๐ ็ธฝ็ฎ้ ๏ฝ ๐ ่ฑๆๅๆ๏ผๆฌ็ฏ๏ผ ๏ฝ ๐ ๅฎๆด็ฟป่ญฏ ๏ฝ โญ ็ฒพ่ฏ็ญ่จ
EPIDERMOLYTIC ICHTHYOSIS
EPIDERMOLYTIC ICHTHYOSISย ย ICD-10:Q80.8
โข Autosomal dominant. Mutation of genes that encode epidermal differentiation keratins, KRT1, and KRT10.
โข Presents at or shortly after birth with erythema, blistering, erosions, and skin fragility.
โข With time becomes hyperkeratotic (Fig. 4-10) but blisters continue (Fig. 4-10).
โข Shedding of hyperkeratotic masses results in circumscribed areas of normal-appearing skin.
โข Involvement of flexural areas and palmar and plantar skin (Fig. 4-11).
โข Associated with unpleasant odor.
โข Secondary pyogenic infections.
โข Dermatopathology: Giant coarse keratohyalin granules, vacuolization of granular layer โ subcorneal blisters.
โข Management: Topical keratolytics, systemic acitretin, or isotretinoin.
FIGURE 4-10 โข Epidermolytic hyperkeratosis: arms and handsโMountain range-like hyperkeratosis of the dorsum of hands with blistering that results in erosions and shedding of large sheets of keratin.
FIGURE 4-11 โข Distribution of epidermolytic hyperkeratosis.