Clinical analysis and etiology of porokeratosis

Abstract

The present study was performed in order to define the clinical manifestations of porokeratosis, with particular emphasis on genital porokeratosis. A total of 55 cases of porokeratosis were retrospectively reviewed between 2000 and 2007 from Huashan Hospital (Shanghai, China). Out of 55 cases, there were 22 cases of porokeratosis of Mibelli, 17 cases of disseminated superficial actinic porokeratosis (DSAP), 15 cases of disseminated superficial porokeratosis and one case of linear porokeratosis. The ratio of males to females was 39:16. Among them, 12 cases had a family history of porokeratosis. During the five-year follow-up period, no malignant transformation was observed and no further aggravation of lesions was detected. The results indicated that the initial region of DSAP in the Chinese population may differ from Caucasians. In combination with other studies, the present study found that genital porokeratosis in the Chinese population is often associated with pruritus. Since no recurrence was observed in cases treated with surgical excision, it was suggested that surgical excision is a viable treatment strategy and should be used for porokeratotic lesions if possible. In addition, regular follow-ups are required, since the aggravation of porokeratosis may cause the development of malignancy transformation.

Porokeratosis Mibelli

“…En sık PM ve DSAP klinik tipleri görülmektedir. 1,5 İnsidansı tam olarak bilinmeyen hastalık, dünyanın her yerinde saptanabilmektedir. 2,4 Porokeratoz her iki cinsiyeti de etkileyebilmekle birlikte, daha sıklıkla erkeklere tesir eden bir hastalıktır.…”

“…2,4 Porokeratoz her iki cinsiyeti de etkileyebilmekle birlikte, daha sıklıkla erkeklere tesir eden bir hastalıktır. 2,3,5 PM daha çok erkeklerde, DSAP ise daha çok kadınlarda görülmektedir. 4,5 PM' de lezyonlar aktinik formlar ile karşılaştırıldığında daha erken yaşlarda başlamaktadır.…”

Porokeratosis of Mibelli: case report of an uncommon penile lesion

“…The most effective treatments for disseminated forms of porokeratosis include topical 5‐fluorouracil, topical retinoids, and topical vitamin D3 analogues . However, for isolated forms, as in this case, the definitive treatment is surgical excision; a retrospective review of seven patients with genital porokeratosis showed no recurrence after 7 years of follow‐up …”

“…There are familial variants that are thought to be autosomal dominant . The predominant symptom is pruritus . Morphologically lesions are characterized by atrophic, spreading, annular patches, surrounded by a ridged hyperkeratotic border…”

Disseminated superficial actinic porokeratosis

“…Education regarding sun protection and regular checks for malignant conditions is needed in patients with DSAP as all of the clinical variants of porokeratosis have the potential for malignant transformation with an overall incidence of 7.5% to 11% [3,5].…”