弥漫性非表皮松解性掌跖角化病1例

1病历摘要 患者女,42岁。闲双侧掌跖角化40余年,于2006年9月来我院就诊。患者自婴儿期起无明显诱凶双手掌出现对称性片状红斑,角化,形状不规则．受累皮肤粗糙增厚,同时累及双足和甲板,指（趾）甲增厚、浑浊,呈灰黄色。皮损逐渐向周同扩展,至青春期时已经累及双手整个掌侧、腕部及于背的大部分,双足跖和足背亦呈弥漫性角化过度,跖部较重,皮损呈对称性,     

Progressive palmoplantar keratoderma:a pedigree study

报告1例进行性掌跖角化病及其家系调查结果.先证者男,17岁.掌跖角化性斑块15年.皮肤科检查见双掌、跖部弥漫性角化性斑块,逾越至手背、足背,形成条状角化性斑块,并特征性累及足跟.皮损组织病理检查示表皮棘层及颗粒层肥厚伴显著正角化过度.诊断:进行性掌跖角化病.该家系4代中有4例该病患者(男3例,女1例),属常染色体显性遗传.     

掌跖角皮症伴牙周病1例

患者男,18岁。掌跖红斑和角化18年,伴牙齿脱落12年。掌跖部和踝关节皮肤有对称性大片红斑、鳞屑、角化性增厚和皲裂。切牙和尖牙部分脱落,牙齿有不同程度松动,牙龈萎缩。诊断:掌跖角皮症伴发牙周病。     

砷角化病1例

报道1例砷角化病。患者女，29岁，3年前因治疗银屑病服用游医自制药物，半月后于掌跖部出现点状角化，全身皮肤点状色素沉着及色素减退，共服药半年，皮损逐渐加重。停药2年后，检测尿砷定量高于正常。皮肤科情况：双侧掌跖部多发疣状角化，全身皮肤斑状色素沉着及色素减退，角化部位皮肤活检符合角化病，对症治疗后皮损稍有改善。     

056 在一例日本表皮松解性掌跖角化病患儿中检出角蛋白9基因突变

表皮松解性掌跖角化病(EPPK)是一种常染色体显性遗传性皮肤病,皮损局限于手掌和足跖部以皮肤弥漫性角化过度为特征,典型者边缘呈红斑,组织学上显示掌跖表皮角化过度,伴中上部空泡变性。角蛋白9(K9)属于Ⅰ型角蛋白,仅表达于掌跖部表     

以掌跖部小水疱为皮损表现的扁平苔藓

报告1例以掌跖部小水疱为皮损表现的扁平苔藓。患者女,46岁。因掌跖部红斑、密集厚壁小疱伴瘙痒3周就诊。皮肤科检查:双手掌、手指屈侧、足跖部见轻度角化性红斑,上见密集粟粒至绿豆大水疱,表面光滑呈淡黄色,半透明,疱壁厚不易破,密集不融合,表面干燥无鳞屑,尼氏征阴性;伴口腔、外阴黏膜损害。手掌皮损组织病理改变符合扁平苔藓。复习中英文文献,掌跖扁平苔藓少见,以掌跖部小水疱为皮损表现的扁平苔藓国内外尚无报道。     

阿维A联合复方甘草酸苷治疗掌跖角化性湿疹疗效观察

掌跖角化性湿疹患者皮疹表现为掌部角化过度,有皲裂倾向及红斑.手指的掌侧也可受累,约10%的患者有跖部损害.由于本病皮损角化过度明显,临床上常用方法难以奏效,我们于2008年7月至2009年8月应用小剂量阿维A(重庆华邦制药股份有限公司生产)联合复方甘草酸苷注射液(北京秦武田制药有限公司生产)治疗掌跖角化性湿疹获得满意的疗效,并与单用阿维A治疗进行对比,现报道如下.     

表皮松解性掌跖角化病1例

报告1例表皮松解性掌跖角化病。患者女,26岁。双侧掌跖角化20余年。皮肤科检查见双侧掌跖对称性角化性斑块。皮损组织病理检查示表皮角化过度,颗粒层增厚,棘层及颗粒层中有较多裂隙,裂隙处细胞界限不清,由淡染物质或透明角质颗粒组成。组织病理改变符合表皮松解性掌跖角化病诊断。采用阿维A治疗后皮损明显改善。     

先天性甲肥厚Ⅰ型一家系调查

报告先天性甲肥厚1个家系,2代共有3人患病,3例患者均有甲增厚,掌跖角化,掌跖多汗,局部摩擦可发生水疱和毛发稀疏等典型表现.先证者手、足背弥漫性角化过度,18只甲营养不良。     

先天性厚甲症

先天性厚甲症是一罕见的遗传性外胚叶缺陷性疾病 ,主要表现为甲过度角化和营养不良 ,掌跖角化、多汗 ,毛囊角化及粘膜白斑和多发性脂囊瘤等。发病机制至今尚未完全清楚。现就先天性厚甲症的分型、临床表现、组织学改变和治疗等作简要综述     

