We report the case of a 29-year-old man who came to our clinic with a one-month history of fever consisting of a 39-day history of coughing, intense pain in the left pleurisy
She had no relevant medical or surgical history.
He had smoked 20 cigarettes a day for 10 years.
Physical examination was strictly normal, except for pulmonary auscultation in which there was a global decrease in vesicular murmur with isolated areas of egophonia.
Several analytical studies were performed that included: complete blood count, general biochemistry and normal blood gas results, creatinine clearance, calcuria, proteinogram, dosage of immunoglobulins and complement, C-reactive protein, rheumatoid factor and angiotensin enzyme markers.
Seizure performed showed a restrictive pattern.
A pulmonary diffusion test showed decreased diffusion (65%), compatible with a reduction in the exchange surface.
A simple chest X-ray showed a bilateral interstitial pattern.
A chest CT scan showed multiple cystic lung lesions, none of them greater than 2 cm, distributed in both lungs especially in the upper and middle lobes.
A lytic lesion was also observed at the level of the fourth left costal arch.
The Surgery Department was asked to perform an open lung biopsy.
Samples were taken from the upper and lower lobes of the left lung.
The histopathological study showed in the lung parenchyma an eosinophil inactivity and mononuclear cells with typical giant cells of Langerhans cells.
Immunohistochemical stains S-100 and CD1a were positive.
Identification electronics study was performed and the characteristics of Langerhans cells with polilobulated core and deep attachments were observed.
Structures formed by two double membranes were also seen, separated by a dense structure that meets the characteristics of a Birbeck granules in its tubular portion.
With the diagnosis of HPCL, the patient was recommended to quit smoking and was scheduled for a control chest CT at 6 months.
In this study, almost complete disappearance of cystic lesions was observed, persisting the costal lytic image.
Clinically, the patient showed significant improvement and was asymptomatic except for the persistence of mild costal pain in the left hemithorax that corresponded to the bone lesion observed on CT.
