Cotti Piccinelli Stefano, Tagliapietra Matteo, Cavallaro Tiziana, Labella Beatrice, Risi Barbara, Caria Filomena, Damioli Simona, Poli Loris, Padovani Alessandro, Ferrari Sergio, Filosto Massimiliano
Department of Clinical and Experimental Sciences, University of Brescia, 25100 Brescia, Italy.
NeMO-Brescia Clinical Center for Neuromuscular Diseases, Guusago, 25064 Brescia, Italy.
Biomedicines. 2023 Sep 6;11(9):2468. doi: 10.3390/biomedicines11092468.
The extent of nerve involvement in leprosy is highly variable in distribution and clinical presentation. Mononeuropathies, multiple mononeuropathies, and polyneuropathies can present both in the context of a cutaneous and/or systemic picture and in the form of pure neuritic leprosy (PNL). The differential diagnosis of leprosy neuropathy remains challenging because it is a very rare condition and, especially in Western countries, is often overlooked. We report one case of the polyneuropathic form of PNL (P-PNL) and one case of multiple mononeuropathy in paucibacillary leprosy. In both cases, the diagnosis was achieved by performing a sural nerve biopsy, which showed subverted structure, severe infiltration of inflammatory cells in nerve fascicles, granulomatous abnormalities, and the presence of alcohol-acid-resistant, Ziehl-Neelsen-positive bacilli inside the nerve bundles. Leprosy remains an endemic disease in many areas of the world, and globalization has led to the spread of cases in previously disease-free countries. In this perspective, our report emphasizes that the diagnostic possibility of leprosy neuropathy should always be taken into account, even in Western countries, in the differential diagnostic process of an acquired sensory polyneuropathy or multineuropathy and confirms that nerve biopsy remains a useful procedure in working up neuropathies with unknown etiology.
麻风病中神经受累的程度在分布和临床表现上差异很大。单神经病、多发性单神经病和多发性神经病既可以在皮肤和/或全身表现的背景下出现，也可以以纯神经炎型麻风（PNL）的形式出现。麻风性神经病的鉴别诊断仍然具有挑战性，因为它是一种非常罕见的疾病，尤其是在西方国家，常常被忽视。我们报告了1例多神经炎型PNL（P-PNL）病例和1例少菌型麻风的多发性单神经病病例。在这两个病例中，通过进行腓肠神经活检做出了诊断，活检显示结构破坏、神经束内炎性细胞严重浸润、肉芽肿异常以及神经束内存在抗酸、齐-尼氏染色阳性杆菌。麻风病在世界许多地区仍然是一种地方病，全球化导致病例在以前无此病的国家传播。从这个角度来看，我们的报告强调，即使在西方国家，在获得性感觉性多发性神经病或多神经病的鉴别诊断过程中，也应始终考虑麻风性神经病的诊断可能性，并证实神经活检在病因不明的神经病检查中仍然是一种有用的方法。
