Most cases are sporadic, but a familial case, with the condition occurring in a mother and her daughter, has been described. It also has been proposed that activation of an autosomal dominant lethal mutation that survives by mosaicism may be the cause of the lesions. The mutated cells may survive in the due to proximity of normal cells. Another theory is that retrotransposable elements may be the cause of all skin conditions along the Lines of Blashko. Some dogs have a coat variation based upon a similar mechanism. The classification of the disease has much to do with the appearance and location of the lesions (phenotype characteristics). As this is a rare condition, it is possible that more than one genotype could cause a similar phenotype. Different genes have been implicated, but the number of patients studied in each case is very low. There is some evidence that interleukins 1 and 6, tumor necrosis factor α, and intercellular adhesion molecule-1 are upregulated in<unk> , similar to psoriasis.
Inflammatory linear verrucous epidermal nevus
Linear verrucous epidermal nevus
Zosteriform speckled lentiginous nevus
Nodular lichen myxedematosus
Porokeratotic eccrine ostial and dermal duct nevus
Systematized epidermal nevus
Waxy keratosis of childhood
Eccrine nevus