4 - 29 - PITYRIASIS RUBRA PILARIS

Question 1

most common subtype and occurs in more than 50% of all cases

Answer 1

Type I (classic adult)

Question 2

diagnostic hallmark of PRP

Answer 2

sharply demarcated islands of unaffected skin (“nappes claires”)

Question 3

It is characterized by its atypical morphologic picture and a long duration of more than 20 years.

Answer 3

Type II (atypical adult)

Question 4

clinical picture resembles ichthyosiform scaling, areas of follicular hyperkeratosis, and sparseness of the scalp hair

The cephalocaudal progression observed in type I is missing, and there is less tendency for the patients to develop erythroderma.

Answer 4

Type II (atypical adult)

Question 5

affects 10% of PRP patients, with the onset usually between the ages of 5 and 10 years

Answer 5

Type III (classic juvenile)

Question 6

This type usually manifests in prepubertal children and young adults

Answer 6

Type IV (circumscribed juvenile)

Question 7

characterized by well-demarcated hyperkeratotic erythematous plaques on the elbows and knees, resembling localized psoriasis

Answer 7

Type IV (circumscribed juvenile)

Question 8

Palmoplantar keratoderma is characteristic for this type of PRP

Answer 8

Type IV (circumscribed juvenile)

Question 9

characterized by an early age of onset and a chronic course

Answer 9

Type V (atypical juvenile) PRP

Question 10

This type harbor a gain-of-function mutation in the CARD14 gene encoding the Caspase Recruitment Domain family member 14 (CARD14)

Answer 10

Type V (atypical juvenile) PRP

Question 11

**Some patients present with scleroderma-like features affecting hands and feet.

Answer 11

Type V (atypical juvenile) PRP

Question 12

This type is associated with acne conglobata, hidradenitis suppurativa, and lichen spinulosus

Answer 12

type VI (HIV-associated)

Question 13

Often resolves within an average of 3 years

Answer 13

Type I (classic adult)

Question 14

The only type of PRP presenting with localized distribution

Answer 14

Type IV ( Circumscribed Juvenile)

Question 15

Similar to type I but appears in years 1 or 2 of life

Answer 15

Type III (Classic Juvenile)

Question 16

most familial cases belong to this type

Answer 16

Type V (Atypical Juvenile)

Question 17

symmetrically distributed lesions of the extensor surfaces frequently progress to erythroderma

Answer 17

Type VI (HIV-associated)

Question 18

Characteristic histopath findings of PRP

Answer 18

alternating horizontal and vertical parakeratosis and orthokeratosis in a checkerboard pattern, hypergranulosis, thickening of the rete ridges, follicular hyperkeratosis, lack of neutrophilic infiltration, and limited vascular dilatation

Question 19

First-line topical Meds for PRP

Answer 19

■ Emollients (water-in-oil emulsion)

■ Keratolytics (salicylic acid, urea)

■ Vitamin D 3 (calcipotriol)

Question 20

generally serve as the first-line therapy in patients with PRP

Answer 20

Oral retinoids

Question 21

first line systemic medications for PRP

Answer 21

■ Retinoids (0.5-0.75 mg/kg acitretin/day)

■ Methotrexate (10-25 mg weekly or higher)

■ Antiretroviral therapy (HIV-associated variant)

Question 22

Second line treatment for PRP

Answer 22