Oral complications associated with D-penicillamine treatment for Wilson disease: a clinicopathologic report.
Oral complications associated with D-penicillamine treatment for Wilson disease: a clinicopathologic report.
复制标题
与 D-青霉胺治疗威尔逊病相关的口腔并发症：临床病理报告。
DOI：
10.1902/jop.2010.090736
复制
发表时间：
2010
期刊：
影响因子：
--
通讯作者：
I. Kaplan
中科院分区：
文献类型：
--
作者：
Ș. Tovaru;Ioannina Parlatescu;A. Dumitriu;A. Bucur;I. Kaplan
背景
肝豆状核变性（WD）是一种抑制肝脏铜释放的遗传性疾病。多器官表现涉及肝脏、神经系统、肾脏、眼睛、心脏和皮肤。弹性纤维损伤是WD D-青霉胺（D-PCA）治疗中最常用药物的并发症。这些变化在口腔中很少被描述。本文介绍了与WD相关的口腔并发症及其D-PCA治疗。
方法
临床，影像学和显微镜评价两个WD女性患者（年龄28和53），治疗D-PCA，口服药物相关并发症的临床和病理证据。
结果
病变包括嘴唇多个红色小丘疹、牙龈肿大、早发性牙周炎和反复口腔念珠菌病。口腔粘膜（牙龈，颊）活检显示在一个案例肉芽肿性炎症，并在两个案件，弯曲，红染色异常弹性纤维厚不规则团块。红色唇丘疹类似于蛇形穿通性弹性组织变性（EPS）。类似的病变在皮肤中也有描述，但以前从未与口腔或口周组织相关。除口腔病变外，其中1例患者出现对药物的全身不耐受，并改用盐酸曲恩汀。
结论
WD患者和其他接受D-PCA治疗的患者可能会出现口腔和口周并发症，在某些情况下，表现出粘膜和牙周组织中弹性纤维受损的特征。这是可能的，这种损害可能是导致WD患者牙周健康状况不佳的因素之一。对病变的识别可以导致对影响治疗剂的替换。
BACKGROUND
Wilson disease (WD) is a hereditary disease inhibiting copper release from the liver. Multi-organ manifestations involve the liver, nervous system, kidneys, eyes, heart, and skin. Elastic fiber damage is a complication of the most frequently used medication in the treatment of WD D-penicillamine (D-PCA). These changes have very rarely been described in the oral cavity. The article describes oral complications associated with WD and its treatment by D-PCA.
METHODS
Clinical, radiographic, and microscopic evaluation was done on two WD female patients (aged 28 and 53), treated by D-PCA, with clinical and pathological evidence for oral drug-related complications.
RESULTS
The lesions included multiple small red papules of the lips, gingival enlargement, early onset periodontitis, and repeated oral candidiasis. Biopsies of oral mucosa (gingiva, buccal) exhibited in one case granulomatous inflammation, and in both cases, thick irregular clumps of tortuous, red-staining abnormal elastic fibers. The red lip papules resemble elastosis perforans serpiginosa (EPS). Similar lesions have been described in the skin, but never before in association with oral or perioral tissue. In addition to the oral lesions, one of the patients developed general intolerance to the drug and was switched to trientine hydrochloride.
CONCLUSIONS
WD patients and others treated by D-PCA may develop oral and perioral complications, in some cases exhibiting features of damaged elastic fibers in the mucosa and periodontal apparatus. It is possible that this damage may be one of the factors responsible for poor periodontal health in WD patients. Recognition of the lesions can lead to replacement of the affecting therapeutic agent.