Kleikamp S, Böhm M, Frosch P, Brinkmeier T
Hautklinik, Klinikum Dortmund gGmbH, Dortmund.
Dtsch Med Wochenschr. 2006 May 26;131(21):1209-13. doi: 10.1055/s-2006-941753.
A 48-year-old obese man presented with thickening, coarseness and hyperpigmentation of the skin, especially of the intertriginous areas, papillomatous to verrucous lesions of the lips and buccal oral mucosa, and hyperkeratosis of the palms ("tripe palms") and soles. He was obese, reported sleep apnea and had a history of hyperuricemia, mixed hyperlipidemia and previous myocardial infarction. He was on a maintenance dose of a proton pump inhibitor for chronic gastro-esophageal reflux.
Immunohistochemical studies of the skin lesion revealed increased epidermal immunoreactivity for the melanocortin-1-receptor. Increased levels of tumor markers CA 19-9 (141100 U/ml), CA 72-4 (755 U/ml) and CEA (189 ng/ml) were found in the serum. Gastroscopic findings were suspicious of adenocarcinoma of the stomach: it was classified histologically as a signet-ring cell, non-mucinous adenocarcinoma. At the time of diagnosis the tumor had already metastasized to perigastric and peripancreatic lymph nodes with peritoneal carcinosis.
Since a curative resection was impossible a gastrojejunostomy was carried out. After this the patient received several courses of chemotherapy according to different schemes. Serum tumor marker levels and cutaneous signs regressed several times.
Marked acanthosis nigricans -- especially when associated with further cutaneous markers of malignancy, e.g. mucocutaneous papillomatosis or so-called tripe palms -- calls for thorough search for malignant tumor, also if metabolic or endocrinological abnormalities co-exist. A pathogenetic role of a-melanocyte-stimulating hormone in the development of the skin changes is suggested.
一名48岁肥胖男性,皮肤出现增厚、粗糙及色素沉着,尤其是在皮肤褶皱处,嘴唇及口腔颊黏膜有乳头瘤样至疣状病变,手掌(“鱼肚掌”)和脚底有角化过度。他体型肥胖,有睡眠呼吸暂停史,既往有高尿酸血症、混合型高脂血症及心肌梗死病史。他正在服用维持剂量的质子泵抑制剂治疗慢性胃食管反流。
皮肤病变的免疫组织化学研究显示,黑素皮质素-1-受体的表皮免疫反应性增加。血清中肿瘤标志物CA 19-9(141100 U/ml)、CA 72-4(755 U/ml)和癌胚抗原(CEA,189 ng/ml)水平升高。胃镜检查结果怀疑为胃腺癌:组织学分类为印戒细胞、非黏液腺癌。诊断时肿瘤已转移至胃周和胰周淋巴结,并伴有腹膜癌。
由于无法进行根治性切除,遂行胃空肠吻合术。此后,患者根据不同方案接受了多个疗程的化疗。血清肿瘤标志物水平及皮肤体征多次消退。
显著的黑棘皮病——尤其是当伴有其他皮肤恶性肿瘤标志物,如黏膜皮肤乳头瘤病或所谓的鱼肚掌时——即使同时存在代谢或内分泌异常,也需要全面排查恶性肿瘤。提示α-黑素细胞刺激素在皮肤改变的发生过程中具有致病作用。