Pulmonary Langerhans cell histiocytosis, a nodular and eventually cystic lung disease of the upper and mid
lung zones, is a disorder of cells with Langerhans cell phenotype.
HP: poorly defined centrilobularmicronodules, widespread ground-glass opacities, mosaic attenuation and air trapping with a predominance of disease in upper and middle
lung zones under appropriate clinical setting.12
A chest X-ray revealed extensive ill-defined bilateral parenchymal opacities in the perihilar, mid, and lower
lung zones (Figure 1).
Systemic sclerosis-ILD is typically a nonspecific interstitial pneumonia (NSIP) pattern with groundglass opacities, interstitial pulmonary fibrosis, and honeycomb cystic changes in the lower
lung zones [5, 6, 23].
PLCH shares it cystic pathogenesis with usual interstitial pneumonia which is distinguished by clustered distribution of its cysts preponderant in the lower
lung zones [14].
The patient also had decreased breath sounds in both lower
lung zones and moderate pitting edema up to the knees.
In the case of emphysema, there is often apical predominant destruction of lung tissue, confining edema in a regional distribution to the lower
lung zones.(4) Reticular patterns of edema, radiographically indicated by the presence of Kerley A and Kerley B lines, have also been described as occurring with increasing frequency in the presence of emphysema.(5) This is attributed to impairment of lymphatic flow in the chronically diseased lung with decreased compliance and elasticity, resulting in stasis and engorgement of the lymphatic network even in the absence of significantly elevated pulmonary venous pressures.
Furthermore, PLCH predominates in the upper and mid
lung zones, a feature common in many smoking-related lung diseases.
Her lung examination revealed crackles in mid
lung zones bilaterally.
Breath sounds were diminished in left
lung zones. Percussion revealed dullness in the same regions.
Numerous small pulmonary nodules in the peripheral
lung zones of the lower lobes suggested pulmonary metastases.