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

Question 23

Characterize Type I (classic adult PRP)

Answer 23

• Characteristically, type I PRP starts with erythematous macules forming patches and with follicular hyperkeratotic papules on the upper half of the body.
• As the disease evolves, a yellow-orange, scaling dermatitis often spreads to a generalized erythroderma over a period of 2 to 3 months

Question 24

Frequently patients develop a waxy, diffuse, yellowish keratoderma of the palms and soles

Answer 24

Type I (classic adult)

Question 25

Nail changes are common in these patients and include** nail plate thickening, splinter hemorrhages, and subungual hyperkeratosis**

Answer 25

Type I (classic adult) ## Footnote In patients with uniform facial involvement, ectropion frequently develops

Question 26

course of Type I (classic adult)

Answer 26

Often resolves within an average period of 3 years

Question 26

Type I (classic adult) occurs in more than how many percent of patients?

Answer 26

>50%

Question 27

distribution of type I PRP

Answer 27

Generalized, beginning on the head and neck, then spreading caudally

Question 28

how many % of patients present with Type II PRP?

Answer 28

5%

Question 29

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

Answer 29

Type II (atypical adult) PRP

Question 30

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

Answer 30

Type II (atypical adult) PRP

Question 31

Difference of type II vs type I PRP

Answer 31

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

Question 32

clinical counterpart of type I PRP in children

Answer 32

Type III (classic juvenile)

Question 33

Type III (classic juvenile) affects how many % of patients?

Answer 33

10%

Question 34

age of onset of Type III (classic juvenile) PRP

Answer 34

onset usually between the ages of 5 and 10 years ## Footnote table 29-1: appears in years 1 or 2

Question 34

clinical course of Type III PRP

Answer 34

often resolves within an average period of 1 - 2 years

Question 35

Type IV (circumscribed juvenile) affects approximately how many % of patients

Answer 35

25%

Question 36

This type usually manifests in prepubertal children and young adults

Answer 36

Type IV (circumscribed juvenile) PRP

Question 37

Palmoplantar keratoderma is characteristic for what type of PRP?

Answer 37

Type IV (circumscribed juvenile) | but may also be absent ## Footnote can also be seen in Type I classic adult

Question 38

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

Answer 38

Type IV (circumscribed juvenile) PRP ## Footnote These lesions do not progress to the more widespread classical type.

Question 39

uncertain clinical course; some cases clear in the late teens

Answer 39

Type IV (circumscribed juvenile) PRP ## Footnote The 3-year remission rate is 32%.

Question 40

the only localized form of PRP

Answer 40

Type IV (circumscribed juvenile) PRP

Question 41

occurs in 5% of patients and is characterized by an early age of onset and a chronic course.

Answer 41

Type V (atypical juvenile) PRP

Question 42

Most patients of the familial PRP belong to this category

Answer 42

Type V (atypical juvenile) PRP

Question 43

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

Answer 43

Type V (atypical juvenile) PRP

Question 44

clinical course of Type V (atypical juvenile) PRP

Answer 44

chronic course; improvement with retinoids but relapses when stopped

Question 45

what are the 3 additional manifestations of type VI (HIV-associated) variant of PRP?

Answer 45

acne conglobata, hidradenitis suppurativa, and lichen spinulosus

Question 46

clinical course of type VI (HIV-associated) variant of PRP

Answer 46

may respond to antiretroviral triple therapy

Question 47

diffrentiate PRP from psoriasis

Answer 47

* PRP often affects the face, * has the typical salmon-colored appearance, * presents with classical islands of healthy skin over the trunk, * distinct areas of follicular hyperkeratosis, and a * waxy palmoplantar keratoderma * Although psoriasis - associated nail changes are missing, nail plates may be hypertrophic

Question 48

what features of psoriasis in histopath are not seen in PRP?

Answer 48

The histologic picture of psoriasis with hypogranulosis, elongation of the rete ridges, vascular dilation, a high frequency of neutrophils manifesting as intraepidermal Munro microabscesses is not shared with PRP

Question 49

This type of PRP has little or no tendency to resolve spontaneously.

Answer 49

Type V PRP