinophilic
pleuritis, elastosis, pleural fibrosis, emphysema, intra-alveolar
macrophages, cholesterol clefts, vasculopathy, intraparenchymal
or intrapleural cysts, and underlying disorders, such as lymphan-
gioleiomyomatosis, infection, malignancy, or endometriosis. In
addition, the presence or absence of fibroblastic foci, a feature not
identified in prior reviews of cases of pneumothorax, was added.
The following clinical data were also extracted from the medical
records when available: asthma and smoking history, site of the
pneumothorax, family history, radiographic findings, predisposing
conditions, recurrence, age, and sex of the patient. Elastic stains
(Verhoeff-van Gieson) were performed on 5 cases. Ninety-two
cases were retrieved (the results from this large review will be
reported elsewhere). The studies were performed with the approval
of institutional review boards of each of the hospitals.
RESULTS
Eleven patients (12% of all patients with SP in the study)
were found to have a distinctive pattern of pleural fibrosis
with islands of fibroblastic foci within a myxoid stroma at
the pleural-parenchymal interface or leading edge. Often,
these lesions exhibited a wedge-shaped configuration, with
the broad base at the pleural surface and the apex toward
the lung parenchyma. In one patient (9%), serial lung
biopsies from separate episodes of pneumothorax were
available for review (biopsies 2 and 8 in Table 1).
The clinical and radiologic findings of our patients are
described in Table 1. Almost all were male. Ten of the
patients (91%) were younger than 25 years old (range, 16–
24); one (9%) was 53. The average age in the larger, 92-
patient group was 54, and the average in the PAFL subset
was 20. In comparison to the main study group, this age
difference is statistically significant (P , .05). The 53-year-
old patient was a nonsmoker. Of the others, 4 (36%) were
nonsmokers, 5 (45%) were smokers, and the smoking status
was unknown for 1 patient (9%). Three (27%) had a history
of asthma; 3 (27%) did not. The asthma history of the others
(5 of 11; 45%) was unknown. In all patients (100%), the
Arch Pathol Lab Med—Vol 136, December 2012
upper lobes or apex was involved by the pneumothorax.
One (9%) also had lower lobe involvement.
On gross examination, 4 of the resected lung specimens
(36%) had recognizable blebs or bullae. The largest bulla
was in a 16-year-old, nonsmoking patient and measured 9
cm at greatest dimension. Other bullae/blebs measured up
to 2 cm in greatest dimension. No masses were identified.
Histopathologic review revealed a distinctive lesion with a
zonal pattern characterized by dense, collagenous fibrosis at
the pleural-subpleural surface and younger, more-active,
fibroblastic-like foci at the pleural-parenchymal interface
(Figure 1, A through C). The fibroblastic-like foci are key to
recognition of the entity. The fibroblastic foci were scattered
along the leading edge (Figure 1, D). Less fibrotic,
subpleural lesions with a ‘‘stuck-on’’ appearance could be
identified (Figure 2, A). The lesions were multifocal and
extended varying lengths along the pleural surface, sepa-
rated by intervening normal pleura (Figure 1, A). As
indicated above, the lesions often assumed a triangular or
pyramidal configuration that was quite striking on low
power. They sometimes extended down pleural septa but
did not seem to disproportionately involve the septa. The
amount of subpleural surface involved varied by patient, and
the lesions ranged in size up to one 310 field, along the
inner pleural surface. The zonal pattern of dense, peripheral
fibrosis with more immature, reactive changes toward the
parenchymal surface was suggestive of temporal heteroge-
neity. The band of fibrosis in closest proximity to the
pulmonary parenchyma often showed a parallel nuclear
orientation of both the collagen bands and nuclei to the
border with the adjacent parenchyma (Figure 2, B). In some
patients, the fibrosis extended superficially into the lung
parenchyma, tracking along the alveolar or interlobular
septa. These areas of fibrosis typically extended only a few
millimeters into the lung and were never associated with
more-extensive interstitial pulmonary fibrosis. Intrapleural
cysts up to 5 mm were noted in the dense pleural fibrosis.
The cysts were lined by reactive hobnail cells, representing
Pneumothorax-Associated Fibroblastic Lesion––Belchis et al 1523
Figure 1. Histopathologic features of pneumothorax-associated fibroblastic lesion (PAFL). A, Wedge-shaped area of zonation demonstrated by
peripheral collagenous fibrosis and intervening normal pleura (brackets indicate 1 PAFL). B and C, Higher-power view illustrating the zonation and
fibroblastic foci (arrowheads) at the leading edge. D, Fibroblastic focus (hematoxylin-eosin, original magnifications 310 [A], 320 [B and C], 340 [D].
either hyperplastic type 2 pneumocytes or mesothelial cells
and were, therefore, different from blebs (intrapleural,
unlined collections of air). Occasional blebs were also
noted. In some of these cysts, the wall showed the same
reactive, fibroblastic appearance as the leading edges of the
lesion showed (Figure 2, C). Vascular proliferation was
noted in the dense pleural and subpleural fibrosis. The
adjacent alveoli ranged from normal to showing mild, type-
2 pneumocyte hyperplasia. Elastic stains demonstrated
disruption of the normal pleural elastic tissue (Figure 2,
D). This fibrotic, zonal lesion was absent in the other cases
(n ¼ 81) reviewed for the larger study, which served as a
random, unbiased internal control.
