Benard Gil, Sakai-Valente Neusa Y, Bianconcini Trindade Maria Angela
Division of Clinical Dermatology, Clinics Hospital, Medical School of the University of São Paulo, São Paulo, Brazil.
Am J Dermatopathol. 2009 May;31(3):288-92. doi: 10.1097/DAD.0b013e318193c74c.
Lepromatous leprosy patients may develop necrotic lesions, usually in the context of Lucio phenomenon (LP) or severe erythema nodosum (EN). The clinical and histopathological characteristics of the necrotic manifestations of both entities may eventually be confounded. We describe a patient with lepromatous leprosy who developed, since the 4th month of her first pregnancy, recurrent necrotic lesions in lower limbs, which, at the postpartum, worsened and led to partial destruction of ears and nose. In addition, she referred painful nodes on upper limbs since 1 year before pregnancy and intermittent swelling and tenderness of the ankles, which together with a right tibial and ulnar neuritis led to the diagnosis of, erythema nodosum leprosum (ENL). The histopathology of a biopsy of the upper limb (ENL) revealed a dermal-hypodermal inflammation with vasculitis and vascular lumen narrowing, whereas biopsy of the lower limb (LP) revealed small vessels with fibrin thrombi on the superficial layer of the dermis without inflammatory infiltrate and no evidence of vasculitis. Thus, besides having several different clinical features, LP and ENL result from different pathogenetic mechanisms. The histopathological and clinical features distinguishing both entities are proposed. This distinction is important because decrease in bacillary load through multidrug therapy is the main target in LP, whereas in ENL, concomitant reduction of the reaction by means of thalidomide or high-dose steroids is recommended.
瘤型麻风患者可能会出现坏死性病变,通常发生在卢西奥现象(LP)或严重结节性红斑(EN)的情况下。这两种病症坏死表现的临床和组织病理学特征最终可能会相互混淆。我们描述了一名瘤型麻风患者,自她首次怀孕的第4个月起,下肢出现反复坏死性病变,产后病情恶化,导致耳朵和鼻子部分毁损。此外,她自述在怀孕前1年上肢出现疼痛性结节,脚踝间歇性肿胀和压痛,同时伴有右侧胫神经和尺神经炎,这些表现共同导致了瘤型麻风结节性红斑(ENL)的诊断。上肢活检(ENL)的组织病理学显示真皮 - 皮下组织炎症伴血管炎和血管腔狭窄,而下肢活检(LP)显示真皮表层小血管有纤维蛋白血栓形成,无炎症浸润且无血管炎证据。因此,除了具有几种不同的临床特征外,LP和ENL是由不同的发病机制引起的。本文提出了区分这两种病症的组织病理学和临床特征。这种区分很重要,因为通过多药联合治疗降低菌量是LP的主要治疗目标,而对于ENL,建议使用沙利度胺或高剂量类固醇同时减轻反应。