Systematized epidermal nevus

Systematized epidermal nevus is a type of epidermal nevus that is characterized by the presence of multiple, widespread lesions that follow the lines of Blaschko. These lesions are typically present at birth or develop in early childhood and can vary in size, shape, and color.

Clinical Presentation

Systematized epidermal nevi are often linear or whorled in appearance and can be found on any part of the body. They are usually hyperpigmented and may be raised or flat. The lesions can be isolated or part of a syndrome, such as epidermal nevus syndrome.

Pathophysiology

The condition is caused by a postzygotic mutation in the keratinocytes, leading to a mosaic pattern of skin involvement. The specific genetic mutations can vary, but they often involve genes related to the Ras/MAPK pathway, which is important in cell growth and differentiation.

Diagnosis

Diagnosis is primarily clinical, based on the characteristic appearance of the lesions. A skin biopsy may be performed to confirm the diagnosis and rule out other conditions. Histologically, the lesions show hyperkeratosis, acanthosis, and papillomatosis.

Treatment

Treatment options for systematized epidermal nevus are limited and primarily focus on cosmetic improvement. Options include:

• Laser therapy
• Surgical excision
• Topical treatments such as retinoids

Prognosis

The prognosis for individuals with systematized epidermal nevus is generally good, although the lesions are often persistent and may require ongoing management. In some cases, there may be an association with other systemic abnormalities, necessitating a multidisciplinary approach to care.

Related Pages

• Epidermal nevus
• Epidermal nevus syndrome
• Blaschko's lines
• Keratinocyte