Andrei 29

Question 1

is a rare, inflammatory skin disease of juvenile or adult onset with distinctive clinical features and a self-limiting or chronic evolution.

Answer 1

Pityriasis rubra pilaris (PRP)

Question 2

___ is the most common subtype and occurs in more than___ of all cases. Character- istically, type I PRP starts with erythematous macules forming patches and with follicular hyperkeratotic papules on the upper half of the body

Answer 2

Type I (classic adult) 
 ;50%

Question 3

As the disease evolves, a yellow-orange, scaling dermatitis often spreads to a generalized erythroderma over a period of _to _months

Answer 3

2 to 3

Question 4

A diagnostic hallmark of PRP is the presence of _____

Answer 4

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

Question 5

Type II (atypical adult) PRP describes an variant, which affects ___of PRP patients. It is characterized by __& __.The clinical picture resembles ichthyosiform scaling, areas of follicular hyperkeratosis, and sparseness of the scalp hair.

Answer 5

5% ; its atypical morphologic picture and a long duration of more than 20 years.

Question 6

The__ observed in type I PRP is missing in type II, and there is less tendency for the patients to develop erythroderma

Answer 6

cephalocaudal progression

Question 7

___is the clinical counter- part of type I PRP in children. It affects 10% of PRP patients, with the onset usually between the ages of___ . The clinical course may be shorter with clearing after_ year

Answer 7

Type III (classic juvenile) ; 5 and 10 years; 1

Question 8

Type IV (circumscribed juvenile) affects approximately 25% of patients. This type usually manifests in __ and __. It is characterized by ___

Answer 8

prepubertal children and young adults. ; well-demarcated hyperkeratotic erythematous plaques on the elbows and knees, resembling localized psoriasis

Question 9

T/F: These lesions (type IV) progresses to the more widespread classical type.

Answer 9

F; do not.

Question 10

__is characteristic for type IV PRP, but maY also be absent. The 3-year remission rate is __

Answer 10

Palmoplantar keratoderma; 32%

Question 11

Type V (atypical juvenile) PRP occurs in 5% of patients and is characterized by__ and ___.

Answer 11

an early age of onset and a chronic course.

Question 12

Most patients of the familial PRP belong to this type__ PRP.

Answer 12

V;

Question 13

Type V prp were recently shown to harbor a gain-of-function mutation in the ___ gene.

Answer 13

CARD14

Question 14

It manifests as hyperkeratotic follicular lesions. Some patients present with scleroderma-like features affecting hands and feet.

Answer 14

Type V prp

Question 15

___variant manifest with follicular papules and prominent follicular plugging. frequently progress to___. Additional manifestations include __, __ &__

Answer 15

The type VI (HIV-associated) ; erythroderma; acne conglobata, hidradenitis suppurativa, and lichen spinulosus.

Question 16

Among adaptive T-cell cytokines, an increase of TH1 cytokines and, in particular,__ cytokines IL-__,__and __was seen

Answer 16

TH17, 17A, IL-17F, and IL-22

Question 17

PRP often affects the__, 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

Answer 17

face

Question 18

PRP is typically a clinical diagnosis based on the __ and __

Answer 18

characteristic skin lesions and the age of onset.

Question 19

Classic adult type I PRP frequently resolves within_ years.

Answer 19

3

Question 20

type III commonly resolves within _ to __

Answer 20

1-2 years

Question 21

less-favorable prognosis for remission is reported for the atypical variants_ and _ although some cases of __improve in the patient’s late teens

Answer 21

types II and IV,; type IV

Question 22

which is associated with CARD14 mutations, has little or no tendency to resolve spontaneously.

Answer 22

Type V PRP,

Question 23

T/f : Local treatment options for PRP regularly do not suffice to control the disease and are used as an adjunct to systemic therapy

Answer 23

T

Question 24

percutaneous resorption of vitamin D analogs restricts treatment to no more than__ of body surface.

Answer 24

30%

Question 25

___serve as the first-line therapy in patients with PRP. They block the proliferation of keratinocytes and reduce hyperkeratosis. Acitretin and isotretinoin doage of ___ are effective;

Answer 25

Oral retinoids; (1-2 mg/kg/day)

Question 26

oral retinoids can induce __ and should be used with caution.

Answer 26

premature closure of epiphyses or hyperostosis,

Question 27

Teratogenicity is also a known adverse effect of __the second most used drug for the treatment of PRP. I

Answer 27

methotrexate,

Question 28

T/f PRP can be aggravated by UV light

Answer 28

T

Question 29

ustekinumab was effective in a number of patients with PRP type I, in a patient with a CARD14 mutation (type V),but not in a patient with type__ PRP.

Answer 29

IV

Question 30

To dampen the local inflammatory response,__ are often initially employed and may be followed by topical calcineurin inhibitors.

Answer 30

topical corticosteroids