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
Number 1 ddx for PR
Syphillis
What are 5 medications known to cause drug induced PR?
o Ketotifen
o Flagyl
o Clonidine
o Bismuth, beta-blocker, barbituates, BCG vaccine
o Arsenic, Ace-Inhibitors (#1), Accutane
o Gold
o Salvarsan
Additionally: TNF inhibitors, HCTZ, omeprazole
5 possible tx’s for PR
None-self resolves
UVB
Sunlight
Erythromycin PO x 14 days
Acyclovir PO-800 mg 5x daily x 1 week
Antihistamines
Rarely oral steroids
3 variants pityriasis lichenoides
Pityriasis lichenoides et variolofirmis acute
Pityriasis lichenoides chronica
Febrile ulceronecrotic Mucha–Habermann disease (FUMHD)
Name 3 possible triggers for PLEVA/PLC
DRugs-tnfs inhibitors, statins, estrogen-progesterone
Radiocontrast dye
Infection: HIV, parvo
?maternal GVHD
What is the main infiltrate in PLEVA? PLC?
Clonal T-cell proliferation thus seen as T-cell lymphoproliferative disase
PLEVA=CD8
PLC=CD4
What are the clinical manifestations of PLEVA
Recurrent and spontaneously regressing crops of erythematous to purpuric papules and papulovesicles, can develop crust and ulcerations.
May have varioliform scarring
Asymptomatic, resolve most often with out scarring
What is febrile ulceronecrotic Mucha–Habermann disease (FUMHD)
Develop large coalescing ulcerative-necrotic lesions
Can have mucosal, gastrointestinal, and pulmonary involvement can be observed
May have malaise, fever, lymphadenopathy, arthritis, and/or bacteremi
What are the clinical features of PLC
Red-brown scaly papules
More indolent course, regress over weeks to months–> result in hypo pigmented macules
What is the prognosis of PLEVA? PLC?
PLC-may regress over several months, often relapsing and remitting with long periods remissions
What is a predictor of length of disease course?
Distribution of lesions:
generalized-short course(11 months)
Peripheral distribution had the longest average clinical course (33 months).
The central distribution variant was intermediate.
Name 6 histopath findings of pityriasis lichenoides
- superficial perivascular interface dermatitis in all cases
- Lymphocytic infiltrate, may be neutrophils
- Focal parakeratosis
- Epidermal edema
- Fun thickness epidermal necrosis
- RBC extravasation
- Lymphocytic vasculitis is sometimes described, but true fibrinoid necrosis of blood vessels is not seen
- necrotic keratinocytes
Name 4 ddx for PLEVA
lymphomatoid papulosis,
arthropod reactions,
cutaneous small vessel vasculitis,
varicella
drug eruption
EM
Name 4 ddc for PLC
Small plaque parapsoriasis
Guttate psoriasis
Exnathematous LP
PR
Syph
Papular dermatitis
Lichenoid drug
Name 4 clonal T-cell related dermatitides
PArapsoriasis
PLEVA/PLC
LYP
Which type of parapsoriasis is associated with MF
LArge plaque
Who gets parapsoriasis
5th decade peak, sl. male predominance
What are the clinical features of small plaque parapsoriasis
Round-oval PATCHES, variably erythematous, fine scale, <5 cm, some with yellow hue
Asymptomatic or mildly pruritic
Widespread on trunk and extremities OR localized on sun protected sites
Name a variant of small plaque parapsoriasis and describe the clinical features
Digitate dermatosis
1-10 cm patches
Elongated, finger-like patches symmetrically distributed on the flanks
Risk MF is LOW
What is the name of the yellow hue small plaque parapsoriasis
xanthoerythrodermia perstan
Name 3 histopath findings of parapsoriasis
-mild, nonspecific spongiotic dermatitis
- focal parakeratosis
- Exocytosis of lymphocytes is variable but usually present. CD4+
Name 5 possible tx option small plaque parapsoriasis
TCS
TCIs
Topical tar
Topical baxarotene
Imiquimod
UVA/PUVA/nbUVB
What is risk of progression from LPP to MF?
10% to 35% over a period of 6–10 years
* Some think this is patch stage MF
Predominant cell infiltrate in large plaque psoriasis
CD4+ T cells
Describe the clinical features of Large plaque parapsoriasis
Variably erythematous to red brown, round to irregularly shaped, slightly scaly patches > 5 cm
May show poikiloderma
What is the variant of Large plaque parapsoriasis? What is the risk of MF progression?
Retiform parapsoriasis
widespread, ill-defined patches in a net-like or zebra-stripe pattern
Almost all progress to MF
Name 5 features LPP on path
parakeratosis
a mild, nonspecific spongiotic dermatitis or an interface lymphocytic infiltrate with a variable degree of lichenoid features
may contain atypical lymphoid cells
epidermal atrophy, telangiectasia, and pigment incontinence if poikiloderma
Contrast the two types of pityriasis rotunda
Type I-occurs mostly in black and asian, associated with internal malignancy, no family history, hyperpigmented plaques
Type II- multiple plaques (>30), family gig story, but no internal malignancy association
What are the two main theories on the pathogenesis of pityriasis rotunda
- Associated with malnutrition (common final pathway for malignancy or infection like TB or leprosy, infection)
- Minor acquired ichthyosis that can be familial
Who gets pityriasis rotunda
Far East (Japan, china), Middle East (Morocco, Italy, Israel), African americans
25-45
Describe the clinical features pityriasis rotunda
large circular and polycyclic lesions that are often 10 cm (but may be up to 30 cm)
fine scale and are moderately hyperpigmented with a sharp margin and no inflammation.
The trunk and extremities are favored.
A hypopigmented halo has been described in some patients, and sometimes the entire lesion can be hypopigmented.
Name 4 histopathological features pityriasis rotunda
Hyperkeratosis without parakeratosis
Diminished granular cell layer
Epidermal atrophy
Pigment incontinence
perivascular lymphohistiocytic infiltrate, and occasional follicular plugging
increased pigmentation basal cellnlayer
What is a treatment that has moderate evidence in pityriasis rotunda
topical lactic acid, urea, tars, emollients, and corticosteroids have provided little benefit.
Topical tretinoin cream 0.1% can result in modest improvement and systemic retinoids warrant consideration for patients with more extensive disease.
However, reversal of any underlying disorder – in particular, malnutrition, infection, or malignancy – should be addressed first.
Who gets granular parakeratosis
Middle age to older adult women
What is the underylgin pathogenesis in granular parakeratosis
Underlying profillagrin to fillagrin processing defect with retention of keratohyaline granules with resulting aggregate keratin filaments during cornification.
Describe the clinical features of granular parakeratosis
Keratotic brown-red papules with conical shape
Occurs in intertriginous areas
May coalesce into larger plaques, may become macerated
Often quite pruritic
