Granulomatose linfomatóide × hanseníase

Abstract

Lymphomatoid granulomatosis íLYG) was first described by LIEBOW, A.A. et a19. Histologically Is characterized by an lymphohistiocytic infiltrate with granulomatous features, polimorphous and plelomorphic, anglocentric and angio destructive and mainly involves lungs, skin, kidneys and CNS. LYG does not involves spleen, bone marrow and lymphnodes. Presently there is a concept that LYG is an angiocentric variant of T-cell peripheral Iimphoma and histologically indistinguishable from poiymorfic reticulosis of the upper airways ímidline lethal granuloma). The prognosis for patients with LYG is guarded. Treatment with cyclophosphamide and prednisone may lead to remission in early cases. The skin is the most commonly Involved extrapuimonary organ and in 13 to 34% of patients the skin lesions precede the pulmonary involvement. The clinical features of the skin lesions may vary, but frequently they are erythematous and violaceous plaque lesions or annular Infiltrated lesions with central clearing. The differential diagnosis of these lesions includes granuloma anular, sarcoidosis and Hansen's disease. Since Hansen's disease is common among us and that LYG includes involvement of cutaneous branches and nerve trunks, with hypo or hyperesthesia in skin lesions and paresthesia of limbs, it is of utmost importance to make differential diagnosis. This report deals with a 42 years old male with cutaneous lesions of LYG and concomitant pulmonary and systemic manifestations. A first skin biopsy roughly suggested tuberculoid leprosy due to a granulomatous and perinhural localization of cellular Infiltrate. The patient. died on respiratory insufficiency and the necropsy findings of the skin revealed important histological modifications. The infiltrate was more polymorphous, plelomorphic and angiocentric. The same histological features were found in CNS, heart, digestive tract, liver, prostate, testes, lungs and kidneys. In these two last organs there were large nodules made of the characteristic cellular Infiltrate and also large necrotic areas.