PRP Flashcards
Overview
Papulosquamous
Unknown cause
Initially present with well defined salmon-red or orange-red dry scaly plaques
Composed of erythematous hyperkeratotic perifollicular papules
Tend to coalesce into widespread plaques
Islands of normal skin in between “islands of sparing”
Cephalocaudal spread —> often starts on the scalp before spreading down over rest of the body
May progress to erythroderma in adults
+/- pruritus in the early stages
Characteristic follicular papules “nutmeg grater” papules
- elbows
- wrists
- dorsal fingers
Orangey discolured palmoplantar keratoderma (thickened and fissured)
Bimodal age distribution for acquired forms (1st and 5th decades)
Rare familial form (Type V atypical juvenile PRP) starts in early childhood
Associated diseases
MOST OF UNCERTAIN SIGNIFICANCE
? SOLID TUMOURS Larynx CA Lung CA Kidney CA Colon CA
—> Despite these case reports, NOT generally thought to be assoc with increased incidence of malignancy
? HAEM MALIGNANCIES
Leukaemia
Sezary syndrome
? AUTOIMMUNE DISEASES Systemic sclerosis Autoimmune thyroiditis Vitiligo Alopecia universalis Lichen planus
INFECTION
Streptococcal URTI (children)
HIV (specific subtype Type IV)
? Hep C
—> paucity of reports, causal link UNLIKELY
FAMILY HX
Mostly in the rare familial Atypical juvenile PRP (type V) form
- AD inheritance
- Heterozygous mutation in CARD14
- Overlaps with PSOR2 susceptibility locus for psoriasis
Type II Atypical adult PRP
Type IV Juvenile Circumscribed PRP
Histology
Changes with evolution of disease
Not pathognomonic but allows distinction from psoriasis and other causes of erythroderma
Hyperkeratosis with follicular plugging
Alternating verticle and horizontal Ortho and parakeratosis in the skin and follicles (checkerboard pattern)
Foci of parakeratosis in the perifollicular shoulder
Patchy vs confluent hypergranulosis
Epidermal acanthosis —> usually psoriasiform
+/- Acantholysis in adenexal epithelium
Dilated dermal capillaries (but not tortuous as in psoriasis)
Sparse Lymphohistiocytic perivascular and dermal infiltrate
No neuts
6 Clinical subtypes
Type I Classical adult onset PRP (generalised sporadic form with acute onset)
Type II Atypical adult onset PRP (generalised sporadic form with acute onset)
Type III Classical juvenile onset PRP (generalised sporadic form with acute onset)
Type IV Circumscribed juvenile PRP
Type V Atypical juvenile PRP (rare familial form with slow and gradual onset)
Type VI HIV-related PRP
Type I Classical adult onset PRP
Most common
50% of cases
Age 40-60 yrs
SUMMARY OF CLINICAL FEATURES Follicular keratoses Orange-red erythema Branny scales Islands of sparing Diffuse oragne yellow PPK @ PRP sandal Nails —> thickened, subungual hyperkeratosis, distal discolouration, splinter haemorrhages Oral mucosa —> diffuse whitish mucosa, lacy white plaques, erosion
CLINICAL ONSET/PROGRESSION
Usually starts without obvious precipitating factors
Erythematous slightly scaly macule on the head, neck, upper trunk
Further macules appear within a few weeks
Rash may initially be mistaken for seb derm
Then appearance of erythematous perifollicular papules with central acuminate/spiny plug
Follicular lesions initially discrete —> then coalesce into groups
Irritation initially absent —> may be pronounced as disease spreads
Inter follicular erythema appears —> follicular lesions gradually submerged in sheets of erythema of slight orange hue —> typically spreading from head to toe
Face uniformly erythematous —> mild ectropion
Scalp —> diffuse bran-like scaling
Erythroderma develops within 2-3 months
Palms soles —> hyperkeratotic and yellow
Nails —> Thickened, discoloured distally, splinter haemorrhages (no dystrophy of nail plate, minimal pitting to suggest psoriasis)
COMPLICATIONS OF PROLONGED ERYTHEMA/ERYTHRODERMA
Eruptive seb Ks
Ectropion
Peripheral oedema
High output cardiac failure (in the elderly)
COURSE/PROGNOSIS
May progress from limited disease to erythroderma in weeks
Evetually clears spontaneously in majority
If Untreated —> eventually resolve ~ 3 yrs in majority of patients
May persist indefinitely in some patients
Type II Atypical adult onset PRP
More chronic form —> May last for many years
5% of cases
Scaling more variable than type I Classical adult onset PRP
Tend to have lamellar scaling legs
Not erythrodermic
May have alopecia
Many patients have
- eczematous features
- PPK coarser
COURSE/PROGNOSIS
NO rapid progression of inflammation from head to toe (as in Type I Classical adult onset PRP)
Erythroderma less common
Chronic but limited in extent
Little tendency for spontaneous resolution
Type III Classical juvenile onset PRP
Most common childhood PRP
Equivalent of Type I Classical adult onset PRP but age of onset between age 5-10 yrs
Clinical presentation same as Type I
May be preceded by Type IV circumscribed juvenile PRP
COURSE/PROGNOSIS
Usually undergoes spontaneous resolution in 1-2 yrs (shorter course than Type I Classical adult onset PRP)
