异维A酸治疗播散性浅表性光线性汗孔角化症

患者女,68岁。全身散在褐色角化性丘疹伴瘙痒2年余。皮损组织病理检查示角化过度,表皮增生,柱状角化不全,部分角化不全柱下可见部分角化不良细胞,真皮浅层片状淋巴细胞浸润。全基因组外显子测序提示患者MVK基因突变。诊断:播散性浅表性光线性汗孔角化症,治疗:口服异维A酸软胶囊、抗组胺药和外用维A酸药物3个月余,皮疹逐渐消退,瘙痒缓解,目前患者仍在随访中。     

Genital porokeratosis: a series of 10 patients and review of the literature

BACKGROUND: Porokeratosis (PK) is an uncommon disorder of epidermal keratinization with a unique clinical appearance, unknown aetiology, and an unpredictable outcome. Genital PK (defined as localized PK confined to the genital area in this study) is extremely rare and is not well documented. OBJECTIVES: To evaluate the clinical manifestations, histopathology, clinical course and treatment response for genital PK. METHODS: We reviewed the clinicohistological data from 10 patients with genital PK seen at Chang Gung Memorial Hospital from 1990 to 2005. RESULTS: Seven patients had lesions only in the genital area, and three patients had genital and adjacent areas involved. All patients were male and the mean age at initial diagnosis was 46.0 years (range 36-59). All but one patient presented with pruritic lesions. Three patients (30%) had diabetes mellitus. No malignant transformation was observed. Surgical excision was the most effective treatment if it could be performed. CONCLUSIONS: Genital PK appeared more frequently in the Asian population than in reports from western countries. Genital PK presented mostly as pruritic lesions in Taiwan, with a wide age distribution. Long-term follow-up might be needed.     

Solar facial porokeratosis

Porokeratosis is comprised of a heterogeneous group of disorders that are inherited in autosomally dominant fashion. It has a wide variety of manifestations. In the last ten years, we have treated cases of porokeratosis that don't fit into the well known categories of porokeratosis. For this reason, the present study was carried out. This study included 15 cases whose ages ranged from 19-37 years with a mean +/- SD of 26.93 +/- 4.52. There were 12 females and 3 males. The duration of the disease ranged from 1-10 years with a mean +/- SD of 4 +/- 2.85 years. Their lesions were located only on the face, mostly on the distal part of the nose and the surrounding perinasal area. The number of lesions on each patient ranged from 1-15, with a mean +/- SD of 4.73 +/- 4.35. The morphology of these lesions consisted of papules with a size range from 0.1-1 cm and a warty appearance. They were skin colored and surrounded by a keratotic rim in the form of a collar. The histopathology showed the picture of porokeratosis with cornoid lamellae and additional features characteristic of this disease. We concluded that facial solar porokeratosis is a variant of porokeratosis and is a disease of the nose of young females with a characteristic clinical and histopathological picture.     

Exclusive facial porokeratosis: histopathologically showing follicular cornoid lamellae

Porokeratosis is a heterogeneous group of disorders that are mostly inherited in an autosomal dominant fashion. It has a wide variety of clinical manifestations with a characteristic histological finding of cornoid lamella. Porokeratosis usually affects the trunk or the extremities. Exclusive facial lesions are rare, although 15% of patients with disseminated, superficial, actinic porokeratosis do have facial lesions. Herein, we discuss the case of a 25-year-old woman who noticed the appearance of well-defined, brownish, macular lesions located on both cheeks. She had no other symptoms. A skin examination showed well-demarcated, multiple, brownish macules with raised hyperkeratotic borders on both cheeks and a half-palm-sized, arcuate-shaped lesion formed by grouped macules on the right cheek. No other parts of the body were affected. Histopathological examination of a biopsy specimen obtained from the hyperkeratotic margin gave a diagnosis of porokeratosis with follicular involvement of the cornoid lamellae. Topical pimecrolimus was applied twice a day to the lesions, although little improvement was achieved after 2 months.     

疣状汗孔角化症1例

报告1例疣状汗孔角化症。患者男，53岁。25岁始双下肢出现黄豆大丘疹并逐渐增多、增大、波及全身。臀部和双下肢皮损呈高度疣状增殖性斑块。临床症状和皮损组织病理学改变符合疣状汗孔角化症。给予角质剥脱剂外用及阿维A口服治疗2周后，疣状皮损全部消退。     

Coexistence of congenital linear porokeratosis and disseminated superficial porokeratosis

The coexistence of two or more forms of porokeratosis in a single individual is rarely reported. We report here on a patient exhibiting the coexistence of congenital linear porokeratosis and disseminated superficial porokeratosis. To our knowledge, this entity has been previously reported only once.     

