hailey hailey

Question 1

main areas of involvement

lesions

Answer 1

Hailey–Hailey disease, or amilial benign pemphigus, is a rare genodermatosis with dominant inheri- tance that is classically described as a blistering disorder but actually presents as an erythematous, erosive, oozing condition with cracks and ssures localized to the nape o the neck, axillae (Fig. 5-6).
■ Submammaryregions,inguinal olds,and scrotum aremajorsitesofinvolvement.
■ Individual lesions consist o microscopically small accid vesicles on an erythematous background
that soon turn into eroded plaques with the described, highly characteristic, ssured appearance
(Fig. 5-6). Crusting, scaling, and hypertrophic vegetative lesions occur.

Question 2

what genetic abnormality.

undermying pathologic process

Answer 2

The underlying pathologic process is acantholysis whereby the ragility o the epidermis results rom
adeectintheadhesioncomplexbetweendesmosomalproteinsandtono laments.
■ The genetic abnormality lies in ATP2CI, which encodes an ATP-powered calcium pump

Question 3

onset

Answer 3

third and ourth decades

Question 4

tx

Answer 4

Treatment rests on antimicrobial therapy, administered both topically and systemically; systemically,
t h e t e t r a c y c l i n e s s e e m t o w o r k b e t t e r t h a n m o s t . M u p i r o c i n t o p i c a l l y . To p i c a l g l u c o c o r t i c o i d s depresstheanti-in ammatoryresponseandacceleratehealing.Inseverecases,dermabrasionor
c a r b o n d i o x i d e l a s e r v a p o r i z a t i o n l e a d s t o h e a l i n g w i t h s c a r s , w h i c h a r e r e s i s t a n t t o r e c u r r e n c e s . Th e condition becomes less troublesome with age