Ch. 9-Other papulosquamous Flashcards

1
Q

Typical onset of PRP?

A

2 peaks
1st/2nd decade

6th decade

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2
Q

Gender differences PRP?

A

Equally affected

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3
Q

What gene is involved in familial PRP

A

CARD14

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4
Q

Name 4 hypothesized triggers for PRP

A

Infection
Trauma
UV exposure
Drugs-imatinib
Malignant-renal and hepatocellular carcinoma
Autoimmune-celiac, MG and hypothyroid have been associated

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5
Q

Name 6 classic findings in PRP

A

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

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6
Q

According to the Griffith classification, what are the 5 variants of pityriasis rubra? What is the 6th additional variant?

A

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

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7
Q

What is prognosis of:
classic adult PRP
Classic juvenile
Atypical adult
Atypical juvenile
Circumscribed/localized juvenile
HIV

A

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

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8
Q

What % of PRP does Type I-VI make up?

A

Type I- 55%
Type II-5%
Type III-circumscribed-25%
Type IV-classic juvenile 10%
Type V-Atypical juvenile 5%
Type VI-<1%

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9
Q

Describe 3-4 differences each from classic PRP in:
-adult atypical
-juvenile atypical

A

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

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10
Q

What is Kaposi varicelliform eruption

A

Eczema herpeticum

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11
Q

NAme 3 features circumscribed PRP

A
  1. Prepubertal onset
  2. Ertyhematous well demarcated plaques over knees, elbows, knuckles and dorsal fingers
    3.Follicular keratotic papules
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12
Q

Name 4 features HIV associated PRP that are unique

A
  1. Erythematous follicular papules w/ Keratotic spines
  2. HS
  3. Acne conglobata
  4. May improved with anti-retrovirals, often fails to respond to typical therapy

*Can also get coalescing plaques

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13
Q

Name 4 features HIV associated PRP that are unique

A
  1. Erythematous follicular papules w/ Keratotic spines
  2. HS
  3. Acne conglobata
  4. May improved with anti-retrovirals, often fails to respond to typical therapy

*Can also get coalescing plaques

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14
Q

5 histopathological features of PRP

A
  1. Alternating vertical and horizontal ortho and parakeratosis “checkerboard pattern”
  2. Dilated hair follicles with follicular plug
  3. Parakeratosis on the shoulder of a dilated follicle
  4. Hypergranulosis
  5. Shortened, thick, rete pegs
    6.Sparse perivascular lymphohistiocytic infiltrate
  6. acantholysis with focal dyskeratosis can be seen
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15
Q

What is the 7th proposed form fruste of PRP

A

Facial discoid dermatosis

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16
Q

Name 3 classes/ exampled ofsystemic treatments for PRP

A

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

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17
Q

Who gets pityriasis rosea

A

10-35 years on average, slight female predominance, peak adolescence

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18
Q

How long does PR last?

A

6-8 weeks, up to 5 months

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19
Q

What are the two proposed causes of PR

A

HHV-6
HHV-7*-predominant

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20
Q

Name the classic cutaneous findings of PR

A

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%

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21
Q

2 differences in darker skin types in PR

A

More papular and hyper pigmented, sometimes follicular

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21
Q

What are 6 morphological variants of Pityriasis Rosea?

A

Purpuric
Vesicular
Pustular
Inverse
Gigantea
EM-like
Atypical
Urticarial

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22
Q

What are 5 features seen on pathology for Pityriasis Rosea?

A

Mounds of parakeratosis
Lymphohistiocytic perivascular and interstitial infiltrate
Mild RBC extravasation
Spongiosis
Papillary dermal edema
Diminution granular layer
Rarely epidermal pustules

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23
Q

Number 1 ddx for PR

A

Syphillis

24
Q

What are 5 medications known to cause drug induced PR?

A

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

25
Q

5 possible tx’s for PR

A

None-self resolves
UVB
Sunlight
Erythromycin PO x 14 days
Acyclovir PO-800 mg 5x daily x 1 week
Antihistamines
Rarely oral steroids

26
Q

3 variants pityriasis lichenoides

A

Pityriasis lichenoides et variolofirmis acute
Pityriasis lichenoides chronica
Febrile ulceronecrotic Mucha–Habermann disease (FUMHD)

27
Q

Name 3 possible triggers for PLEVA/PLC

A

DRugs-tnfs inhibitors, statins, estrogen-progesterone

Radiocontrast dye

Infection: HIV, parvo

?maternal GVHD

28
Q

What is the main infiltrate in PLEVA? PLC?

