Pityriasis Rubra Pilaris

Question 1

pathogenesis

Answer 1

dysfunction of keratinocytes and a defect in vitamin A, can be autoimmune

Question 2

clinical features

Answer 2

follicular hyperkeratosis on an erythematous base, rough papules on the dorsal aspects of fingers spreads to trunk and extremities, red-orange plaque w island sparing into erythematous plaque that exfoliates

Question 3

palms and soles get

Answer 3

waxy orange red keratoderma

Question 4

nails

Answer 4

thicken, get yellow-brown and subungal debris

Question 5

Type 1 Classic adult pattern

Answer 5

clinical signs as discussed, resolves in 3 years

Question 6

Type 2 - Atypical pattern

Answer 6

5% of all cases, eczematous dermatitis with ichthyosis scale of legs, keratoderma with a coarse scale, alopecia - chronic course

Question 7

Type 3

Answer 7

classic like type 1 but in children ages 2-adolescence, resolves in 3 years w out treatment

Question 8

Type 4

Answer 8

focal type of disease in children with onset prior to puberty, erythema with papules on elbows and knees

Question 9

Type 5

Answer 9

children, variable course, erythema with hyperkeratosis of follicles, scleroderma of hands and feet, familial link

Question 10

Type 6

Answer 10

In HIV patients, follicular hyperkeratosis with HS symptoms, not responding to traditional therapy

Question 11

Pathology

Answer 11

psoriaform derm with hyperkeratosis and checkerboard pattern

Question 12

ddx

Answer 12

scaly scalp mimic seb derm, confused with psoriasis but is distinguished with orange red color and island of sparing, no oil drop spots on nails like in psoriasis

Question 13

treatment

Answer 13

systemic isotretinoin for 3-6 months, acitretin 25mg daily for 3-6 months but has risk of birth defects years after finishing therapy, MTX

Question 14

topical therapies

Answer 14

topical steroids, tar, retinoids, calcineurin inhibitors, vit d analogues

Question 15

unresponsive cases may need

Answer 15

acitretin with MTX