Epidermal nevus - Skin in Infant/Neonate
Occasionally, epidermal nevi may be associated with other cutaneous, CNS, skeletal, and ocular abnormalities previously known as epidermal nevus syndrome. This entity is now thought to include many distinct genetic diseases, all sharing a phenotype reflecting genetic mosaicism.
Cutaneous skeletal hypophosphatemia syndrome, which has epidermal or melanocytic nevi, elevated fibroblast growth factor-23, and hypophosphatemia, has been found to have a genetic mosaicism of the RAS gene in affected tissues.
D23.9 – Other benign neoplasm of skin, unspecified
239107007 – Epidermal nevus
- Verruca vulgaris – Verrucous papules and plaques, however, do not tend to assume a whorled pattern and do not respect the midline.
- Linear lichen planus
- Epidermal nevi may reflect a somatic mosaicism for K1/K10 mutations as seen in epidermolytic ichthyosis. If these patients have gonadal mosaicism, offspring may have full-blown epidermolytic ichthyosis. Mosaicism for ATP2A2 mutations can cause segmental Darier's disease. As in epidermolytic ichthyosis, if these patients have gonadal mosaicism, offspring may have full-blown Darier's disease.
- Nevus sebaceus – Papillomatous, yellow-orange, linear plaque on the scalp or face.
- Psoriasis – May present in a segmental fashion (nevoid psoriasis) but responds to topical steroids.
- Inflammatory linear verrucous epidermal nevus (ILVEN) – Congenital pruritic, linear, psoriasiform plaque.
- Lichen striatus – Presents in childhood as asymptomatic linearly arranged, small, flat-topped, pink to skin-colored papules within the lines of Blaschko, usually on an extremity. These lesions spontaneously resolve over months to a few years.
- Mosaic Darier's disease – Skin biopsy reveals acantholytic dyskeratosis.