• | a group of heterogenous disorders having in common a distinctive histopathologic structure |
• | risk of malignant degeneration: higher in variants with larger, chronic lesions; much lower in DSAP |
• | at least 4 clinical forms: |
Porokeratosis of Mibelli
• | autosomal dominant or sporadic |
• | pathognomonic sign = “the Great Wall of China” |
Disseminated superficial actinic porokeratosis (DSAP)
• | during summer, lesions may become more erythematous and hyperkeratotic |
• | predominantly sun-exposed sites; especially legs |
• | no evidence of skin cancer arising (in one large study) |
• | tx: 5-FU, cryotherapy, or topical retinoids |
Porokeratosis palmaris et plantaris
• | autosomal dominant; rare |
Linear porokeratosis
|