单侧线状型汗孔角化症一例

患者,男,31岁。左侧腰腹部丘疹、斑块31年,破溃、结痂1年。既往以“银屑病”治疗多年效果欠佳,行组织病理学确诊为线状型汗孔角化症。给予阿维A 40mg/d治疗2个月效果欠佳。     

单侧线状型汗孔角化症一例

患者,女,37岁。出生后右大腿外侧出现一角化性丘疹,缓慢扩大呈暗红色斑块并增多,伴堤状隆起,呈线状排列,无家族史。皮肤组织病理检查符合汗孔角化症。     

汗孔角化症合并假性阿洪病1例

报告1例汗孔角化症合并假性阿洪病病例.患者女,16岁,自2岁起,左手背出现褐色角化性丘疹,并逐渐蔓延至左上肢及左手示指及中指,临床症状及组织病理改变符合汗孔角化症,5年前左手中指第一指关节屈侧横沟出现线形缩窄.     

疣状增生性汗孔角化症1例

报告1例具有显著家族史的疣状增生性汗孔角化症患者，多年来皮损表现为播散性浅表性汗孔角化症的典型皮疹，但近两年来腹股沟处出现罕见的疣状损害。结合该病例复习文献进行了讨论。     

偏侧性汗孔角化症1例

报告1例偏侧性汗孔角化症。患者男，49岁，自出生后于左膝关节伸侧出现多个环状褐色斑，逐渐扩大，渐波及左侧躯体，部分皮损为线条状角化性斑块，伴瘙痒。无家族史。皮肤组织病理检查结果符合汗孔角化症。     

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.     

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。     

炎症性浅表播散性汗孔角化症一例

患者,男,64岁。全身褐色环形斑块2年,伴瘙痒2个月。组织病理示：表皮角化不全,角化过度,可见角化不全柱,其下方棘层散在角化不良细胞,基底层灶状液化变性,真皮见团块状淋巴细胞为主的炎细胞浸润。诊断：炎症性浅表播散性汗孔角化症。给予阿维A 30 mg/d口服治疗,皮损部分消退,目前随访中。     

Drugs associated with development of porokeratosis: A systematic review

Porokeratosis is a rare disorder characterized by atrophic macules or patches, with a well‐defined ridge‐like hyperkeratotic border called cornoid lamella. Although the exact pathogenesis is unknown, drug associated cases have recently been reported in the literature. As such, we systematically reviewed and identified drugs associated with drug‐induced porokeratosis, their resultant effects, and whether there was a casual relationship between the use of a drug and the development of porokeratosis. We searched for articles which reported drug‐induced porokeratosis in MEDLINE and Embase in June 2020. After full‐text review, 25 studies were included for analysis. We identified 26 patients with drug‐induced porokeratosis. The most common therapies associated with development of porokeratosis is biologic use, phototherapy, and radiotherapy. The most common clinical variants were the disseminated superficial or actinic types (60%), which occurred in psoriasis patients undergoing phototherapy, and eruptive disseminated type (24%) which occurred in the context of biologic therapies. The Naranjo score ranged from possible to probable for the identified treatments. Clinicians should consider drug reactions as possible triggering events for porokeratosis, especially for patients taking biologics, phototherapy, and radiotherapy. Large‐scale studies are required to confirm our findings and further explore the pathogenesis for drug‐induced porokeratosis.     

毛囊性汗孔角化病一例

患者男,30岁,因左胫部褐红色斑块覆鳞屑3年余于2016年7月来我院就诊。患者3年前发现左胫部出现约壹元硬币大小褐红色斑块,其上覆鳞屑,无瘙痒、疼痛等症状。发病前无明显诱因。曾在外院就诊,诊断为“湿疹”,外用他克莫司无效,皮损逐渐扩大。1年前右腿出现黄豆大小类似皮损,伴瘙痒,就诊于多家医院,均按湿疹治疗（具体用药不详）无效。既往体健,否认慢性病史,家中无类似病史……     

Treatment of disseminated superficial actinic porokeratosis with topical diclofenac gel: a case series

Disseminated superficial actinic porokeratosis (DSAP) is the most common of the of five clinical variants of porokeratosis. These are disorders of keratinization and the distinctive pathological feature is the cornoid lamella at the margin. DSAP usually manifests in the third or fourth decades of life with a female preponderance and with multiple lesions over sun‐exposed areas. A diverse range of treatments is employed though evidence of efficacy remains largely anecdotal. We report a series of eight patients with DSAP treated with 3% diclofenac gel (Solaraze® gel).     

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.     

A case of the hyperkeratotic variant of porokeratosis Mibelli

Herein, we report a case of the hyperkeratotic variant of porokeratosis Mibelli. A 58-year-old woman presented with two brownish verrucous plaques on the buttock for 20 years, and extremely hyperkeratotic verrucous nodules and brownish macules on the right foot for 1 year. Histopathological findings of the buttock were typical of porokeratosis. However, histopathological findings of the right foot were atypical for porokeratosis, including extreme hyperkeratosis and an increased number of and unusual sized cornoid lamellae.