色素沉着—息肉病症候群三例报告

色素沉着—息肉病症候群在国内见于报告者已有50多例,此症的特点是皮肤色素斑和胃肠道息肉病。由于其症状的特殊性,它不仅和皮肤科有关,也和外科,内科、放射科和肿瘤科等有关。我们在临诊中见到三例,一例既有色素斑又有肠道息肉,可称完全型;另两例有血缘关系,其中一人有色素斑无明显的息肉,另一例有肠道息肉而无皮肤色素斑。兹报告如下以供参考。病例报告病例一:男性,21岁壮族,末婚,学生,文山州西畴县人。患者从出生后不久,即发现在口周围,唇部,口腔粘膜,手足掌蹠等处有黑色或深褐色色素斑。其数目逐渐增     

色素沉着-息肉综合征3例

色素沉着-息肉综合征亦称口周色素沉着-肠息肉综合征或口周雀斑-肠息肉综合征或黑斑息肉病,为一种少见的遗传性皮肤病,多见于青少年.近年笔者诊治3例,并结合文献进行讨论.现报告如下.     

色素沉着息肉综合征1例

<正>患者女,22岁。口周、唇部及双手黑色皮疹近7年。皮肤科检査:鼻部、口周及唇部见分布较密集0.5~4.0mm的黑褐色斑,手背及手指末梢有同样的点状、条索状皮损。其祖母、母亲及姐姐有同样症状,其母皮疹于40岁后自行消退。患者于去年在外院检查出肠息肉。色素沉着息肉综合征又称口周色素沉着肠道息肉综合征、口周黑子     

色素沉着－肠息肉综合征1例

色素沉着－肠息肉综合征１例刘铭锐，郭祖才，刘凯兰州医学院第二附属医院皮肤科（邮政编码７３００３０）患儿女，１５岁。因唇面部色素班就诊。患儿５岁时先在上唇发生色素斑，呈雀斑样色素沉着，逐渐增多，延及上下唇、颊部、鼻部、手指及口腔颊粘膜。曾在兰州某医院作...     

姐弟同患色素沉着-息肉综合征

报告2例色素沉着-息肉综合征患者.两患者系姐弟,皮损均表现为口唇黑斑,内镜检查均见消化道多发息肉.口唇皮损组织病理可见基底层色素增多,真皮浅层见少量噬色素细胞.家系调查发现该家族3代7人,5人患病,其中男4例,女1例.诊断:色素沉着-息肉综合征.治疗:胃肠多发息肉电凝电切术.     

父子同患口周色素沉着-肠息肉综合征

口周色素沉着-肠息肉综合征又称Peutz-Jeghers综合征（PJS）,是一种少见的常染色体显性遗传性疾病,以口唇、手指、足趾等部位黑、褐色色素沉着和胃肠道多发息肉为主要特征.1笔者诊治2例,报道如下.     

色素沉着-息肉综合征伴发肠梗阻1例

患者，男，26岁。出生时口唇有一粟粒大黑痣，5-6岁时黑痣增多，口唇、口周、口腔粘膜及手足均出现散在黑痣，7岁时出现腹痛、便血；以肠套叠、肠梗阻，在某医院手术治疗后小肠切除5cm。于本次入院前无明显诱因饱餐后出现恶心、呕吐，胃内容物多为宿食，有酸臭味，吐后自觉舒服，自服治胃药（药名及剂量不详），未见好转，于5天前，恶心、呕吐加重，伴腹痛，饮水呛咳，出现腹胀，而入我院外科治疗。患者父亲有口周色素斑，无肠道息肉症状。     

消化道多发性息肉综合征(Peutz-Jeghers Syndrome)

本综合征是指在唇部和颊部粘膜出现色素斑点合并有胃肠息肉并有家族史。这是一种特殊疾患,早已为人们所熟悉。色素斑:在皮肤及粘膜出现0.5～10毫米大、棕色或蓝棕色、圆形或不规则形、境界明显的色素斑。本综合征不可缺少的及在诊断上有价值的是发生在唇红部和颊部的色素斑。亦常见于口围皮肤,育时也发生于眼围、鼻孔。此外,也可以发生于四肢伸侧,较少发生在手指、足趾之屈侧以及肩胛部、肘部、膝关节部等处之伸侧。色素斑发生在典型部位     

