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.
Highlights
Linear porokeratosis (LP), punctate porokeratosis (PP), disseminated palmoplantar porokeratosis (DPP) and genital porokeratosis are uncommon disorders resulting from epidermal keratinization
Patients were classified into four clinical variants: i) 22 cases of Porokeratosis of Mibelli (PM); ii) 17 cases of disseminated superficial actinic porokeratosis (DSAP); iii) 15 cases of disseminated superficial porokeratosis (DSP); and iv) one case of LP
The number of uninherited cases with PM and DSP was significantly higher than inherited cases (Fig. 1), no difference was observed in the DSAP cases (Fig. 1)
Summary
Linear porokeratosis (LP), punctate porokeratosis (PP), disseminated palmoplantar porokeratosis (DPP) and genital porokeratosis are uncommon disorders resulting from epidermal keratinization. Porokeratosis of Mibelli (PM), disseminated superficial porokeratosis (DSP) and disseminated superficial actinic porokeratosis (DSAP) are more common subtypes of porokeratosis, from which an increasing number of people suffer. Porokeratosis includes several clinical variants with a wide range of clinical presentations, which all have histologically in common the presence of a cornoid lamella. According to the distribution of the lesion, porokeratosis may be classified into two types, the localized variants, including PM, LP and PP, and the extensive variants, including DSP, DSAP and DPP [1]. Certain mutations that are associated with porokeratosis, including frameshift mutations, have been identified [2,3]. These events are confined to certain pedigrees [4]. There have been a number of case reports, no more than 31 cases have been investigated individually
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