By Gary M. White, MD

Epidermal nevus syndrome or systematized epidermal nevus

In the epidermal nevus syndrome or systematized epidermal nevus (SEN), epidermal nevi are widespread, often following Blaschko's lines.

Epidermal Nevus Syndromes

As more and more genetic mutations are subdividing these entities, the term epidermal nevus syndromes has been used [Pediatr Dermatol. 2017 Oct 16, ahead of print]. They include:


Wide-spread verrucous lesions in an infant are seen. They may be whorled or linear, and distributed either locally, segmentally, unilaterally or diffusely.

Cutaneous skeletal hypophosphatemia syndrome is the combination of epidermal or melanocytic nevi and hypophosphatemic rickets [JAAD 2016;75;420].


A team approach with an emphasis on finding any systemic abnormalities should be performed. The skin changes are not reliably cleared with any therapy other than simple excision of small lesions. Laser vaporization to the mid dermis can be helpful locally, but significant scarring can also occur. Tretinoin QD combined with 5% 5-FU BID has produced dramatic clearing in one case.

Additional Pictures

Epidermal nevus syndrome or systematized epidermal nevus


A 3-year-old boy presents with widespread warty eruption all over the body including the genitalia but sparing the scalp, face and palms and soles. The eruption began when the child was 3 weeks of age. [Indian J Dermatol. 2015 Jul-Aug; 60(4): 397–399]


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