Department of Dermatology, NRS Medical College, Kolkata, India.
Indian J Dermatol Venereol Leprol. 2009 Sep-Oct;75(5):551. doi: 10.4103/0378-6323.55427.
Porokeratosis is a disorder of keratinization showing a well-defined lesion with a hyperkeratotic ridge on the border that contains the coronoid lamella. We report familial (autosomal dominant with reduced penetrance) disseminated plaque type (Mibelli's type) porokeratosis in a father and son. In the father, there were multiple horns and a large squamous cell carcinoma in a large lesion over the perianal region that reached up to the squamo-columnar junction of the anal mucosa and even invaded the anal sphincteric muscles. Disseminated lesions of the Mibelli's type, development of horns, and malignancy in this unusual location have not been previously reported.
角化病是一种角化异常的疾病，表现为边界处有一个明确的病变，伴有角蛋白过度角化的嵴，其中包含冠状层。我们报告了一例父亲和儿子患有家族性（常染色体显性遗传，外显率降低）播散性斑块型（米贝利型）角化病。在父亲身上，肛门周围的大病变上有多个角和一个大的鳞状细胞癌，病变甚至延伸到肛门黏膜的鳞柱交界处，甚至侵犯肛门括约肌。米贝利型角化病的播散性病变、角的形成和这个不寻常部位的恶性肿瘤以前没有报道过。
