EPIDERMAL NEVUS, SYSTEMATIZED
By Gary M. White, MD
In the epidermal nevus syndrome or systematized epidermal nevus (SEN), epidermal nevi are widespread, often following Blaschko's lines.
- Systemic abnormalities are very common and may affect the musculoskeletal, ocular and neurologic systems.
- The term ichthyosis histrix has been used for SEN. SEN may involve one half of the body and has been called nevus unius lateris.
- SEN appears to be stem from a post-zygotic mosaic mutation in RAS genes.
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:
- Nevus sebaceous,
- Keratinocytic epidermal nevus
- Nevus comedonicus
- Congenital hemidysplasia with ichthyosiform nevus and limb defects syndrome (CHILD Syndrome)
- Phakomatosis pigmentokeratotica
- Becker's nevus
- Porokeratotic adnexal ostial nevus
- Inflammatory linear verrucous epidermal nevus
- Cutaneous-skeletal hypophosphatemia syndrome
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.
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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