P. Moerman et al., LYMPHANGIOMATOSIS OF THE BODY-WALL - A REPORT OF 2 CASES ASSOCIATED WITH CHYLOTHORAX AND FATAL OUTCOME, PEDIATRIC PATHOLOGY & LABORATORY MEDICINE, 17(4), 1997, pp. 617-624
We report on two cases of an unusual but distinctive variant of lympha
ngiomatosis, presenting at birth with predominantly cutaneous involvem
ent of the body wall and complicated by chylothorax. The lesion manife
sted clinically as a slowly progressive, diffuse, and fluctuant skin s
welling Eventually, almost the entire trunk became affected. There wer
e no bone lesions. Histologically, the soft tissues were diffusely inf
iltrated by interconnecting mazelike lymphatic vessels. The two infant
s died from infection at the age of 23 days and 10 months, respectivel
y. Our experience confirms that premortem histologic diagnosis of lymp
hangiomatosis is difficult to establish. Awareness of the condition an
d knowledge of its various clinical presentation forms are essential f
or proper recognition, assessment of the outcome, and evaluation of ne
w therapeutic measures such as interferon.