Caspase-3在皮肤鳞状细胞癌及光线性角化病中的表达

目的：检测Caspase-3在皮肤鳞状细胞癌及光线性角化病组织中的表达。方法： 应用免疫组化法检测16例皮肤鳞状细胞癌皮损、27例光线性角化病皮损及24例正常皮肤组织中Caspase-3蛋白的表达。结果：Caspase-3在皮肤鳞状细胞癌、光线性角化病及正常皮肤组织的表达率分别为37.50%,51.85%,79.17%,其表达含量在皮肤鳞状细胞癌、光线性角化病、正常皮肤组织逐渐增加。结论：Caspase-3蛋白表达下调可能参与皮肤鳞状细胞癌及光线性角化病的发病过程。     

共聚焦激光扫描显微镜鉴别脂溢性角化病与鲍恩病

目的：探讨共聚焦激光扫描显微镜（CLSM）在脂溢性角化病与鲍恩病鉴别中的应用价值。方法选88例临床诊断为脂溢性角化病和18例临床诊断为鲍恩病的患者典型皮损做CLSM检查,然后取该处皮损行组织病理学检查。结果脂溢性角化病CLSM图像特征：全部有表皮脑回状结构,另有角蛋白充填的囊性包裹体,表皮突呈小梁状下延;基底层细胞排列呈条索状或放射状9例,基底层及真皮层可见折光性明亮的结构6例。鲍恩病CLSM图像特征：表皮中下层细胞灶状排列紊乱,体积较大,细胞形态不规则,有明显的异形,真皮浅层散在单个核细胞浸润。结论脂溢性角化病与鲍恩病在CLSM成像上有不同的特征性,CLSM可为二者的鉴别诊断提供帮助。     

High prevalence of cutaneous human papillomavirus DNA on the top of skin tumors but not in "Stripped" biopsies from the same tumors

Genomes of human papillomaviruses (HPV) are common in biopsies from non-melanoma skin cancers but are also found on healthy skin and it is possible that HPV positivity in tumor biopsies by PCR may merely reflect contamination of the lesion surface. To investigate this issue, 229 immunocompetent patients were tested for HPV DNA in swab samples collected on top of skin tumors and in biopsies of the same tumors, obtained after stripping with tape to remove superficial layers. HPV DNA was detected on top of 69% (159 of 229) of the lesions, and in 12% (28 of 229) of the stripped biopsies (p<0.001). The difference was seen for all four types of tumors studied. Seborrheic keratosis had 79% (34 of 43) HPV positivity on top of lesions versus 19% (eight of 43) in biopsies; actinic keratosis had 83% (38 of 46) HPV positivity on top versus 11% (five of 46) in biopsies; basal cell carcinoma had 63% (69 of 109) on top versus 8% (nine of 109) in biopsies and squamous cell carcinoma had 58% (18 of 31) on top versus 19% (six of 31) in biopsies. HPV DNA is common in superficial layers of lesions, but is not necessarily present throughout tumors.     

UV radiation and keratosis follicularis

We carried out provocation studies on the lesions of keratosis follicularis by the use of UV radiation. Nonerythema-producing doses of UV-B elicited the lesions in uninvolved skin sites in a 34-year-old man with this disease. The elicited lesions were compatible with those of keratosis follicularis both clinically and histopathologically. Similar irradiation with UV-A produced no visible changes in the test area.     

Lewandowsky and lutz dysplasia: report of two cases in a family

Lewandowsky and Lutz dysplasia, also known as epidermodysplasia verruciformis (EV), is an inherited disorder in which there is widespread and persistent infection with human papilloma virus, defect in cell-mediated immunity and propensity for malignant transformation. Differential clinical and histopathologic evolutions of lesions in two cases of familial EV are compared and discussed in detail. Cases were followed up for 7 years. Detailed history, clinical features and investigations, including skin biopsy from different sites at different times, were examined. Generalized pityriasis versicolor like hypopigmented lesions in both the cases, together with variable pigmented nodular actinic keratosis like lesions on sun-exposed areas, were present. Multiple skin biopsies done from various sites on different occasions revealed features typical of EV along with lesions, i.e., actinic keratosis, Bowen's disease, basal and squamous cell carcinoma, in the elder sibling. However, skin biopsy of the other sibling showed features of EV and seborrheic keratosis only till date. This study reveals that the disease progression is variable among two individuals of the same family. Malignant lesions were seen only on sun-exposed areas and may be associated with other skin lesions or infections such as angiokeratoma of Fordyce and tinea cruris, as seen in this report.     

