Focal palmoplantar keratoderma

Question 1

keratotic punctata of palmar creases also referred tonas

Answer 1

Keratotic pits of palmar creases.

common in african descent.

Question 2

Primary lesion of keratosis punctata of palmar creases

Answer 2

1-5 mm depression filled w a comedonlike keratinous plug. localized in creases of palms and fingers .

Question 3

a group of AD palmoplantar keratodermas w streaking hyperkeratosis involving the fingers and extending onto the palm.

Answer 3

Striate keratoderma

Question 4

Striate keratoderma . in some patients have mutation in

Answer 4

Desmolglein 1(DSG-1) or desmoplakin and keratin 1( KRT-1)

Question 5

is a syndrome, is one form w deminished desmosomes, clumping of keratin filaments and enlarged keratohyalin granules.

Answer 5

Brunauer- Fohs Siemens syndrome

Question 6

main symptom of striate keratoderma is

Answer 6

Pruritus. onset bet 15-68 yo.

african predominate. men.

Question 7

Histo for striate kerstoderma

Answer 7

pyknotic, vacuolated epithelium, basal layer spongiosis, dialted occluded sweat ducts, blood vessels and lymph vessels.

Question 8

Acrokeratoelastoidosis presents as

Answer 8

With translucent to erythematous papules at the margins of the palms . both sporadic and AD forms reported. small round firm papules occur over dorsal hands, knuckles, and lateral margins of palms and soles.
asymptomatic most often.

Question 9

Histo for acrokeratoelastoidosis.

Answer 9

dermal elastorrhexis.

Question 10

Porokeratosis plantaris discreta:
age; gender. characterization
tx

Answer 10

Adults
female

char by sharply marginated rubbery wide based papule that on blunt dissection reveals an opaque plug without bleeding on removal. lesions are multiple, painful and usually 7-10mm diameter. confined to weight bearing area of sole beneath metstarsal heads.

tx begin w fitted foot pads to redistribute the weight. surg excision, blunt dissection, cryotx.

Question 11

is an AD and sporadic forms. clinically char by crateriform keratotic papules and plaques along borders of the hands and feet.

Answer 11

Focal acral hyperkeratosis

Question 12

Diffuse palmoplantar keratoderma tx

Answer 12

Poorly responsive to tx.
5% salicylic acid, 12% ammonium lactate and 40% urea.

syst retinoid tx is impractical because of bone toxicity.

Question 13

Vorner and Unna Thost PPK are caused by what mutation

Answer 13

KRT1 and KRT9.

Question 14

which is epidermilytic and non epidermolytic.
Unna thost
Vorner

Answer 14

Vorner- epidermolytic

Unna thost- non epidermolytic

Question 15

Diffuse palmoplantare keratodermas without transgrediens or Vorner and Unna Thost PpK (palmoplantar parakeratosis) manifestation.

Answer 15

Dominantly inherited.
have marked congenital thickening of the epidermal horny layer of palms and soles. usually symmetric and affet all parts equally. there is thick yellow keratin and arches of the feet are generally spared. uniform thickening forms a rigid plate.

Question 16

what do you call the Diffuse palmoplantar kerstodermas w trangrediens

Answer 16

Mal de Meleda type PPK is a rare AR form of PPK

hyperkeratosis does not remain confined to the palms and extensor surfaces of the arms are freq affected.

Question 17

Mal de Meleda ppk has what mutation

Answer 17

SLURP-1 mutation. gene encoding secreted lymphocyte antigen 6/ urokinase type plasminogen activatir receptor related protein 1.

Question 18

tylosis esophageal cancer gene has been localized to what chromosome

Answer 18

chromosome 17q25.

Question 19

descibe the mutilating kerstoderma of Vohwinkel.

Answer 19

Honeycomb palmoplantar hyperkeratosis asoc w startfish like or honeycomb keratosis on the backs of hands and feet, linear keratosis of elbows and knees and annular contriction (pseudo-ainhum) of digits. w may prgress to amputation.

AD.
women
caucasion.

onset in infancy or early childhood.

Question 20

vohwinkel keratoderma without deafness us due to what mutation
with deafness also

Answer 20

w/o deafness- mutation in loricrin and Vohwinkel

deafness- GJB2 (connexin 26)