Gallardo F, García-Muret M P, Servitje O, Estrach T, Bielsa I, Salar A, Abella E, Barranco C, Pujol R M
Department of Dermatology, Hospital del Mar - Institut Municipal d'Assistència Sanitària, Barcelona, Spain.
J Eur Acad Dermatol Venereol. 2009 Jun;23(6):639-47. doi: 10.1111/j.1468-3083.2008.03020.x. Epub 2009 Jan 13.
The presence of a prominent granulomatous tissue reaction in skin biopsies from primary cutaneous or systemic malignant lymphomas with secondary cutaneous involvement is a rare but well-known phenomenon.
This paper aims to characterize and study a series of cutaneous lymphomas showing a prominent granulomatous component.
The clinical, histopathological and evolutive features of granulomatous variants of mycosis fungoides (5 patients, 2 of them associating 'granulomatous slack skin' features), Sézary syndrome (1 patient), CD30(+) cutaneous T-cell lymphoma (2 patients), CD4(+) small/medium pleomorphic cutaneous T-cell lymphoma (1 patient), primary cutaneous B-cell lymphoma (3 patients) and peripheral T-cell lymphoma with secondary epithelioid granulomatous cutaneous involvement (4 patients) were reviewed.
The observed features were clinically non-distinctive. Only those cases presenting with granulomatous slack skin features were clinically suspected (2 patients). Non-necrotizing granulomata (11 patients) and granuloma annulare-like (4 patients) were the most frequently observed histopathological patterns. In five cases, no diagnostic lymphomatous involvement was initially observed. From our series, no definite conclusions regarding prognosis could be established.
The diagnosis of cutaneous lymphoma may be difficult when a prominent cutaneous granulomatous inflammatory infiltrate obscures the true neoplastic nature of the condition. However, the presence of concomitant lymphoid atypia may help to suspect the diagnosis. In doubtful cases, the clinical evolution and the demonstration of a monoclonal lymphoid B- or T-cell population may lead to a definite diagnosis.
在原发性皮肤或系统性恶性淋巴瘤伴继发性皮肤受累的皮肤活检中,出现显著的肉芽肿组织反应是一种罕见但广为人知的现象。
本文旨在对一系列具有显著肉芽肿成分的皮肤淋巴瘤进行特征描述和研究。
回顾了蕈样肉芽肿肉芽肿变体(5例患者,其中2例伴有“肉芽肿性皮肤松弛”特征)、 Sézary综合征(1例患者)、CD30(+)皮肤T细胞淋巴瘤(2例患者)、CD4(+)小/中多形性皮肤T细胞淋巴瘤(1例患者)、原发性皮肤B细胞淋巴瘤(3例患者)以及继发性上皮样肉芽肿性皮肤受累的外周T细胞淋巴瘤(4例患者)的临床、组织病理学和演变特征。
观察到的特征在临床上无明显特异性。只有那些表现为肉芽肿性皮肤松弛特征的病例在临床上受到怀疑(2例患者)。非坏死性肉芽肿(11例患者)和环状肉芽肿样(4例患者)是最常观察到的组织病理学模式。在5例病例中,最初未观察到诊断性淋巴瘤浸润。从我们的系列研究中,无法得出关于预后的明确结论。
当显著的皮肤肉芽肿性炎性浸润掩盖了病情的真正肿瘤性质时,皮肤淋巴瘤的诊断可能会很困难。然而,同时存在的淋巴细胞异型性可能有助于怀疑诊断。在可疑病例中,临床演变以及单克隆淋巴细胞B或T细胞群的证实可能会导致明确诊断。