Adad S J, Fleury R N, Nunes A
Depto. de Patologia, Faculdade de Medicina do Triâgulo Mineiro, Uberaba, MG.
Hansenol Int. 1989 Jun;14(1):32-41.
Lymphomatoid granulomatosis (LYG) was first described by LIEBOW, A.A. et al. Histologically is characterized by an lymphohistiocytic infiltrate with granulomatous features, polymorphous and pleiomorphic, angiocentric and angiodestructive and mainly involves lungs, skin, kidneys and CNS. LYG does not involves spleen, bone marrow and lymph nodes. Presently there is a concept that LYG is an angiocentric variant of T-cell peripheral lymphoma and histologically indistinguishable from polymorphic reticulosis of the upper airways (midline 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 extrapulmonary 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 annular, 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 perineural 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, pleiomorphic 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.
淋巴瘤样肉芽肿病（LYG）最早由利博（LIEBOW, A.A.）等人描述。其组织学特征为伴有肉芽肿样特征的淋巴组织细胞浸润，具有多形性和异形性，以血管为中心且具有血管破坏性，主要累及肺、皮肤、肾脏和中枢神经系统。LYG不累及脾脏、骨髓和淋巴结。目前有一种观点认为，LYG是T细胞外周淋巴瘤的血管中心型变体，在组织学上与上呼吸道多形性网状细胞增生症（中线致死性肉芽肿）难以区分。LYG患者的预后不容乐观。早期病例使用环磷酰胺和泼尼松治疗可能会导致病情缓解。皮肤是最常受累的肺外器官，13%至34%的患者皮肤病变先于肺部受累。皮肤病变的临床特征可能各不相同，但常见为红斑和紫斑性斑块病变或中央消退的环形浸润性病变。这些病变的鉴别诊断包括环状肉芽肿、结节病和麻风病。由于麻风病在我们当中很常见，且LYG包括皮肤分支和神经干受累，皮肤病变有感觉减退或过敏以及肢体感觉异常，因此进行鉴别诊断至关重要。本报告涉及一名42岁男性，患有LYG皮肤病变并伴有肺部和全身表现。首次皮肤活检因细胞浸润呈肉芽肿性和神经周定位，大致提示为结核样麻风。患者死于呼吸功能不全，皮肤尸检结果显示有重要的组织学改变。浸润更为多形性、异形性且以血管为中心。在中枢神经系统、心脏、消化道、肝脏、前列腺、睾丸、肺和肾脏中也发现了相同的组织学特征。在最后这两个器官中有由特征性细胞浸润形成的大结节以及大片坏死区域。
