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Lymphomatoid Granulomatosis : Clinical Features II

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Lymphomatoid granulomatosis is predominantly a pulmonary disease with bilateral lung involvement in >90% of cases at presentation. Extrapulmonary lesions are seen in about one-third of cases.

LUNG: The patients with lung lesions present with cough, chest pain, and dyspnea. Constitutional symptoms such as fever, malaise and weight loss are frequent. Plain chest films show bilateral rounded opacities or diffuse fluffy infiltrates ranging in size from a few mm to several cm suggestive of metastases. The lesions usually involve middle and lower lobes and may show cavitation. Intrathoracic adenopathy or peripheral lymphadenopathy is rare.

CENTRAL NERVOUS SYSTEM: With CNS involvement, the presentation depends upon the location of the lesion and can include altered mental status, dementia, ataxia, hemiparesis, seizures, upper motor neuron signs, and cranial nerve palsies. Computed tomography scan of the brain may be normal or show multiple cortical infarcts or mass lesions.

SKIN: Skin lesions in LG are varied and consist of dermal or subcutaneous papules, plaques, or nodules with/without ulceration on the extremities and trunk.

This image shows angiocentric infiltrate composed predominantly of small lymphocytes (T cells) around a blood vessel in lung parenchyma.

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