Dominant ichthyosis vulgaris

Question 1

lesions
prediliction sites
assoc w what disease

Answer 1

■ Characterized by usually mild generalized xerosis with scaling, most pronounced on lower legs; in severe cases large, tessellated scales.
■ Hyperlinear palms and soles.
■ Peri ollicular hyperkeratosis (keratosis pilaris) usually on arms and legs.
■ Frequently associated with atopy.

Question 2

age

gender

Answer 2

AGEOFONSET 3to12months.

SEX Equal incidence in males and emales. Autosomal dominant inheritance. INCIDENCE Common (1 in 250).

Question 3

pThogenesis. what component is reduced

Answer 3

Etiologyunknown.T ereisreducedorabsent laggrin. Epidermis proli erates normally but
keratin is retained with a resultant thickened stratum corneum.

Question 4

lesions.
site of prediiliction
what area is spared?

Answer 4

Xerosis (dry skin) with ne, powdery scaling but also larger, rmly adher- ent tacked-down scales in a sh-scale pattern (Figs.4-1and4-2).Di usegeneralinvolve- ment, accentuated on the shins, arms, and back, buttocks, and lateral thighs; axillae and the antecubital and popliteal ossae spared

T e aceisusuallyspared butthecheeksand oreheadmaybeinvolved

Question 5

DP

Answer 5

DERMATOPATHOLOGY Compact hyperkeratosis; reduced or absent granular layer; small, poorly
ormed keratohyalin granules by electron microscopy, germinative layer f attened

Question 6

Tx

Answer 6

HYDRATIONOFSTRATUMCORNEUM Immersionin abath ollowedbytheapplicationo petrola- tum. Urea-containing creams bind water in the stratum corneum

KERATOLYTICAGENTS Propyleneglycol– glycerin–lactic acid mixtures. Propylene glycol (44 to 60% in water); 6% salicylic acid in propylene glycol and alcohol, used under plasticocclusion(bewareo hypersalicism). α -Hydroxy acids (lactic acid or glycolic acid) control scaling. Urea-containing creams and lotions(2to10%)aree ective. SYSTEMICRETINOIDS Isotretinoin and acitretin are very e ective, but care ul monitoring or toxicityisrequired.Onlyseverecasesmay require intermittent therapy.