EPIDERMODYSPLASIA VERRUCIFORMIS - A CASE ASSOCIATED WITH PRIMARY LYMPHATIC DYSPLASIA, DEPRESSED CELL-MEDIATED-IMMUNITY, AND BOWENS DISEASE CONTAINING HUMAN PAPILLOMAVIRUS-16 DNA
EPIDERMODYSPLASIA VERRUCIFORMIS - A CASE ASSOCIATED WITH PRIMARY LYMPHATIC DYSPLASIA, DEPRESSED CELL-MEDIATED-IMMUNITY, AND BOWENS DISEASE CONTAINING HUMAN PAPILLOMAVIRUS-16 DNA
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DOI：
10.1001/archderm.123.11.1511
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发表时间：
1987-11-01
影响因子：
--
通讯作者：
FARAS, AJ
中科院分区：
文献类型：
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作者：
OSTROW, RS;MANIAS, D;FARAS, AJ
疣状表皮发育不良是一种罕见的遗传性疾病，其特征是全身皮肤感染人乳头瘤病毒(HPV)，细胞免疫功能低下，皮肤主要暴露在阳光下的部位有向鳞状细胞癌转化的倾向。我们观察到一位37岁的男性，先天性淋巴管发育不良，有腹股沟和足部鳞状细胞癌病史，四肢都有水肿，有许多扁平的疣，躯干和手臂上有花斑疹样丘疹。腹股沟有尖锐湿疣病变，拇指指尖周围有一个疣状结节。活检发现躯干和手臂病变为疣，拇指病变为博文S病。4个手臂病变的分子杂交结果显示仅存在HPV3DNA；拇指表皮内癌组织中检测到HPV16DNA。免疫学检查显示对常规皮肤试验无效，丝裂原刺激的淋巴细胞转化抑制，B淋巴细胞计数下降，T淋巴细胞辅助/抑制比严重逆转。
Epidermodysplasia verruciformis is a rare, often hereditary disease characterized by a generalized cutaneous infection with human papillomavirus (HPV), depressed cell-mediated immunity, and a propensity for transformation of the warty lesions to squamous cell carcinoma on primarily sun-exposed areas of the skin. A 37-year-old man with congenital lymphatic dysplasia and a history of squamous cell carcinoma of the groin and foot was observed by us to have edema of all four extremities, numerous flat warts, and pityriasis versicolor-like papules over the trunk and arms. Condylomatous lesions were noted in the groin and a periungual verrucous nodule on the thumb. Biopsies showed the trunk and arm lesions to be verrucae and the thumb lesion to be Bowen''s disease. Results of molecular hybridization studies from four lesions of the arms showed the presence of only HPV 3 DNA; HPV 16-related DNA was detected in the intraepidermal carcinoma on the thumb. Immunologic evaluation revealed anergy to routine skin testing, depressed mitogen-stimulated lymphocyte transformation, decreased B-lymphocyte count, and a severe reversal of the T-lymphocyte helper:suppressor ratio.