Some patients might evolve into Type IV Circumscribed juvenile PRP)
Recurrence in adulthood reported
Type IV Circumscribed juvenile PRP
Prepubertal children usually age <12 yrs
25% of cases
Well circumscribed plaques of erythema and follicular hyperkeratosis on elbows and knees
+/- scattered erythematous scaly macules trunk and scalp
+/- PPK
DDX
Keratosis circumscripta
COURSE/PROGNOSIS
Uncertain but may last indefinitely
Variable course
Reports of disease remitting in teenage years
Type V Atypical juvenile PRP
Chronic childhood type
5% of cases
Present at birth or starts in early childhood —> erythema, hyperkeratosis
Follicular hyperkeratosis ++
Keratoderma +
Some cases have scleroderma-like changes digits hands, feet
FAMILIAL PRP —> usually presents as this subtype
starts in early childhood
AD inheritance
Heterozygous mutation in CARD14
Overlaps with PSOR2 susceptibility locus for psoriasis
May be difficult to distinguish from ichthyoses, erythrokeratoderma
COURSE/PROGNOSIS
Chronic but limited in extent
Little tendency for spontaneous resolution
Type VI HIV related PRP
Tends to resemble Type I Classical adult onset PRP
Resistant to Rx
May respond to antiretroviral Rx
ASSOCIATIONS —> HIV ASSOC FOLLICULAR SYNDROME
Truncal acne conglobata
Some patients develop elongated filiform keratoses on the face, trunk (lichen spinulosis)
HS
COURSE/PROGNOSIS
Spontaneous remission w/o Rx reported
DDX of eruptions with erythema + follicular hyperkeratosis
Psoriasis
- Scalp scaling adherent —> bran-like in PPK
- PPK less common —> constant in PPK
- Islands of sparing less common —> characteristic in PPK
- Nail pitting and nail salmon patches common —> absent in PPK
- Nail growth rate markedly increased —> moderately increased in PPK
- Epidermal cell turnover markedly increased —> moderately increased in PPK
- Munro microabscesses on histo characteristic —> absent in PPK
- Assoc seronegative arthropathy common —> rare in PPK
- Good response to UVB —> poor response in PPK
- Good reponse to TCS —> limited response in PPK
- Good response to MTX —> variable response in PPK
- Good/excellent response to anti-TNF (etanercept, adalimumab, infiliximab) —> variable response in PPK
- Excellent response to IL12/23 (Ustekinumab) —> probably EXCELLENT response in PPK
Erythrokeratoderma variabilis
Sezary syndrome
Other CTCL i.e. mycosis fungoides
Other forms of erythroderma
- Atopic dermatitis
- Drug eruption
Seb derm
Follicular ichthyosis
Keratosis pilaris
Complications of erythroderma
Dependant oedema
High output cardiac failure esp in elderly patients
Investigations
Clinical features
Skin bx for histology (refer to histo features)
No other tests indicated
Consider
- HIV serology
- Age appropriate malignancy screen
If erythrodermic -
- FBC (exclude secondary infection)
- ELFTs (renal, hepatic, hypoalbuminaemia)
Management
Rare disease w/o large controlled trials of Rx interventions
Recommendations based on case reports, small series, retrospective reviews
Claims of Rx success to be balanced against a disease with spontaneous resolution natural Hx
ERYTHRODERMA (requires Intense supportive care)
Admit
Bed rest
Space blanket (Prevent hypothermia)
QID obs
- HR
- BP
- temp
DVT prophylaxis
- Dalteparin 5000 IU S/C
IVF if dehydrated —> Manage electrolyte imbalance
- monitor fluid and electrolyte imbalance
- daily EUC
If hypoalbuminaemia —> Manage protein loss
- high protein diet
- dietician consult
If ectropion
- Cellufresh eye drops Q4-6H during day
- Refresh Lacrilube eye ointment (WSP ointment) nocte
If itchy
- Sedating antihistamine (promethazime 10 - 25mg) at night
Manage any secondary skin infection or sepsis
- antibiotics
GENERAL SKIN CARE
Scalp bran-like scaling
- 5% SA in WSP nocte
Body (treat dry skin, restore barrier function)
- soap avoidance
- Aqueous cream as soap substitute
- Apply WSP/Dermeze emolients liberally QID
- Liquid paraffin baths
Palms, soles keratoderma
- 5-12% SA (keratolytic) OR
- 10-12% Lactic acid (keratolytic) OR
- 20-40% propylene glycol (keratolytic) OR
- urea
SPECIFIC RX - FIRST LINE
Mild to mod potency TCS (symptomatic relief in patients with pruritus, no long term effect on course of PRP)
- Aristocort ointment +/- wet wraps (itch)
Oral acitretin 0.5 - 0.75mg/kg/day up to 50mg/day
SPECIFIC RX - SECOND LINE
Topical calcipotriol cream BD (Type IV Circumscribed juvenile PRP)
- suitable for localised PRP subtypes
- not suitable for extensive skin involvement as will use > 100g per week (30g covers whole body)
Topical tazarotene (Type IV Circumscribed juvenile PRP)
Oral MTX up to 20mg weekly —> responses not as good as in psoriasis, advocate combo with acitretin
S/C ustekinumab —> consider for severe Type I Classical adult onset PRP where acitretin or MTX failed or C/I
CSA
AZA
SPECIFIC RX - THIRD LINE
Anti-TNF (etanercept, adalimumab, infiliximab)
NbUVB —> consider of systemic Rx C/I
Extracorporeal photochemotherapy —> consider if systemic Rx C/I