脐肠系膜管残留致脐息肉

报告1例脐息肉。患儿男,12岁。脐窝起一黄豆大红色丘疹伴反复出血6年余,组织病理示皮损表皮缺如,局部含有大量肠腺腺腔和血管。诊断为脐肠系膜管残留致脐息肉。     

色素沉着一息肉综合征中色素沉着的治疗经验

色素沉着一息肉综合征即Peutz-Jeghers综合征,至今,对本病色素沉着的治疗报道不少,亦未见有治疗成功之报告.作者曾遇见一家3代中有10例现症患者,并对其中一例的色素斑试用冷冻治疗获得满意效果,现报告如下.     

Clinical characteristics of pigmented macules on palms and/or soles appearing after middle age in Japan

We have examined the clinical characteristics of pigmented macules on palms and/or soles. A total of 238 inpatients and 111 healthy persons over 40 years old in Japan were included in our analysis. We found: (a) that such pigmented macules may occur not only on healthy persons but also on patients who suffer from various internal disorders, (b) that, in both males and females, the incidence of pigmented macules is relatively high in malignancy patients in comparison with non-malignancy and control individuals, (c) that the incidence is higher in males than in females, (d) that, in both males and females, there was a tendency toward distribution on both palms and soles in malignancy patients in comparison with the non-malignancy and control individuals (p<0.01), and (e) that, among cases with pigmented macules, the number of pigmented macules is empirically greater in inpatients (the malignancy and non-malignancy groups) than in the controls.     

Ruvalcaba-Myhre-Smith Syndrome

In 1980 a syndrome was first described in two adult males, consisting of macrocephaly, pigmented macules on the glans and shaft of the penis, and hamartomatous intestinal polyps. Since then, 10 additional cases have been identified. Herein, we present two new cases and review the cutaneous manifestations as well as additional features in patients with the Ruvalcaba-Myhre-Smith syndrome.     

HYPOPIGMENTED MACULES IN ACANTHOLYTIC DISORDERS

Background. Widespread hypopigmented macules are rarely seen in heavily pigmented patients with Darier's disease. Previous hypotheses concerning the cause of decreased pigmentation suggest it is a postinflammatory phenomenon or that the hypomelanosis is evidence of sub-clinical acantholysis. Patients. This report presents 2 patients: a new case of disseminated guttate leukoderma in a black patient with Darier's disease and the first such case in a patient with transient acantholytic dermatosis (Grover's disease). Direct immunofluorescence and electron-microscopic studies were carried out on lesional biopsies. Observations. Numerous small hypopigmented macules were observed in two black patients followed for acantholytic disorders. Three biopsies of the hypopigmented macules revealed acantholysis, while one showed only decreased melanin. Direct immunofluorescence studies were negative. Electron-microscopic studies of the leukodermic macules showed sparse melanocytes and melanosomes that were mostly pigmented stage IV melanosomes. Conclusions. Disseminated guttate leukoderma can occur in transient acantholytic dermatosis, as well as in Darier's disease. It is readily apparent on darkly pigmented skin because of contrast. The etiology of this phenomenon is still unknown.     

伴中性粒细胞浸润的色素性荨麻疹

报告1例伴中性粒细胞浸润的色素性荨麻疹．患儿女,5岁。躯干、四肢色素沉着斑伴瘙痒1年余．体格检查示躯干、四肢广泛分布大量色素性斑片及少量红斑、抓痕．组织病理检查示真皮内大量中性粒细胞浸润及核尘,未见血管纤维素样坏死．高倍镜下见血管周围较多肥大细胞浸润．诊断为伴中性粒细胞浸润的色素性荨麻疹。     

