Ch. 9-Other papulosquamous Flashcards
Typical onset of PRP?
2 peaks
1st/2nd decade
6th decade
Gender differences PRP?
Equally affected
What gene is involved in familial PRP
CARD14
Name 4 hypothesized triggers for PRP
Infection
Trauma
UV exposure
Drugs-imatinib
Malignant-renal and hepatocellular carcinoma
Autoimmune-celiac, MG and hypothyroid have been associated
Name 6 classic findings in PRP
Orange-red hyperkeratotic follicular papules nutmegrater papules
Coalescing orange-red plaques with islands of sparing
Erythroderma
Orange-red waxy keratoderma
Erythema with fine diffuse scaling to scalp
Spreads caudally
*Nails change-thickened plate with a yellow–brown discoloration and subungual debris.
*The mucous membranes are rarely involved, but they may show features similar to oral lichen planus.
*Can show photo aggravation
According to the Griffith classification, what are the 5 variants of pityriasis rubra? What is the 6th additional variant?
Type I-Classic adult PRP
Type II-Atypical adult PRP
Type III-Circumscribed juvenile PRP
Type IV- Classic juvenile PRP
Type V- Atypical juvenile PRP
Type VI-HIV associated PRP
What is prognosis of:
classic adult PRP
Classic juvenile
Atypical adult
Atypical juvenile
Circumscribed/localized juvenile
HIV
Classic adult - 80% clear in 3 years
Classic juvenile-same
Atypical adult-Chronic course, 20% clear in 3 yrs
Atypical juvenile-chronic course
Circumscribed-variable
HIV-variable
What % of PRP does Type I-VI make up?
Type I- 55%
Type II-5%
Type III-circumscribed-25%
Type IV-classic juvenile 10%
Type V-Atypical juvenile 5%
Type VI-<1%
Describe 3-4 differences each from classic PRP in:
-adult atypical
-juvenile atypical
Adult:
1. Coarse, lamellar keratoderma
2. eczematous dermatitis
3. Icthyosiform scaling to legs
4. occasional alopecia
Juvenile:
1. itchyosiform scaling
2. sclerodermoid changes to hands and feet
3. If familial with CARD14 gene, typically present-with atypical juvenile
What is Kaposi varicelliform eruption
Eczema herpeticum
NAme 3 features circumscribed PRP
- Prepubertal onset
- Ertyhematous well demarcated plaques over knees, elbows, knuckles and dorsal fingers
3.Follicular keratotic papules
Name 4 features HIV associated PRP that are unique
- Erythematous follicular papules w/ Keratotic spines
- HS
- Acne conglobata
- May improved with anti-retrovirals, often fails to respond to typical therapy
*Can also get coalescing plaques
Name 4 features HIV associated PRP that are unique
- Erythematous follicular papules w/ Keratotic spines
- HS
- Acne conglobata
- May improved with anti-retrovirals, often fails to respond to typical therapy
*Can also get coalescing plaques
5 histopathological features of PRP
- Alternating vertical and horizontal ortho and parakeratosis “checkerboard pattern”
- Dilated hair follicles with follicular plug
- Parakeratosis on the shoulder of a dilated follicle
- Hypergranulosis
- Shortened, thick, rete pegs
6.Sparse perivascular lymphohistiocytic infiltrate - acantholysis with focal dyskeratosis can be seen
What is the 7th proposed form fruste of PRP
Facial discoid dermatosis
Name 3 classes/ exampled ofsystemic treatments for PRP
Retinoids–> isotretinoin or acitretin
MTX
TNF-alpha inhibitors, secukinumab ustekinumab
Others:
-other immunosuppresives
-anabolic steroids
*TCS, TCIs, tar, vitamin D3 can be used as adjuncts
*UV therapy can exacerbate but some case series nbUVB or PUVA + retinoid show improvement
Who gets pityriasis rosea
10-35 years on average, slight female predominance, peak adolescence
How long does PR last?
6-8 weeks, up to 5 months
What are the two proposed causes of PR
HHV-6
HHV-7*-predominant
Name the classic cutaneous findings of PR
Herald patch, solitary on trunk, 2-4 cm (1-10 cm descibred), pink-salmon or pink-brown scaly plaque, enlarges over days with a advancing border, trailing scale, in-toeing, branny scale
followed days later by multiple smaller scaly papules and plaques, round to oval, often following langers lines, trunk and extremities. Similar central fine scaling or sometimes collarette scale, a advancing border
Face, palms, soles typically spared
Minute pustules sometimes may be seen
Priuritus in 25%
2 differences in darker skin types in PR
More papular and hyper pigmented, sometimes follicular
What are 6 morphological variants of Pityriasis Rosea?
Purpuric
Vesicular
Pustular
Inverse
Gigantea
EM-like
Atypical
Urticarial
What are 5 features seen on pathology for Pityriasis Rosea?
Mounds of parakeratosis
Lymphohistiocytic perivascular and interstitial infiltrate
Mild RBC extravasation
Spongiosis
Papillary dermal edema
Diminution granular layer
Rarely epidermal pustules