Epidermal, Appendageal, and Dermal Tumors

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)
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