窄波UVB诱导正常皮肤色素斑的研究

目的 观测窄波UVB诱导正常人腹部皮肤色素沉着斑的颜色变化指标。方法 采用窄波UVB治疗仪，以1.5倍的最小红斑量照射30例志愿者的腹部皮肤，形成人工色素沉着斑。在照射前、照射后1，2，4，6，8周，分别用 CM-2600d分光光度计测量局部皮肤的L*、a*、b*、△L、△a、△b、 △E值，以SIAscopy仪检测色素斑处血红蛋白、胶原蛋白、黑素总量，并捕获相应图像。同时，用数码相机拍摄志愿者腹部照片。结果 照射后局部皮肤呈淡灰色，4 h后出现局部潮红，1周后色素沉着最明显，2例受试者照射部位出现细小脱屑，所有受试者均无水疱发生。CM-2600d分光光度计测量照射部皮肤，结果显示，照射后1周，L*下降到最低，a*值升至最高，b*变化不明显。1周后L*值开始升高，而a*值下降，直到照射后8周，各自接近照射前水平。SIAscopy仪检测提示，照射后1周，皮肤血红蛋白增加最明显，以后迅速下降，低于照射前水平。而总黑素含量和胶原蛋白照射后持续缓慢增加，第6周达最高。结论 窄波UVB可诱导正常皮肤色素斑形成，通过观察皮肤色素的变化规律，客观地评估美白产品的功效。     

Reflectance confocal microscopy-assisted diagnosis of lichen planus pigmentosus distributed along multiple unilateral Blaschko's lines in a child: A case report

A boy with a 2-year history of asymptomatic, linear pigmented macules involving the right side of the trunk and right upper limb. RCM revealed the dermal papillary rings were destroyed, and numerous irregular particulate structures with high refractive values were distributed in the superficial dermis. The RCM features implied the possibility of interface dermatitis. RCM was a complementary diagnostic tool for linear pigmented macules.     

Dowling-Degos disease involving the vulva and back: case report and review of the literature

Dowling-Degos disease is a rarely encountered pigmentary disorder in which small brown-to-black macules appear in a clustered or reticulated pattern primarily at flexural sites. It usually occurs as an autosomal dominant trait but sporadic cases have also been reported. Dowling-Degos disease is sometimes associated with other cutaneous abnormalities, many of which appear to occur as a result of abnormal follicular development. The histology is distinctive with marked, heavily pigmented, slender, and often branched, elongation of the rete ridges. Dowling-Degos disease is caused by one of several loss-of-function mutations in the keratin 5 gene. Similar mutations are found in patients with Galli-Galli disease and that disorder is now considered to be a subset of Dowling-Degos disease. Medical therapy is ineffective but two patients have responded well to ablative laser therapy. We report a patient with the sporadic form of the disease who developed pigmented macules in the rarely involved sites of the lower back and vulva. Her vulvar lesions were treated with Er:YAG laser ablation.     

特发性发疹性斑状色素沉着症

报告1例特发性发疹性斑状色素沉着症.患者男,27岁.因背部、胸部出现浅灰色色素沉着斑1个月余就诊.否认发病前有炎症性皮肤病史,无用药史.皮肤科检查:背部、胸部散在直径0.5～2.5 cm浅灰色色素沉着斑,呈均匀一致的细网纹状,皮损境界大致清楚,互不融合,表面光滑无鳞屑.皮损组织病理检查:基底层黑素增多,基膜完整,未见细胞空泡化变性,真皮有较多噬黑素细胞.4个月后随访,约75%色素斑已消退或颜色变淡.诊断:特发性发疹性斑状色素沉着症.     

Pigmented macules on the palms and/or soles of Japanese subjects: influences of clinical severity and duration of illness.

We have suggested that pigmented macules on the palms and/or soles occur in response to various systemic diseases. In the present paper, we evaluated the influences of clinical severity and duration of illness in Japanese subjects. In males, cases who suffered from cerebral vascular accident (CVA) for long periods and/or severely tended to have numerous pigmented macules. In females, cases with CVA did not show any similar tendency. As the result of our prior and present studies, we conclude that underlying systemic disease itself can play a pivotal role in the development of pigmented macules on the palms and/or soles of Japanese subjects. We emphasize that such a pigmented macular eruption with characteristic clinical features is an important sign of internal disorders, including malignancy.     

Atypical pigmented penile macules

Two patients with pigmented lesions of the penis are described. The lesions consisted of asymptomatic, multifocal, irregular macules, with variegated pigmentation. The main differential diagnostic problem was with mucocutaneous melanoma. Histologic examination of the lesions showed basal layer hyperpigmentation. No cytologic atypia of melanocytes was detectable. The diagnosis in both cases was melanotic macules. Because of their atypical clinical appearance, genital melanotic macules are often misinterpreted as mucocutaneous melanoma. However histopathologic study solves the problem because genital melanotic macules show no melanocytic proliferation nor melanocytic atypia.