Segard-Drouard M, Lefebvre I, Catteau B, Pannequin C, Delaporte E, Janin A, Thomas P
Clinique Dermatologique, Hôpital Claude Huriez, CHRU, Lille.
Ann Dermatol Venereol. 1997;124(9):615-8.
Paraneoplastic pemphigus is a bullous skin disease with characteristic polymorphous clinical presentation and precise histological and immunological features. We report a case of paraneoplastic pemphigus associated with chronic lymphoid leukemia involving the tracheobronchial epithelium.
A patient with chronic lymphoid leukemia developed pluriorificial lesions. There were several conjunctival, buccal and genital erosions associated with erosive plaques on the trunk, Nikolski's sign and bullous lesions suggestive of paraneoplastic pemphigus. Histology examination of a bulla showed intraepidermal blistering and suprabasal acantholysis. Direct immunofluorescence evidenced intercellular IgG and C3 deposits. Search for anti-intercellular substance antibodies was positive with fluorescence on specific paraneoplastic pemphigus substrates. At immunotransfer, the serum recognized several bands corresponding to 250, 230, 210 and 190 kD antigens, confirming the diagnosis of paraneoplastic pemphigus. Several days later, the patient's general condition deteriorated with bronchorrhea. Bronchial endoscopy visualized ulceronecrotic plaques. Tracheal biopsy evidenced acantholytic cells and intraepithelial cleavage. General corticosteroid therapy was initiated and led to improvement of the skin lesions but the patient died rapidly from pneumonia. Autopsy confirmed the presence of epithelial cleavage and acantholysis involving the trachea and bronchi.
This case illustrates the difficulty of diagnosing paraneoplastic pemphigus in the early stages. The pluriorificial lesions were suggestive of a Stevens-Johnson syndrome. Besides the genital, conjunctival and buccal mucosa, other mucosa can be involved. In our case, despite the absence of an immunological element, histology was highly suggestive of specific tracheobronchial localizations. The presence of such lesions, which should be searched for in all cases with bronchopulmonary manifestations, worsens the prognosis.
副肿瘤性天疱疮是一种大疱性皮肤病，具有特征性的多形性临床表现以及精确的组织学和免疫学特征。我们报告一例与慢性淋巴细胞白血病相关的副肿瘤性天疱疮，累及气管支气管上皮。
一名慢性淋巴细胞白血病患者出现多部位皮损。有多处结膜、口腔和生殖器糜烂，伴有躯干上的糜烂性斑块，尼氏征阳性及大疱性皮损，提示副肿瘤性天疱疮。一个水疱的组织学检查显示表皮内水疱形成和基底层上棘层松解。直接免疫荧光显示细胞间IgG和C3沉积。在特定的副肿瘤性天疱疮底物上进行抗细胞间物质抗体检测，荧光显示为阳性。免疫印迹法显示，血清识别出对应于250、230、210和190kD抗原的多条条带，确诊为副肿瘤性天疱疮。几天后，患者的一般状况恶化，出现支气管溢液。支气管内镜检查可见溃疡性坏死斑块。气管活检显示棘层松解细胞和上皮内裂隙。开始全身性糖皮质激素治疗，皮肤病变有所改善，但患者很快死于肺炎。尸检证实气管和支气管存在上皮裂隙和棘层松解。
该病例说明了早期诊断副肿瘤性天疱疮的困难。多部位皮损提示史蒂文斯-约翰逊综合征。除生殖器、结膜和口腔黏膜外，其他黏膜也可受累。在我们的病例中，尽管缺乏免疫学因素，但组织学高度提示气管支气管有特异性定位。在所有有支气管肺表现的病例中均应查找此类病变，其存在会使预后恶化。
