edidermolytic hyperkeratosis

Question 1

Mutation in what
lesions
tx

Answer 1

Autosomaldominant.Mutationo genesthatencodeepidermaldi erentiationkeratins,KRT1,andKRT10.
■ Presents at or shortly a ter birth with erythroderma and blistering, generalized, or localized.
■ With time becomes keratotic and verrucous (Fig. 4-10) but blisters continue (Fig. 4-10).
■ Shedding o hyperkeratotic masses results in circumscribed areas o normal-appearing skin.
■ Involvement o exural areas and palmar and plantar skin (Fig. 4-11).
■ Associated with unpleasant odor (like rancid butter).
■ Secondary pyogenic in ections.
■ Dermatopathology: Giant coarse keratohyalin granules, vacuolization o granular layer → subcorneal
blisters.
■ Management:Topicalα-hydroxyacids,systemicacitretin,orisotretinointhatinitiallyleadto
increased blister ormation but later improve skin dramatically. Determine dose care ully, monitor sidee ects,andobservecontraindications.