A

Clonal T-cell proliferation thus seen as T-cell lymphoproliferative disase

PLEVA=CD8
PLC=CD4

29
Q

What are the clinical manifestations of PLEVA

A

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

30
Q

What is febrile ulceronecrotic Mucha–Habermann disease (FUMHD)

A

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

31
Q

What are the clinical features of PLC

A

Red-brown scaly papules
More indolent course, regress over weeks to months–> result in hypo pigmented macules

32
Q

What is the prognosis of PLEVA? PLC?

A

PLC-may regress over several months, often relapsing and remitting with long periods remissions

33
Q

What is a predictor of length of disease course?

A

Distribution of lesions:
generalized-short course(11 months)
Peripheral distribution had the longest average clinical course (33 months).
The central distribution variant was intermediate.

34
Q

Name 6 histopath findings of pityriasis lichenoides

A
  1. superficial perivascular interface dermatitis in all cases
  2. Lymphocytic infiltrate, may be neutrophils
  3. Focal parakeratosis
  4. Epidermal edema
  5. Fun thickness epidermal necrosis
  6. RBC extravasation
  7. Lymphocytic vasculitis is sometimes described, but true fibrinoid necrosis of blood vessels is not seen
  8. necrotic keratinocytes
35
Q

Name 4 ddx for PLEVA

A

lymphomatoid papulosis,
arthropod reactions,
cutaneous small vessel vasculitis,
varicella
drug eruption
EM

36
Q

Name 4 ddc for PLC

A

Small plaque parapsoriasis
Guttate psoriasis
Exnathematous LP
PR
Syph
Papular dermatitis
Lichenoid drug

37
Q

Name 4 clonal T-cell related dermatitides

A

PArapsoriasis
PLEVA/PLC
LYP

38
Q

Which type of parapsoriasis is associated with MF

A

LArge plaque

39
Q

Who gets parapsoriasis

A

5th decade peak, sl. male predominance

40
Q

What are the clinical features of small plaque parapsoriasis

A

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

41
Q

Name a variant of small plaque parapsoriasis and describe the clinical features

A

Digitate dermatosis
1-10 cm patches
Elongated, finger-like patches symmetrically distributed on the flanks
Risk MF is LOW

42
Q

What is the name of the yellow hue small plaque parapsoriasis

A

xanthoerythrodermia perstan

43
Q

Name 3 histopath findings of parapsoriasis

A

-mild, nonspecific spongiotic dermatitis
- focal parakeratosis
- Exocytosis of lymphocytes is variable but usually present. CD4+

44
Q

Name 5 possible tx option small plaque parapsoriasis

A

TCS
TCIs
Topical tar
Topical baxarotene
Imiquimod
UVA/PUVA/nbUVB

45
Q

What is risk of progression from LPP to MF?

A

10% to 35% over a period of 6–10 years
* Some think this is patch stage MF

46
Q

Predominant cell infiltrate in large plaque psoriasis

A

CD4+ T cells

47
Q

Describe the clinical features of Large plaque parapsoriasis

A

Variably erythematous to red brown, round to irregularly shaped, slightly scaly patches > 5 cm

May show poikiloderma

48
Q

What is the variant of Large plaque parapsoriasis? What is the risk of MF progression?

A

Retiform parapsoriasis

widespread, ill-defined patches in a net-like or zebra-stripe pattern

Almost all progress to MF

49
Q

Name 5 features LPP on path

A

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

50
Q

Contrast the two types of pityriasis rotunda

A

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

51
Q

What are the two main theories on the pathogenesis of pityriasis rotunda

A
  1. Associated with malnutrition (common final pathway for malignancy or infection like TB or leprosy, infection)
  2. Minor acquired ichthyosis that can be familial
52
Q

Who gets pityriasis rotunda

A

Far East (Japan, china), Middle East (Morocco, Italy, Israel), African americans

25-45

53
Q

Describe the clinical features pityriasis rotunda

A

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.

54
Q

Name 4 histopathological features pityriasis rotunda

A

Hyperkeratosis without parakeratosis

Diminished granular cell layer

Epidermal atrophy

Pigment incontinence

perivascular lymphohistiocytic infiltrate, and occasional follicular plugging

increased pigmentation basal cellnlayer

55
Q

What is a treatment that has moderate evidence in pityriasis rotunda

A

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.

56
Q

Who gets granular parakeratosis

A

Middle age to older adult women

57
Q

What is the underylgin pathogenesis in granular parakeratosis

A

Underlying profillagrin to fillagrin processing defect with retention of keratohyaline granules with resulting aggregate keratin filaments during cornification.

58
Q

Describe the clinical features of granular parakeratosis

A

Keratotic brown-red papules with conical shape

Occurs in intertriginous areas

May coalesce into larger plaques, may become macerated

Often quite pruritic