Other Papulosquamous disorders Flashcards

(51 cards)

1
Q

Small plaque Parapsoriasis things

What is it/ lesion

description Histologically (brief)

Predominant cell type

Differential for this type of lesion

A

Chronic asymptomatic, erythematous scaly patches, usually <5 cm lesions in diameter

Histo: mild, nonspecific spongiotic dermatitis with parakeratosis

Cell: Predominance of CD4+ T cells with dominant T-Cell clonality in the superficial lymphoid infiltrate

Male dominance

Sometimes erythematous/ pruritic, sometimes with fine scale, generally asymptomatic `

DDx: Pityriasis rosea, Drug eruption (PR like), pityriasis lichenoides chronica, psoriasis, MF, secondary syphilis, nummular dermatitis

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2
Q

Large plaque parapsoriasis is / isn’t a distinct entity from small plaque

DDx of Large plaque parapsoriasis

Chance of turning into MF?

A

Is distinct, is thought to be even related to early MF or CTCL

Chronic, asymptomatic, erythematous larger (>5 cm) scaly patches

May or May not exhibit telangiectasia, atrophy, hypo/hyperpigmentation → this triad = poikilodermatous variant

Histo: nonspecific spongiotic dermatitis with lichenoid features, predominance of CD4+ T cells in lymphocytic infiltrate

Ddx: MF, drug eruption (MF like), psoriasis, poikilodermatous autoimmune connective tissue disease (Dermatomyositis), radiodermatitis

Rate of converting from large plaque parapsoriasis to overt lymphoma/ MF? 10-35%/ year

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3
Q

Standard treatments for Small and large plaque parapsoriasis

A

Topical corticosteroids, topical coal tar, phototherapy, ?Bexarotene, ?calcineurin inhibitors, imiquimod?

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4
Q

What is shown, describe

A

Scaly, atrophic, red-brown papules-plaques in a reticular/ net like pattern

Retiform parapsoriasis

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5
Q

Large plaque psoriasis with telangiectasia, atrophy, hypo/hyperpigmentation →

A

poikilodermatous variant

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6
Q

Pityriasis lichenoides spectrum (two main variants)

A

Pityriasis lichenoides et varioliformis Acuta (PLEVA)

Pityriasis lichenoides chronica

Both entities = recurrent crops of spontaneously regressing erythematous to purpuric papules

PLEVA lesions = occasionally vesiculopustular/ crusted

PLEVA generally CD8+ cells predominate

PLC = Scaly

PLC CD4+ cells predominate

Histo = interface dermatitis with necrotic keratinocytes with T cell infiltrate, often monoclonal

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7
Q

Describe the lesions

A

A) widespread erythematous papules/ papulovesicles with occasional crusted lesions

B) same as A with ulcers

C) multiple red brown papules with some scale (more characteristic of PLC)

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8
Q

In Pityriasis Lichenoides: in terms of average length of disease activity how does diffuse distribution versus peripheral dist. change length??

A

Diffusely distributed represents shorter disease course generally with peripheral distribution lasting longer

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9
Q

Histological pathology/ description of pityriasis lichenoides

A

Dense cellular infiltrate (usually in the Dermis) → C

Epidermal focal Parakeratosis, occasional keratinocyte necrosis, mild erythrocyte extravasation (B)

A = chronic form of PLC, more prominent parakeratosis, less intense destruction of the epidermis

B = Acute PLEVA, scattered necrotic keratinocytes + parakeratosis, also note the extravasated erythrocytes in the dermis

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10
Q

DDX for PLEVA (Pityriasis Lichenoides Acuta

A

lymphatoid papulosis

cutaneous small vessel vasculitis

varicella, enteroviral exanthem

arthropod reaction

erythema multiforme

lichenoid drug eruption

folliculitis

dermatitis herpetiformis

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11
Q

Two types of parapsoriases: PLEVA and PLC (pityriasis lichenoides)

What are they

Adults or kids? male or female?

What are the suspicions of the pathogenesis?

A

Both have recurrent crops of spotneously regressing erythematous papules

Both are more prevalent in pediatric population, male predominance

Postulated to be a response to foreign antigens (infxn/ drugs), possible interaction with HIV/ Parvo B19, Meds (Estrogen/progesterone, TNF-Alpha inhibitors, statins, radiocontrast dye, maternal keratinocytes found in some patients (GVHD?)

PLEVA lesions crust and are occasionally vesiculopustular

PLC lesions are scaly

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12
Q

What is febrile ulcernecrotic mucha-habermann disease?

A

a variant of pityriasis lichenoides (PLEVA typically) that refers to diffuse involvement of the skin with confluent large, necrotic skin lesions with mucosal, GI, pulmonary involvement → increased TNF-A

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13
Q

Therapeutic ladder for pityriasis lichenoides

A
  1. Topical steroids
  2. topical coal tar
  3. oral erythromycin → tetracyclines → azithromycin
  4. Sunlight → UVB → PUVA
  5. MTX → cyclosporine
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14
Q

How is Pityriasis rubra pilaris (PRP) aquired?

