Darier disease

Question 1

caused by loss of function of

Answer 1

Caused by loss-o - unction mutation in the ATP2A2 gene

Question 2

AD?

Answer 2

ad

Question 3

lesions

Answer 3

Arare autosomal-dominant inherited disease with late onset.
■ Multiple discrete scaling, crusted, and pruritic papules mainly in seborrheic and
■ Malodorous and dis guring, also involving nails and mucous membranes.
■ Itchingand/orpainul.
■ Histologically characterized by suprabasal acantholysis and dyskeratosis

Question 4

age

Answer 4

1st- 2nd decade

Question 5

precipitating factors

Answer 5

Frequentlyworsein summer with heat and humidity; also exac- erbated by UVB, mechanical trauma, and bacterialinections

Question 6

dist

Answer 6

Correspondingtothe“seborrheic areas”: chest (Fig. 5-3), back, ears, nasolabial
olds, orehead(Fig.5-4),andthescalp;axilla, neck, and the groin

Question 7

lesionsin palms and sole

Answer 7

Multiple, at, cobblestone- like papules

Question 8

dermatopath

Answer 8

DERMATOPATHOLOGY Dyskeratotic cells in
the spinous layer (corps ronds) and stratum corneum (grains); suprabasal acantholysis and cle s(lacunae);andpapillaryovergrowtho the epidermis and hyperkeratosis

Question 9

tx

Answer 9

Sunscreens, avoidance o riction and rub- bing(turtlenecksweaters),antibiotictherapy (systemicandtopical)tosuppressbacterial
in ection, topical retinoids (tazarotene and ada- palene), or, most ef ective, systemic retinoids (isotretinoin or acitretin).