pityriasis rubra pilaris

Question 1

manif and tx

Answer 1

Rare,chronic,papulosquamousdisorderotenprogressingtoerythroderma.
■ Sixtypesexist.
■ Follicular hyperkeratotic papules, reddish-orange progressing to generalized erythroderma. Sharply
demarcatedislandso una ected(normal)skin.
■ Waxy,di use,orangekeratodermao thepalmsandsoles;thenailsmaybea ected.
■ Most e ective therapy is MTX, systemic retinoids

Question 2

classification 6

Answer 2

Type 1: Classic Adult. Generalized, beginning on head and neck.
Type 2: Atypical Adult. Generalized, sparse hair.
Type 3: Classic Juvenile. Appears within the
rst2yearso lie,generalized
Type 4: Circumscribed Juvenile- prepubertal , localized

Type 5: Atypical Juvenile. Onset in
rst ew
years o li e, amilial, generalized.
Type 6: HIV-Associated. Generalized, as-
sociated with acne conglobata, hidradenitis suppurativa, and lichen spinulosus

Question 3

manif

Answer 3

Both insidious and rapid onset occur.
SKIN LESIONS All types o PRP. An eruption o
ollicularhyperkeratoticpapuleso reddish- orange color usually spreading in a cephalo- caudaldirection

Distribution. ypes 1, 2, 3, 5, and 6: General- ized, classically beginning on the head and neck, then spreading caudally

Question 4

nail lesion

Answer 4

NAILS Common but not diagnostic. Distal yellow-brown discoloration, nail plate thicken- ing, subungual hyperkeratosis, and splinter hemorrhages

Question 5

hp

Answer 5

Hyperkeratosis, acanthosis with broad short rete ridges, alternating orthokeratosis, and parakeratosis. Keratinous plugs o ol- licular in undibula and peri ollicular areas o parakeratosis. Prominent granular layer may distinguishPRP rompsoriasis.Super cial perivascularlymphocyticin ltrate

Question 6

prog

Answer 6

A socially and psychologically disabling condi- tion.Longduration;type3o enresolvesa er 2 years; type 4 may clear. ype 5 has a very chronic course. ype 6 may respond to highly activeantiretroviraltherapy(HAAR ).

Question 7

tx

Answer 7

emollients, keratolytic agents, vitamin D3 (calcipotriol

Moste ectivetreatmentconsistso systemic administration o M X or retinoids (both as in psoriasis). In type 6: HAAR . T e anti- NF agents, e.g., in iximab and etaner- ceptaree ective.