4 - 29 - PITYRIASIS RUBRA PILARIS Flashcards
most common subtype and occurs in more than 50% of all cases
Type I (classic adult)
diagnostic hallmark of PRP
sharply demarcated islands of unaffected skin (“nappes claires”)
It is characterized by its atypical morphologic picture and a long duration of more than 20 years.
Type II (atypical adult)
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.
Type II (atypical adult)
affects 10% of PRP patients, with the onset usually between the ages of 5 and 10 years
Type III (classic juvenile)
This type usually manifests in prepubertal children and young adults
Type IV (circumscribed juvenile)
characterized by well-demarcated hyperkeratotic erythematous plaques on the elbows and knees, resembling localized psoriasis
Type IV (circumscribed juvenile)
Palmoplantar keratoderma is characteristic for this type of PRP
Type IV (circumscribed juvenile)
characterized by an early age of onset and a chronic course
Type V (atypical juvenile) PRP
This type harbor a gain-of-function mutation in the CARD14 gene encoding the Caspase Recruitment Domain family member 14 (CARD14)
Type V (atypical juvenile) PRP
**Some patients present with scleroderma-like features affecting hands and feet.
Type V (atypical juvenile) PRP
This type is associated with acne conglobata, hidradenitis suppurativa, and lichen spinulosus
type VI (HIV-associated)
Often resolves within an average of 3 years
Type I (classic adult)
The only type of PRP presenting with localized distribution
Type IV ( Circumscribed Juvenile)
Similar to type I but appears in years 1 or 2 of life
Type III (Classic Juvenile)
most familial cases belong to this type
Type V (Atypical Juvenile)
symmetrically distributed lesions of the extensor surfaces frequently progress to erythroderma
Type VI (HIV-associated)
Characteristic histopath findings of PRP
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
First-line topical Meds for PRP
■ Emollients (water-in-oil emulsion)
■ Keratolytics (salicylic acid, urea)
■ Vitamin D 3 (calcipotriol)
generally serve as the first-line therapy in patients with PRP
Oral retinoids
first line systemic medications for PRP
■ Retinoids (0.5-0.75 mg/kg acitretin/day)
■ Methotrexate (10-25 mg weekly or higher)
■ Antiretroviral therapy (HIV-associated variant)
Second line treatment for PRP
Characterize Type I (classic adult PRP)
- 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
Frequently patients develop a waxy, diffuse, yellowish keratoderma of the palms and soles
Type I (classic adult)
Nail changes are common in these patients and include** nail plate thickening, splinter hemorrhages, and subungual hyperkeratosis**
Type I (classic adult)
In patients with uniform facial involvement, ectropion frequently develops
course of Type I (classic adult)
Often resolves within an average period of 3 years
Type I (classic adult) occurs in more than how many percent of patients?
> 50%
distribution of type I PRP
Generalized, beginning on the head and neck, then spreading caudally
how many % of patients present with Type II PRP?
5%
It is characterized by its atypical morphologic picture and a long duration of more than 20 years.
Type II (atypical adult) PRP
The clinical picture resembles ichthyosiform scaling, areas of follicular hyperkeratosis, and sparseness of the scalp hair.
Type II (atypical adult) PRP
Difference of type II vs type I PRP
The cephalocaudal progression observed in type I is missing, and there is less tendency for the patients to develop erythroderma.
clinical counterpart of type I PRP in children
Type III (classic juvenile)
Type III (classic juvenile) affects how many % of patients?
10%
age of onset of Type III (classic juvenile) PRP
onset usually between the ages of 5 and 10 years
table 29-1: appears in years 1 or 2
clinical course of Type III PRP
often resolves within an average period of 1 - 2 years
Type IV (circumscribed juvenile) affects approximately how many % of patients
25%
This type usually manifests in prepubertal children and young adults
Type IV (circumscribed juvenile) PRP
Palmoplantar keratoderma is characteristic for what type of PRP?
Type IV (circumscribed juvenile)
but may also be absent
can also be seen in Type I classic adult
It is characterized by well-demarcated hyperkeratotic erythematous plaques on the elbows and knees, resembling localized psoriasis
Type IV (circumscribed juvenile) PRP
These lesions do not progress to the more widespread classical type.
uncertain clinical course; some cases clear in the late teens
Type IV (circumscribed juvenile) PRP
The 3-year remission rate is 32%.
the only localized form of PRP
Type IV (circumscribed juvenile) PRP
occurs in 5% of patients and is characterized by an early age of onset and a chronic course.
Type V (atypical juvenile) PRP
Most patients of the familial PRP belong to this category
Type V (atypical juvenile) PRP
Some patients present with scleroderma-like features affecting hands and feet.
Type V (atypical juvenile) PRP
clinical course of Type V (atypical juvenile) PRP
chronic course; improvement with retinoids but relapses when stopped
what are the 3 additional manifestations of type VI (HIV-associated) variant of PRP?
acne conglobata, hidradenitis suppurativa, and lichen spinulosus
clinical course of type VI (HIV-associated) variant of PRP
may respond to antiretroviral triple therapy
diffrentiate PRP from psoriasis
- 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
what features of psoriasis in histopath are not seen in PRP?
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
This type of PRP has little or no tendency to resolve spontaneously.
Type V PRP
