Chen T-J, Chou Y-C, Chen C-H, Kuo T-T, Hong H-S
Department of Dermatology, Chang Gung Memorial Hospital, Taipei, Taiwan.
Br J Dermatol. 2006 Aug;155(2):325-9. doi: 10.1111/j.1365-2133.2006.07295.x.
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.
To evaluate the clinical manifestations, histopathology, clinical course and treatment response for genital PK.
We reviewed the clinicohistological data from 10 patients with genital PK seen at Chang Gung Memorial Hospital from 1990 to 2005.
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.
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.
汗孔角化症（PK）是一种不常见的表皮角化异常疾病，具有独特的临床表现、病因不明且预后不可预测。生殖器汗孔角化症（在本研究中定义为局限于生殖器区域的局限性汗孔角化症）极为罕见，且文献记载较少。
评估生殖器汗孔角化症的临床表现、组织病理学、临床病程及治疗反应。
我们回顾了1990年至2005年在长庚纪念医院就诊的10例生殖器汗孔角化症患者的临床组织学资料。
7例患者仅生殖器区域有病变，3例患者生殖器及邻近区域受累。所有患者均为男性，初诊时平均年龄为46.0岁（范围36 - 59岁）。除1例患者外，所有患者均有瘙痒性病变。3例患者（30%）患有糖尿病。未观察到恶变。若能进行手术切除，则是最有效的治疗方法。
生殖器汗孔角化症在亚洲人群中比西方国家报告的更为常见。在台湾，生殖器汗孔角化症主要表现为瘙痒性病变，年龄分布广泛。可能需要长期随访。
