Spontaneous pneumothorax with Isolated pulmonary langerhans cell
histiocytosis in an adult case: A common manifestation of rare disease
Mitra Samareh Fekri1, Faranak
Salajegheh2, Mohsen Nakhaie3,
Mohammad Rezaei Zadeh Rukerd3
- Cardiovascular Research Center, Institute of Basic and Clinical
Physiology Sciences, Kerman University of Medical Sciences, Kerman,
Iran, Kerman, Iran
- Clinical Research Development Unit, School of Medicine, Afzalipour
Hospital, Kerman University of Medical Sciences, Kerman, Iran
- Gastroenterology and Hepatology Research Center, Institute of Basic
and Clinical Physiology Sciences, Kerman University of Medical
Sciences, Kerman, Iran
Corresponding Author: Mohammad Rezeai Zadeh Rukerd, MD
Gastroenterology and Hepatology Research Center, Institute of Basic and
Clinical Physiology Sciences, Kerman University of Medical Sciences,
Kerman, Iran
E-mail: Mohammadrezaei75@yahoo.com
A 30-year-old man presented with sudden chest pain and evidence of
multiple cystic lesions in both long and left-sided pneumothorax in
HRCT. H&E stained section and IHC for CD1a and S100 were positive in
lung samples. The patient was treated with the diagnosis of Isolated
pulmonary langerhans cell histiocytosis.
Keywords: pulmonary langerhans cell histiocytosis, spontaneous
pneumothorax, cavitary long lesion
A 30-year-old man had sudden chest pain and shortness of breath two
hours before admission. He denies any past medical history and family
history and there was no history of medication usage, cigarette smoking,
or alcohol consumption.
In the physical examination of chest, there were evidences of
asymmetrical chest expansion and reduced breath sounds in left side.
There were no lesions found during the inspection of the scalp, fingers,
and nails.
The high resolution computed tomography (HRCT) scan of the chest
revealed widespread broad spread bizarre shape cystic formation in both
lungs as well as left-sided pneumothorax (Figure 1). A chest tube was
placed on the left side of the chest for the patient. Finally, the
patient underwent upper and lower lung lobes wedge biopsy. Hematoxylin
and eosin (H&E) stained section and immunohistochemistry (IHC) for CD1a
and S100 on both blocks (positive in many of the cyst wall cells)
confirmed pulmonary langerhans cell histiocytosis (PLCH). The patient is
evaluated in terms of the involvement of the body’s other organs. The
technetium-99m-methylenediphosphonate ((99m) Tc-MDP) whole body bone
scan showed no abnormalities in bone structures. Dynamic pituitary
magnetic resonance imaging (MRI) was perform and there was no abnormal
signal intensity in pituitary. Finally, the patient was treated with the
diagnosis isolated PLCH.
Langerhans Cell Histiocytosis (LCH) is a clonal disorder of langerhans
cells, which may affect various organs, such as bones, skin, pituitary
gland, and lungs.1,2
PLCH is a rare cystic pulmonary disease in adults that occurs almost
(more than 95%) in cigarette smokers.1 Isolated PLCH
is seen in around 50% of PLCH patients.2
PLCH have several manifestations, one of which is spontaneous
pneumothorax, which occurs in about 30-45% of patients, and in 10-30%
of patients, spontaneous pneumothorax is the first presentation of
PLCH.2
As a result, PLHC disease should be evaluated even in adults with no
previous medical history and no history of cigarette smoking who have
spontaneous pneumothorax and evidence of multiple lung cystic lesions,
and other organs should also be checked for LCH involvement.