Ectrodactyly-ectodermal dysplasia-clefting syndrome (EEC): report of a case with perioral papillomatosis

We report a 13-year-old boy with ectodermal dysplasia, ectrodactyly, and syndactyly, hypospadias, photophobia, conductive hearing loss, and perioral papillomatosis. His father had ectrodactyly and hypotrichosis. The clinical picture suggested ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome. The presence of perioral papillomatosis, classically seen in Goltz syndrome, has been reported only once before in EEC syndrome.     

融合性网状乳头瘤病1例

患者男,21岁,前胸,后背分布深褐色扁平丘疹,呈网状排列,上覆少量鳞屑,组织病理示表皮角化过度,棘层轻度乳头瘤样增生,基底层色素增加,经用芳维甲酸乙酯治疗2个月后痊愈。     

抗真菌治疗融合性网状乳头瘤病一例

患者女,16岁,因腹背部色素沉着伴角化1年,于2010年7月来本中心就诊.患者1年前腹背部出现褐色色素沉着伴表皮轻度角化,近2～3个月受累面积缓慢增多,无明显自觉症状.否认家族中有类似病例.半年前曾在外院按鱼鳞病、黑棘皮病分别给予20％尿素乳膏、0.1％维A酸软膏外用治疗无效.体检:各系统检查无异常.皮肤科检查:腹背部见融合的斑状或片状褐色色素沉着,成不规则网状排列,表面轻度角化,触之有粗糙感,似触细砂纸,无鳞屑.取皮屑真菌镜检阴性.为明确诊断,取腹部皮损行组织病理学检查:表皮角化过度,角质层内可见散在真菌孢子,棘层疣状增生,真皮浅层散在炎症细胞浸润,呈融合性网状乳头瘤样改变.PAS染色:角质层内有较多圆形孢子(图1).诊断:融合性网状乳头瘤病.     

A case of early onset confluent and reticulated papillomatosis with an unusual localization

Confluent and reticulated papillomatosis is a relatively rare dermatosis of unknown origin. It is characterized by papules that become confluent in the center and reticulated at the periphery. The sites of predilection are the neck, interscapular region, inframammary area and abdomen. In a 15-year-old girl diagnosed with confluent and reticulated papillomatosis, the lesions first appeared on her knees and elbows when she was 4-years-old, and on the interscapular area when she was 13. Similar lesions arose on the left hand a few weeks previous to her visit. Two biopsy specimens were taken from the interscapular and elbow areas. The histopathological findings of this case fit the diagnosis of confluent and reticulated papillomatosis. We expect good results from azithromycin therapy.     

泛发型斑点状黑子样痣1例

报告1例泛发型斑点状黑子样痣。患儿男，2岁，就诊时发现双上肢和躯干等部位有巨大的咖啡斑，在其基础上散布多个斑点状黑子样痣和Spitz痣。根据病变的发生过程和组织病理改变，诊断为斑点状黑了样痣的一个亚型。     

A case of confluent and reticulate papillomatosis (Gougerot-Carteaud) with an unusual location

A 15-year-old female with a brown hyperkeratotic plaque in the pubic region confirming with the diagnosis of confluent and reticulate papillomatosis (CRP) is presented. The lesion disappeared rapidly after 3 weeks of treatment with 50% propylene glycol in water. The etiological role of Pityrosporum orbiculare in CRP is discussed.     

Proteus syndrome with widespread portwine stain naevus

Proteus syndrome is a rare condition comprising asymmetrical overgrowth of different parts of the body in association with various cutaneous abnormalities. We describe a 3-year-old boy with Proteus syndrome, who presented with hemihypertrophy of the right leg, asymmetric macrodactyly, subcutaneous masses and a widespread portwine stain interspersed with angiokeratomas on the right leg, scrotum and on the middle and left side of the back. Doppler ultrasound of the right leg did not show hypercirculation, but did reveal the absence of the right superficial femoral vein.     

急性泛发性扁平苔藓一例

忠昔,男,40岁,主刚双下肢红色丘疹3个月,泛发全身7d,于2011年4月15日就诊。3个月前无明显诱因患者小腿伸侧对称性出现3～5个绿豆大小紫红色丘疹伴瘙痒,夜间较重。     

Partial unilateral lentiginosis with ipsilateral Lisch nodules and axillary freckling

A 9-year-old Taiwanese girl had an unusual combination of multiple lentigines clustered on her left face, 3 cafe-au-lait spots on her left arm and ipsilateral axillary freckling. Two Lisch nodules of the iris were recognized in the left eye but none in the right eye, which is why we established a diagnosis of segmental neurofibromatosis 1. No family member had any sign of neurofibromatosis 1. Moreover, the patient had a large speckled lentiginous nevus involving the left side of her trunk, and her father showed a similar skin disorder on his left buttock. These lesions were taken to be unrelated to the patient's segmental neurofibromatosis 1. This case provides further evidence in favor of the concept that partial unilateral lentiginosis represents a mosaic manifestation of type 1 neurofibromatosis.     

乳腺癌广泛转移1例

患者女性，70岁。因左乳单纯癌于1993年10月15日行左乳癌改良根治术。术后进行了放疗、化疗。2000年12月枕部发现结节，且皮疹逐渐增多。经皮损组织病理和免疫组化诊断为“乳腺癌皮肤转移”。内脏CT示：内脏多发转移。全身骨扫描示：骨转移。     

骨髓增生异常综合征并发Sweet综合征

报告1例骨髓增生异常综合征并发Sweet综合征。患者男,65岁。头晕、乏力,面、颈部和四肢出现疼痛性红斑7个月。骨髓检查诊断为骨髓增生异常综合征。皮损组织病理检查：真皮水肿,有大量成熟的中性粒细胞及少量嗜酸性粒细胞浸润,伴核碎裂及溢血,诊断为Sweet综合征。