PULMONARY LYMPHANGIOMATOSIS REVEALED BY AN INTERSTITIAL SYNDROME IN A YOUNG ADULT

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Vol. 87 No. 4 (2009): Issue Apr 2009

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Aïda Ayadi-Kaddour
Jouda Cherif
Mona Mlika
Maged Béji
Faouzi El Mezni

Abstract

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.
Aims: The purpose of this article is to describe the main clinical and pathological features of this rare entity difficult to diagnose.
Case: 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.
Conclusion: 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.

Keywords:

Lymphangiomatosis, Lung, Thoracic wall

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