GIANT PEDUNCULATED SEBORRHEIC KERATOSIS OF PENIS

Seborrheic keratosis of the penis is a rare entity. It has been mistaken as genital warts and differentiation is only made on histopathology. We are reporting a case presenting as multiple giant polypoidal lesions on the penile skin for the last 20 years. Seborrheic keratosis should be considered in the differential diagnosis of pedunculated lesions of the penis. The histopathology after shave excision will be diagnostic.     

Imaging actinic keratosis by high‐definition optical coherence tomography. Histomorphologic correlation: a pilot study

With the continued development of non‐invasive therapies for actinic keratosis such as PDT and immune therapies, the non‐invasive diagnosis and monitoring become increasingly relevant. High‐definition optical coherence tomography is a high‐resolution imaging tool, with micrometre resolution in both transversal and axial directions, enable to visualize individual cells up to a depth of around 570 μm filling the imaging gap between conventional optical coherence tomography and reflectance confocal microscopy. We sought to determine the feasibility of detecting and grading of actinic keratosis by this technique using criteria defined for reflectance confocal microscopy compared to histology. In this pilot study, skin lesions of 17 patients with a histologically proven actinic keratosis were imaged by high‐definition optical coherence tomography just before excision and images analysed qualitatively. The surrounding normal looking skin has been used as control group. In lesional skin, dyskeratotic and atypical keratinocytes could be noticed with this new technique. An atypical honeycomb pattern in variable degree or a disarranged epidermal pattern could be observed. A good correlation between the dimension of atypia and/or disarrangement of the spinous–granular layer on en face images and the histopathological grading could be demonstrated. Relevant cross‐sectional imaging criteria could be defined for the different histopathological variants of actinic keratoses. The surrounding skin displayed features of photodamage. Using features already suggested by reflectance confocal microscopy, the study implies that high‐definition optical coherence tomography facilitates in vivo diagnosis of actinic keratosis and allows the grading of different actinic keratosis lesions for increased clinical utility.     

Case-based experience in the use of 5-fluorouracil cream 0.5% as monotherapy and in conjunction with glycolic acid peels for the treatment of actinic keratosis

Abstract

Actinic keratosis, commonly indicative of photodamage, requires treatment secondary to the risk of progression to squamous cell carcinoma. A number of effective treatments for actinic keratosis are available, including topical and lesion-directed therapies. While lesion-directed therapies such as cryotherapy are appropriate for isolated lesions, topical 5-fluorouracil is an effective modality for the treatment of multiple facial actinic keratoses. 5-Fluorouracil, available in a number of formulations, offers patients the benefit of treating subclinical lesions and may help to improve the overall appearance of the skin. In many cases, combination therapy is a better treatment option than monotherapy. The cases presented here demonstrate the use of topical 5-fluorouracil cream 0.5% as monotherapy and in conjunction with glycolic acid peels to treat facial actinic keratoses in two patients with extensive histories of prior actinic keratosis and skin cancer.     

A Case of Seborrheic Keratosis Distributed along Skin Cleavage Lines

A 65-year-old woman with seborrheic keratosis following skin cleavage lines is reported. The mostly brownish-yellow lesions were located on the back. The round, oval, spindle, comet, and slightly raised papules varied from 1 mm to 2 cm in diameter. The distribution of lesions was unusual; they tended to follow skin cleavage lines on her lower back and waist. The arrangement of lesions was streamlined. The skin biopsy specimen revealed hyperkeratosis, acanthosis, and papillomatosis. The acanthosis was caused by proliferation of squamous and basaloid cells.     

Lichenoid keratosis is frequently misdiagnosed as basal cell carcinoma

Lichenoid keratosis (LK), also known as benign lichenoid keratosis or lichen planus‐like keratosis, is a solitary, pink to red‐brown scaly plaque representing a host immunological response to a variety of precursor lesions. LK is often misdiagnosed as a dermatological malignancy owing to its clinical resemblance to basal cell carcinoma (BCC) or Bowen disease. We performed a retrospective analysis of the pathology records of a series of LK lesions with reference to the demographic features and accuracy of clinical diagnosis. The pathology records from 2008 to 2009 of 263 consecutive patients with a histological diagnosis of LK from a specialized skin laboratory were retrieved. Data relating to clinical diagnosis, age, sex, anatomical location, time of year of presentation and any coexistent pathological lesions adjacent to the LK were recorded. Mean age at presentation was 64 years (range 34–96), and 58% of patients were female. The most common anatomical site was the chest/anterior torso, followed by the back and legs. The most common coexisting lesion was solar keratosis at 14%, followed by seborrhoeic keratosis (SK) at 7.8%. The correct clinical diagnosis of LK was made in 29.5% of cases. The most common clinical diagnosis was BCC (47%), while SK was the preferred diagnosis in 18%. A clinical diagnosis was not given in 5.5% of cases. In conclusion, it appears that LK is frequently misdiagnosed, with misdiagnosis occurring in > 70% of cases in this study.