Beylot-Barry M, Doutre M S, Vergier B, Pellegrin J L, Joly P, Leng B, Beylot C
Service de Dermatologie, Hôpital du Haut-Lévêque.
Ann Dermatol Venereol. 1998 Oct;125(10):720-3.
The precise diagnostic criteria for paraneoplastic pemphigus defined by Anhalt are not always present. In many cases, the clinical and histological features are misleading.
We observed a case of paraneoplastic pemphigus in a 60-year-old man followed for low-grade inactive nodal lymphoma. The clinical course was long. The main features were lichenoid cutaneous and mucosal lesions without bullae. The only histology finding was basal vacuolization, lymphocyte infiltration and suspended keratinocyte necrosis without acantholysis. This clinical and pathological situation continued for one year. Just a few days prior to the patient's death, the clinical situation suddenly worsened with major bullae involving most of the skin surface and mucosal areas including the esophagus and respiratory tract. Cyclosporine and bolus injections of corticosteroids did not prevent the fatal outcome.
This unusual lichenoid presentation has not been previously described. Associated skin erosions usually occur early in the disease course. The long lichenoid presentation for several months without acantholysis emphasizes the difficulties encountered in the diagnosis of paraneoplastic pemphigus. This diagnosis should be entertained in cancer patients who develop lichenoid lesions.
安哈尔特所定义的副肿瘤性天疱疮的确切诊断标准并非总是存在。在许多情况下，临床和组织学特征具有误导性。
我们观察到一例60岁男性的副肿瘤性天疱疮病例，该患者因低度惰性淋巴结淋巴瘤接受随访。临床病程较长。主要特征为无大疱的苔藓样皮肤和黏膜病变。唯一的组织学发现是基底细胞空泡化、淋巴细胞浸润和悬浮的角质形成细胞坏死，无棘层松解。这种临床和病理情况持续了一年。就在患者死亡前几天，临床情况突然恶化，出现了累及大部分皮肤表面和黏膜区域（包括食管和呼吸道）的大疱。环孢素和大剂量注射皮质类固醇未能阻止致命结局。
这种不寻常的苔藓样表现此前尚未见报道。相关的皮肤糜烂通常在病程早期出现。数月来持续的苔藓样表现且无棘层松解，凸显了副肿瘤性天疱疮诊断中所遇到的困难。对于出现苔藓样病变的癌症患者，应考虑这一诊断。
