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Epidermal Nevus (EN)


Epidermal Nevus (EN) (Figure 2.11C)
  • Figure 2.11 A: Chronic bullous disease of childhood (Courtesy of Dr. Michelle B. Bain) B: Hailey–Hailey disease (Courtesy of Dr. Paul Getz) C: ILVEN (Courtesy of Dr. Paul Getz)
    Figure 2.11
    A: Chronic bullous disease of
    childhood
    (Courtesy of Dr. Michelle
    B. Bain    )
    B: Hailey–Hailey disease
    (Courtesy of Dr. Paul Getz)
    C: ILVEN
    (Courtesy of Dr. Paul Getz)
    Hamartoma of epidermis and papillary dermis; onset typically at birth (± adolescence, rare in adulthood)
  • Presents as hyperpigmented papillomatous papules and plaques along lines of Blaschko
  • Ichthyosis hystrix: extensive bilateral systematized lesions
  • ILVEN (inflammatory linear verrucous epidermal nevus): erythematous scaly plaque along lines of Blaschko; not associated with any neurologic defects
  • Epidermal nevus syndrome (Schimmelpenning syndrome): sporadic; epidermal nevus, underlying CNS, ocular, cardiac, and skeletal defects, biopsy to r/o epidermolytic hyperkeratosis (EHK)
   
 
Of note, if biopsy of EN shows EHK, the patient may be at risk with offspring with full-blown EHK
 
   

 

 

 

 

 

 

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