Additional findings in individual patients included eosin-
ophilic vasculitis; reactive eosinophilic pleuritis; intra-
alveolar macrophages; hemorrhage; patchy, subpleural and
peribronchiolar, chronic inflammation; and atelectasis. The
presence of these findings was variable from case to case.
Areas of patchy, subpleural airspace enlargement or distal
acinar emphysema were also present (see Table 2 for a list of
the additional major pathologic features).
Fibroblastic foci were not specific for this lesion. Six
additional cases (6 of 81; 7.4%) contained fibroblastic foci,
but they lacked the wedge-shaped PAFL lesions. In these 6
1524 Arch Pathol Lab Med—Vol 136, December 2012
cases without true PAFL lesions, the fibroblastic foci could
be ascribed to other conditions: 2 patients (33%) had usual
interstitial pneumonia, 2 (33%) had occupational lung
disease (pneumoconiosis and mixed dust pneumoconiosis
from coal work) and 2 (33%) were heavy smokers with
advanced emphysema. One of the latter patients had also
been treated for lung cancer with chemotherapy and
radiotherapy. Because true PAFL lesions were absent in
these cases, they were not included in the analysis.
COMMENT
Pneumothorax is associated with a wide variety of
disorders, ranging from lymphangioleiomyomatosis and
inherited disorders of collagen synthesis to pulmonary
endometriosis. Given the diversity of conditions associated
with pneumothorax, it is most likely that several different
mechanisms are responsible for a common result. With that
in mind, we hoped that careful review of these cases would
reveal morphologic features that segregate cases of pneu-
mothorax into relatively homogeneous clinical categories.
Our review identified a distinct pattern of pneumothorax-
associated fibroblastic lesions in a subset of cases of SP.
Pneumothorax-Associated Fibroblastic Lesion––Belchis et al
Figure 2. A, Stuck-on appearance of a small pneumothorax-associated fibroblastic lesion. B, Parallel fibrosis. C, Intrapleural microcyst with myxoid
fibrosis in the wall. Note the laminar collagen and fibroblasts parallel to the pleural surface. D, Disruption of elastic tissue (hematoxylin-eosin, original
magnifications 320 [A] and 340 [B and C]; Verhoeff-van Gieson stain, original magnification 320 [D]).
We think there is a substantial overlap between patients
with the histologic entity that we have described and the
cases reported by Lichter and Gwynne8 in their pioneering
study of SP. Of note, Lichter and Gwynne8 selected cases
based on clinical features (young age, absence of disease)
and not pathologic findings. They then examined the
pathology and described emphysema and cyst formation,
atelectasis, fibrosis with dense subpleural scars, chronic
inflammation, pigment deposition, alveolar cell prolifera-
tion, bronchial lesions with peribronchial fibrosis, and
vascular lesions. Our cases also demonstrated these
features. In addition, we were struck by the presence of
fibroblastic foci, a feature not identified in their analysis. It is
the recognition of fibroblastic foci in conjunction with the
subpleural zonal pattern of fibrosis that is distinctive in this
subset of patients and that can aid in the recognition of a
specific clinicopathologic entity. As mentioned above,
fibroblastic foci are not specific for any one entity but must
be a part of the entire morphologic lesion. The fibroblastic
foci play a similar role in their use in recognizing usual
interstitial pneumonia, where again, they are not patho-
gnomonic for usual interstitial pneumonia but are a well-
recognized, morphologic feature.13,14
Arch Pathol Lab Med—Vol 136, December 2012
Although the key features of PAFL (zonation, fibroblastic
foci, and wedge-shaped configuration) were not recognized
by Lichter and Gwynne,8 one of their photographs is
suggestive of a PAFL lesion. Of course they, like others,
were struck by the clinicopathologic presentation of the
cases and hypothesized a ‘‘nonspecific,’’ infectious etiology
abetted by ‘‘some local inherent predisposition.’’
Our findings are of interest because we took a different
approach in our study of patients with pneumothorax. We
deliberately included all patients in our larger morphologic
review, with a theory that, although some had underlying
conditions that could account for the development of
pneumothorax, others might have unique, histologic lesions
not yet recognized. Following review of our pathologic
findings, we noted that a subset of these patients12 had an
interesting, distinctive, subpleural lesion, with a zonation
pattern and fibroblastic-like foci at the leading edge.
Analysis of this group revealed it to be composed
predominantly, but not solely, of young men. This is a
similar group to those identified on clinical grounds by
Lichter and Gwynne,8 who also identified 2 young women
in their group.
