表现为发疹性瘙痒性丘疹的汗孔角化病

报告1例表现为发疹性瘙痒性丘疹的汗孔角化病.患者男,50岁.臀部丘疹伴剧烈瘙痒7年,逐渐融合成斑块,丘疹突然增多并累及双下肢1年.皮损组织病理检查符合汗孔角化病.给予角质剥脱剂外用及阿维A口服治疗4周,皮损暂无明显改善.     

角化性皮肤病

20 0 13170　生殖器及肛周发疹性瘙痒性丘疹性汗孔角化病 1例 /王耀祖 (上海二医大瑞金医院皮肤科 )…∥临床皮肤科杂志 .- 2 0 0 1,30 (2 ) .- 12 5～ 12 6男 ,4 1岁 ,1984年阴茎出现一绿豆大、褐色、略高于皮面的环状损害 ,无明显自觉症状。 1994年阴囊出现黄豆大圆形红色斑丘疹 ,瘙痒剧烈。以后类似皮损逐渐增多 ,累及肛周 ,治疗效果差。皮肤组织病理 :表皮轻度角化过度 ,灶性角化不全处颗粒层消失 ,表皮凹陷处有角化不全鸡眼样板 ,其下颗粒层部分消失 ,表皮棘层不规则肥厚 ,棘细胞排列不规则 ,细胞有异形 ,并有少量异常核分裂 ,真皮浅层…     

混合型汗孔角化病

报告1例混合型汗孔角化病.患者男,45岁.面部、背部及四肢出现角化性丘疹20余年,腹股沟、外阴及肛周出现结节5年.皮损组织病理检查符合汗孔角化病表现.     

单发于肛周的家族性慢性良性天疱疮1例

家族性慢性良性天疱疮又名Hailey-Hailey病,是不规则显性基因遗传性疾病,临床少见。现将所见１例单发于肛周的家族性慢性良性天疱疮报告如下。1 临床资料 患者女,３０岁,肛周红斑、丘疹伴瘙痒３年。３年前,无明显诱因肛周出现红斑、丘疹,伴瘙痒,搔抓后渗出明显,反复发作,局部皮肤渐增厚.曾多次到各地医院就诊,以“湿疹、乳房外paget病、鲍温样丘疹病、汗管瘤、尖锐湿疣”等病给予相应治疗,未见明显效果。无家族发病史,否认婚外性生活史。体检:系统检查未见异常。皮肤科情况：肛周红斑,表面有灰褐色、灰白色褶皱或角化性丘疹,…     

肛周生殖器部位丘疹性棘层松解性角化不良1例

患者女,41岁。外阴、肛周出现丘疹伴瘙痒2年,无糜烂、疼痛不适。病理检查示:表皮角化过度,棘层肥厚,棘层松解,可见散在角化不良细胞,真皮浅层血管增生,少许淋巴细胞浸润。诊断为:肛周生殖器部位丘疹性棘层松解性角化不良。     

播散性浅表性光线性汗孔角化症1家系调查

患者男,68岁。面颈、躯干及四肢褐色丘疹结节40余年,伴瘙痒1月。皮肤科情况:面、颈、躯干及四肢见大小不等的环形或不规则形角化性褐色斑片,边缘呈线性和堤状隆起,中央萎缩。臀部、肛周及双下肢见角化性结节和疣状增生。家系调查:3代有8人出现类似皮损。皮损组织病理示:角化过度,柱状角化不全,角化不全柱下颗粒层消失。诊断:播散性浅表性光线性汗孔角化症。     

肛周生殖器部位丘疹性棘层松解性角化不良6例临床分析

目的分析肛周生殖器部位丘疹性棘层松解性角化不良的临床及病理特点。方法回顾性分析2013年1月至2022年12月在北京大学第三医院确诊的6例肛周生殖器部位丘疹性棘层松解性角化不良患者的临床及组织病理学资料。结果 6例患者中, 女3例, 男3例, 发病年龄22 ～ 58岁, 发病至确诊时间1 ～ 18个月,均无类似皮损家族史。6例患者临床表现均为肛周或生殖器部位密集分布多发肤色或灰白色、粟粒大小丘疹, 部分表面糜烂、结痂, 可伴瘙痒。组织病理表现类似:角化过度, 灶性角化不全, 表皮内出现棘层松解及角化不良细胞。6例患者外用糖皮质激素或他克莫司软膏等治疗, 疗效不一, 部分皮损可持续存在, 反复发作。结论丘疹性棘层松解性角化不良罕见, 好发于肛周生殖器部位, 诊断时需注意结合临床特征及组织病理。     

外生殖器部位的棘层松解性皮病

报告1例发生于外生殖器部位的棘层松解性皮病.患者女,311.外阴、肛周出现灰白色丘疹6个月,无明显疼痛、瘙痒等不适,部分皮损轻度糜烂、结痂.皮损组织病理检查:表皮灶性角化过度、角化不全,棘层肥厚,棘细胞松解,角化不良.表皮下血管增生,淋巴细胞浸润,直接免疫荧光检查阴性.     

