Focal palmoplantar keratoderma Flashcards
keratotic punctata of palmar creases also referred tonas
Keratotic pits of palmar creases.
common in african descent.
Primary lesion of keratosis punctata of palmar creases
1-5 mm depression filled w a comedonlike keratinous plug. localized in creases of palms and fingers .
a group of AD palmoplantar keratodermas w streaking hyperkeratosis involving the fingers and extending onto the palm.
Striate keratoderma
Striate keratoderma . in some patients have mutation in
Desmolglein 1(DSG-1) or desmoplakin and keratin 1( KRT-1)
is a syndrome, is one form w deminished desmosomes, clumping of keratin filaments and enlarged keratohyalin granules.
Brunauer- Fohs Siemens syndrome
main symptom of striate keratoderma is
Pruritus. onset bet 15-68 yo.
african predominate. men.
Histo for striate kerstoderma
pyknotic, vacuolated epithelium, basal layer spongiosis, dialted occluded sweat ducts, blood vessels and lymph vessels.
Acrokeratoelastoidosis presents as
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.
Histo for acrokeratoelastoidosis.
dermal elastorrhexis.
Porokeratosis plantaris discreta:
age; gender. characterization
tx
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.
is an AD and sporadic forms. clinically char by crateriform keratotic papules and plaques along borders of the hands and feet.
Focal acral hyperkeratosis
Diffuse palmoplantar keratoderma tx
Poorly responsive to tx.
5% salicylic acid, 12% ammonium lactate and 40% urea.
syst retinoid tx is impractical because of bone toxicity.
Vorner and Unna Thost PPK are caused by what mutation
KRT1 and KRT9.
which is epidermilytic and non epidermolytic.
Unna thost
Vorner
Vorner- epidermolytic
Unna thost- non epidermolytic
Diffuse palmoplantare keratodermas without transgrediens or Vorner and Unna Thost PpK (palmoplantar parakeratosis) manifestation.
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.
what do you call the Diffuse palmoplantar kerstodermas w trangrediens
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.
Mal de Meleda ppk has what mutation
SLURP-1 mutation. gene encoding secreted lymphocyte antigen 6/ urokinase type plasminogen activatir receptor related protein 1.
tylosis esophageal cancer gene has been localized to what chromosome
chromosome 17q25.
descibe the mutilating kerstoderma of Vohwinkel.
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.
vohwinkel keratoderma without deafness us due to what mutation
with deafness also
w/o deafness- mutation in loricrin and Vohwinkel
deafness- GJB2 (connexin 26)