The patchy pleural distribution and presence in only 12%
of all cases we examined (D.A.B., K.S., C.D.G., unpublished
Pneumothorax-Associated Fibroblastic Lesion––Belchis et al 1525
 Table 2. Pathologic Features
Case No. Pleura
1 Patchy pleural fibrosis with dilated
vascular channels intervening pleural
without fibrosis; type-2 pneumocyte
hyperplasia most clearly associated
with areas of fibrosis
2 Hyalinized pleural fibrosis
3 Pleural fibrosis with intrapleural cyst
formation, lining cells either
hyperplastic type-2 pneumocytes or
reactive mesothelial cells
4 Pleural fibrosis with intrapleural cysts,
foci suggestive of elastosis
5 Reactive eosinophilic pleuritis, pleural
fibrosis, no cysts
6 Reactive pleural fibrosis
7 Pleural fibrosis with elastosis, intrapleural
cysts
8 Intra-alveolar pigmented macrophages
9 Subpleural fibrosis with elastosis,
intrapleural cysts, mesothelial lining
cells
10 Subpleural cysts, reactive pleural fibrosis, Intra-alveolar macrophages
elastosis, interlobular reactive fibrosis
11 Reactive pleural fibrosis Giant cells
12 Pleural fibrosis with extension into the Intra-alveolar macrophages
subpleural pulmonary parenchyma
Abbreviation: DIP, desquamative interstitial pneumonia.
data, 2012) argue against this being a nonspecific, reactive
process. It is possible that these foci represent sites of old
disease because, like most patients biopsied for pneumo-
thorax, 8 out of 11 of our patients (73%) have had recurrent
pneumothorax (Table 1). However, we did encounter cases
in our larger review (D.A.B., K.S., C.D.G., unpublished data,
2012) that were recurrent but did not have PAFL, and in the
one case where we have tissue from both the initial
pneumothorax and the recurrence (biopsies 2 and 8, Table
1), PAFL are present in both. The cases from the larger
review serve as negative controls; that is, the patients
experienced SP but did not exhibit PAFL. Similarly,
although PAFL is most common in the young, it is not
present in the pathology specimens of every young person
with a SP. In the larger study of pneumothorax cases, there
were 20 patients (21.7%) younger than 26 years, and PAFL
was only identified in 10 of these (50%). The PAFL lesions
are not described in any systematic fashion in the pathology
specimens of lymphangioleiomyomatosis, Birt-Hogg-
Dubé,15,16 Marfan syndrome, or Langerhans cell histiocyto-
sis,17 entities with high rates of pneumothorax. This suggests
that PAFL is integral to the development of a specific type of
pneumothorax, rather than a nonspecific reactive effect.
The pathogenesis of SP is unknown. Currently, pneumo-
thoraces are believed to arise from a combination of
emphysema-like changes, which are blebs and bullae, and
abnormalities in the structure of the lung called pleural
porosity.18 As most cases of primary pneumothorax are
found to have emphysema-like changes and many diseases
that lead to secondary pneumothorax are associated with
cystic changes in the lung, it has been postulated that these
cysts rupture and lead to alveolar and pleural air leakage.18–20
However, fluorescein studies in patients with primary SP
show leakage of fluorescent dye away from the emphysema-
like changes.18,21 In addition, 6% to 15% of patients without
pneumothorax have been found to have blebs on thoracos-
1526 Arch Pathol Lab Med—Vol 136, December 2012
Macrophages Hemorrhage
Lightly pigmented intra-alveolar Pulmonary congestion and acute
macrophages, multinucleated giant hemorrhage
cells with cholesterol clefts
Intra-alveolar lightly pigmented Acute hemorrhage
macrophages
Numerous intra-alveolar macrophages, Not prominent
DIP-like pattern
Intra-alveolar pigmented macrophages Pulmonary congestion
DIP-like pattern Pulmonary congestion and
hemosiderin-laden macrophages
Not significant Congestion, acute hemorrhage
Intra-alveolar macrophages, foreign-body Congestion and acute hemorrhage
giant cells with cholesterol clefts
Intra-alveolar macrophages, cholesterol
clefts
Congestion and hemorrhage
Congestion and hemorrhage
copy, so blebs alone are not sufficient for pneumotho-
rax.19,22,23 It is theorized that the pleura is structurally
abnormal, leading holes or pores to form, allowing the
leakage of air, so-called pleural porosity.18,20 Whether the
pleura is abnormal in the same way in each condition is
unclear. It is possible that PAFL is a result of either a primary
structural abnormality of the pleura or an abnormal healing
process.
In summary, we present a unique histologic lesion arising
in a subset of predominantly male patients presenting with
primary SP. This lesion occurs in the apices of the lung and
in young patients who have no, or relatively short, smoking
histories. We note a strong, but not complete, overlap
between patients carrying our histologic lesion and the
clinically defined set of patients first identified by Lichter
and Gwynne.8 The etiology of this lesion remains uncertain.
Although this may be a florid, reactive-reparative process, it
is also possible that these patients have an underlying
abnormality that predisposes them to pneumothorax. We
look forward to independent confirmation of our findings
and, with a histologic entity in hand, further evaluation of a
possible etiology.
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