Authors Erica Lin MD, Kenneth Chen MD, Grace Lin MD, Matthew Nobari MD Division of Pulmonary and Critical Care Medicine and Department of Pathology UC San Diego, La Jolla, CA, 92037, USA
Case
Presentation
A 32 year-old female with a past medical history of asthma presented
to the emergency department for evaluation of an abnormal computed
tomography (CT), initially obtained for hemorrhoidal bleeding. She
reports a one-year history of gradually worsening shortness of breath
and cough productive of yellow sputum. She is a current tobacco user and
was previously employed as a smog technician. On physical examination,
she had decreased air entry bilaterally. Laboratory studies were notable
for ANA 1:80. Her chest imaging is shown below.
Figure 1: Axial (Left) and coronal (Right) images of CT chest
Question 1
What is the likely diagnosis?
A. Lymphangioleiomyomatosis (LAM) B. Birt-Hogg-Dube C. Pulmonary Langerhans Cell Histiocytosis D. Lymphocytic interstitial pneumonia
Question 2
Which of the following is the best initial treatment?
A. Smoking cessation B. Corticosteroids C. Antibiotics D. Pirfenidone
Answer
Question 1
C. Pulmonary Langerhans cell histiocytosis
Question 2
A. Smoking cessation
Discussion
The patient’s clinical and radiographic presentation is most
consistent with Pulmonary Langerhans Cell Histiocytosis. Choice A, B,
and D are incorrect based on the radiographic findings. High-resolution
CT chest can help distinguish amongst these cystic lung diseases. For
example, the CT findings in LAM (Choice A) typically show multiple
uniform thin-walled round cysts diffusely.1 BHD (Choice B) presents with lentiform thin-walled cysts in a basilar distribution on imaging.2
The cysts in LIP (Choice D) are random in distribution, may contain
internal structures, are often bordered by a vessel and can be
accompanied by ground-glass opacities.2
Pulmonary Langerhans Cell Histiocytosis (LCH) is a rare lung disorder
characterized by Langerhans cells. It was previously termed as
eosinophilic granuloma of the lung or pulmonary histiocytosis X.3,4 Studies have debated whether PLCH is an abnormal reactive process or a cellular proliferative process.1,4 Isolated PLCH occurs in young adults and almost exclusively in smokers, suggesting a possible contributing factor.1,3–5
While it is usually limited to the lungs, extrapulmonary manifestations
can occur and include bone lesions, diabetes insipidus and skin
changes.5 The classic radiographic findings demonstrate
bronchiolocentric nodular and reticular opacities and bizarre-shaped
cysts of varying sizes in a mid-to-upper zone distribution with sparing
of the costophrenic angle.1,4 Bronchoscopy with
transbronchial biopsy shows Langerhans cells that stain positive for
S-100 and CD1a and pigment-laden macrophages (Figure 2).1,3,4 The most important recommendation is smoking cessation, as cessation may result in stabilization or remission of disease.3–5
To return to our case, we did recommend tobacco cessation as an
initial treatment. After a long discussion, she recently quit smoking.
She will continue to follow-up with pulmonary and hematology to assess
whether her symptoms improve with this conservative measure.
Figure 2: Langerhans cell with S100 (Left) and CD1a (Right) immunostaining in our patient
References
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