[Pulmonary lymphangiomatosis revealed by an interstitial syndrome in a young adult].
BACKGROUND
Diffuse pulmonary lymphangiomatosis is an extremely rare congenital lesion affecting the lymphatic pulmonary system. It is carcterized by a multifocal proliferation and a pulmonary lymphatic vessels dilatation. It is essentially observed in newborns and infants. The diagnosis is based on histological findings.


OBJECTIVE
The purpose of this article is to describe the main clinical and pathological features of this rare entity difficult to diagnose.


METHODS
We report a case of a 19-year old girl who presented with a 2-month standing dyspnea. Radiographic findings consisted in a soft tissue infiltration and thickening in the two lobes. Based on histological and immunohistochemical findings, the diagnosis of diffuse pulmonary lymphangiomatosis was retained.


CONCLUSIONS
Diffuse pulmonary lymphangiomatosis is a difficult diagnosis which can be confused with lymphangioleiomyomatosis. Its worse prognosis could be improved by an early diagnosis enabling the use of interferon alpha 2B at the proper time.
Ayadi-Kaddour
Aïda
A
Service d'Anatomie Pathologique, Hôpital A. Mami de L'Ariana, Tunisie.
Cherif
Jouda
J
Mlika
Mona
M
Béji
Maged
M
El Mezni
Faouzi
F
D000328:Adult; D004417:Dyspnea / Q000209:etiology*; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000175:diagnosis*; D008202:Lymphangioma / Q000175:diagnosis*