发疹性丘疹型汗孔角化症1例

患者,男,38岁。周身褐色斑疹4年,加重并出现红褐色角化性丘疹伴瘙痒2个月。皮肤科情况:躯干、四肢见2~4 mm红褐色角化性丘疹,密集或散在分布,局部融合,其间散在边缘稍隆起的环状堤状皮疹或萎缩性斑疹。组织病理示:表皮角化过度,棘层增厚,局部见一角化不全柱,其下颗粒层消失,真皮血管周围灶性淋巴细胞浸润。诊断:发疹性丘疹型汗孔角化症。     

播散性浅表光线型汗孔角化症1例

我院发现播散性浅表光线型汗孔 角化症1例,现报告如下。 1病历摘要 患者,男,31岁,以“面部、颈、躯干散在环状角化性褐色小丘疹伴轻度瘙痒10年”来我院就诊。患者10年前无明显诱因先于面颊部出现数个针头大小褐色皮疹,有时伴轻度瘙痒,     

伴炎症反应的汗孔角化病三例

汗管角化症是一种少见的慢性角化性皮肤病,以中央轻度萎缩边缘堤状角质嵴围绕的皮损为特征。组织病理学特征为角样板层。本病一般无临床症状,但伴明显瘙痒的汗孔角化症已经有文献报告,并被命名为“发疹性瘙痒性丘疹型汗管角化症”或“处于炎症反应期的DSP”。笔者近期诊断三例这样的病患并推荐使用“发疹性瘙痒性丘疹型汗管角化症”来命名这一特殊类型的汗孔角化症。     

Porokeratosis ptychotropica: a lesser‐known variant

We report a case of the rare porokeratosis variant porokeratosis ptychotropica (PP). A circumferential perianal plaque and the characteristic histology of multiple cornoid lamellae with underlying dermal amyloid deposition were seen. Amyloid deposition was seen in the biopsied intertriginous area of the plaque only, which, in concordance with other cutaneous amyloid deposition disorders, may suggest a role for friction in the pathogenesis of this histological finding. We review the literature on PP and summarize the poor response seen to treatments.     

Squamous cell carcinoma in a renal transplant recipient with linear porokeratosis.

A 40-year-old man developed squamous cell carcinoma on a perianal lesion of linear porokeratosis after renal transplantation. The tumor metastasized to the left inguinal lymph node 25 months after the primary tumor was excised. p53 overexpression was observed in the tumor cells, but not in the porokeratotic lesion. Interestingly, continuous subcutaneous infusion of peplomycin for the lymph node metastasis significantly improved the warty lesions of porokeratosis. In this patient, immunosuppressive agents might have accelerated the development of carcinoma on a skin area with malignant potential.     

Genitogluteal porokeratosis: an unusual clinical presentation

A 51-year-old man presented with a 12-year history of an expanding, irritable rash on his buttocks, groin and scrotum. He gradually developed erythematous, annular plaques with ridged borders and depressed centres. He also had a verruciform eruption in his perianal area. A clinical diagnosis of porokeratosis confined to the genitogluteal area was confirmed histopathologically. Oral acitretin resulted in symptomatic and cosmetic improvement. He continues to be followed up to evaluate treatment outcomes.     

Eruptive pruritic papular porokeratosis: a pruritic variant of porokeratosis

Porokeratosis is a chronic skin disorder characterized clinically by the presence of crater-like patches with an elevated thick keratotic border and central atrophy. Histology reveals cornoid lamellae. While porokeratosis is practically asymptomatic, a pruritic variant has been reported. We recently encountered an 82-year-old man with pruritic porokeratosis. He presented with erythematous papules and intensively itchy patches on his lower limbs that had been present for 6 months. Histopathological examination revealed the characteristic cornoid lamellae. We describe this case in detail and provide a review of the published work.     

Pediatric penile porokeratosis: A case report

We present what we believe to be the second case of pediatric penile porokeratosis and the youngest case reported. A 6‐year‐old boy presented with a pruritic, verrucous growth at the urethral meatus that recurred after two meatotomies. The diagnosis of porokeratosis was confirmed by biopsy. Porokeratosis should be added to the differential diagnosis of chronic hyperkeratotic penile lesions in children.     

Inflammatory stage of disseminated superficial porokeratosis

Disseminated superficial porokeratosis (DSP) is a keratinization disorder characterized by multiple small lesions with a slightly elevated, sharply defined ridge over the whole body. Unusual DSP cases with acute exacerbation of their lesions accompanied by severe pruritus have been reported and designated as "eruptive pruritic papular porokeratosis" or "inflammatory DSP". Histologically, the pruritic lesions in the majority of these unusual DSP cases had a dense infiltration of eosinophils and lymphocytes in the vicinity of blood vessels in the upper dermis. In this report, we describe an additional case of DSP with a similar clinical course and histopathological findings. A review of the literature showed that the pruritic condition in these unusual DSP cases can be transient and is not necessarily related to spontaneous regression. We propose the term "Inflammatory stage of DSP" for describing this unusual variant of DSP.     

Eruptive Pruritic Papular Porokeratosis

Three cases of an unusual variant of porokeratosis (Mibelli) were described. Patients with disseminated superficial porokeratosis for some years suddenly developed intensively pruritic erythematous papules. Skin biopsies revealed that these papules contained cornoid lamellae on their tops. Pruritic papules subsided in several months, leaving slightly hyperkeratotic brown annular lesions which were shown to contain typical cornoid lamellae histopathologically. This type of porokeratosis has not been reported in the literature.     

Disseminated superficial porokeratosis

A 62-year-old woman with psoriasis and psoriatic arthritis presented for evaluation and treatment of a one-week history of pruritic, pink spots on her trunk and extremities. Several weeks prior, therapy with certolizumab pegol and methotrexate was started for her psoriatic arthritis. A biopsy specimen was consistent with the diagnosis of porokeratosis. Owing to the setting of immunosuppression and presence of symmetric pruritic lesions on non-sun exposed areas, the diagnosis of disseminated superficial porokeratosis was made.