Pulmonary involvement in the Birt-Hogg-Dube syndrome may be difficult to differentiate from lymphangioleiomyomatosis (LAM).
Parameters:
(1) distribution
(2) size and shape
(3) epithelial lining
(4) smooth muscle infiltration
(5) relationships to pulmonary vessels
(6) relationship to pleura
(7) immunostaining with HMB-45 (indicating melanogenesis)
|
Parameter
|
BHDS
|
LAM
|
|
distribution
|
basilar, peripheral or subpleural
|
diffuse
|
|
shape
|
elliptical, lentiform
|
round
|
|
epithelial lining
|
continuous
|
discontinuous
|
|
smooth muscle infiltration
|
no
|
yes
|
|
relationship with pulmonary vessels
|
abut proximal arteries and veins
|
none
|
|
relatationship to pleura
|
may abut pleural surface
|
none
|
|
HMB-45 staining
|
negative
|
positive
|
