PRP- Pityeiasis Rubra Pilaris

Question 1

is a chronic skin disease char by small follicular ppules and coalesce into salmon colored pink scaling patches and often solid confluent yellow orange palmoplantar hyperkeratosis.

Answer 1

PRP

Question 2

Most important diagnostic feature

Answer 2

Papules . being more or less acuminate (pointy) , salmon colored to reddish brown about pinhead sized and topped by a central horny center.

Question 3

Two peaks of age

Answer 3

first 5 yrs of life or in 50s

Question 4

are they symmetric and diffuse w char small islands of normal skin within the affected areas? Hyperkeratosis of palms and soles?

Answer 4

Yes

Question 5

Griffith’s classification.

Answer 5

I- Classic adult type. most often seen. carries a good prognosis with 80% involuting over an3 year period.
Type 2- atypical adult type
III- Classic juvenile type. clearing of the disease within 1 yr although it may recur even into adulthood.
4- Circumscribed juvenile type. Poorer prognosis for spont recovery.
5- infancy or early childhood. has a more chronic course. assoc w CARD14 mutation.
6- HIV

Question 6

histo:

Answer 6

hyperkeratosis
follicular plugging
focal parakeratosis at the follicular orifice.

PRP- HFP

Question 7

tx

Answer 7

Systemic retinoids.
topical tazarotene.
Isotret may induce prolonged remissions or cures. may take 6-9 months for full involution.
acutretin and methotrexate
ustekinumab - familial PRP for card14 mut.
topical calc inh, lactic acid, urea cont prep.