Which gene is implicated?

A

Typically genetically, autosomal dominant most common, occasional A. Recessive

Gene: CARD14 aka PSORS2

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15
Q

Define the multiple, well described clinical features of CLASSIC PRP (pityriasis rubra pilaris)

A

Follicular hyperkeratosis on an erythematous base

Rough papules on the proximal fingers

Papules on the trunk - extremities that coalesce to form large salmon colored to orange-red plaques with “distinct - islands of sparing

These coalescing plaques progress to an erythrodermic appearance with varying degrees of exfoliation

PALMS/ SOLES involved with a distinct orange-red waxy keratoderma

Scalp → erythema with a fine diffuse scale

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16
Q

Describe the lesions

A

A - Orange-red follicular papules coalescing into large scaly plaques with islands of sparing, obvious follicular hyperkeratosis

B - dorsal digits with clustered keratotic follicular papules

c - Salmon colored plaques with islands of sparing

D - erythrodermic with follicular accentuation of the scale

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17
Q

The most common form of PRP begins where and progresses ____

Are there typically symptoms?

How long does it last?

A

Beings on head/neck and progresses caudally

Commonly involves pruritus and burning

Type 1 classically lasts (accounts for >50% of all cases) less than 3 yrs

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18
Q

Type 2 PRP is classically seen as

A

palmoplantar keratoderma with a coarse/ lamellated scale and ichthyosiform scaling on extremities with occasional allopecia

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19
Q

In what age group is PRP type 3-5 seen in classically?

A

Juveniles/ adolescents

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20
Q

Type 3 PRP is similar to ___

When is peak onset?

A

The classic adult form type 1 and clears within 3 years

Peaks of onset in first 2 years of life and adolescence

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21
Q

Type 4 PRP

How common vs other forms

What is it like

Type 5 PRP is similar to ?

A

Type 4 is in juveniles with focal involvement, most common of the juvenile varieties

Type 5 is similar to type 2 as it has extremity involvement with follicular hyperkeratosis, erythema, scleroderma like changes of hands/feet -> Accounts for most familiar forms of PRP

22
Q

Talk about the differential of PRP

What is the major entity when thinking of other possibilities

How about specifically for scalp?

A

psoriasis is most common other possibility

Distinctive orange-red palmoplantar keratoderma plus the keratotic follicular papules with islands of sparing of trunk, a fine scale, no family history of psoriasis, no oil drop changes/ pitting/ onycholysis.

Scalp - similar to seb derm, seb derm responds to therapy however

23
Q

Histological differences between PRP and psoriasis

A

Psoriasis - hypogranulosis, vasodilation, elongated Rete ridges, neutrophilic migration to the epidermis (munro microabscesses)

PRP - thickened granular layer, shorter thicker rete pegs, no vascular dilation, no neuts in the horn

24
Q

Treatments for PRP

A
  1. Isotretinoin (1-1.5 mg/kg/day) can induce significant clearing within 3-6 months if non-genetic variant
  2. MTX - weekly oral/ subcutaneous doses
  3. case reports suggest tnf-alpha inhibs (infliximab, adalimumab, etanercept)
    1. Secukinumab - Cosentyx (Il-17a inhibitor) and ustekinumab - stelara (IL12/23)
25
Pityriasis rosea is an acute, self limited papulosquamous eruption that favors otherwise healthy adolescents/ young adults, is self resolving, is thought to have a viral etiology. What are some other epidemiological facts, typical length of time? Which virus is implicated?
most commonly seen between 10-35 yo, female predominant, lasts 6-8 weeks (can last as much as 5 months) Virus implicated is HHV7, sometimes 6
26
Classic presentation of PR How does it differ in presentation in darker skinned individuals?
Solitary lesion appears on trunk and enlarges over several days (herald patch) The herald patch is pink-salmon-brown colored and is either a patch or plaque, slightly raised advancing margin, ranging from 2-4 cm but can be as large as 10 cm Center has small fine scales, the long axis of lesions follows langer cleavage lines, on the back the lesion orientation follows a “christmas tree patterns”, occasional minute pustules can be seen *In dark skinned individuals : lesions tend to be more papular and hyperpigmented*
27
Microscopic features of PR
Small mounds of parakeratosis, spongiosis and mild lymphohistiocytic perivascular interstitial papillary dermal infiltrate, sometimes mild erythrocyte extravasation and sometimes epidermal pustules Bonus: Mounded parakeratosis differential **Pityriasis rosea-like eruptions**: Triggered by medications or vaccinations **Guttate / eruptive psoriasis:** May be very similar clinically and histopathologically *Herald patch is usually not present in psoriasis* Histopathologically: neutrophils present in the parakeratosis; dilated vessels in the papillary plates are more common **with psoriasis** **Secondary syphilis:** Clinically, may be similar but no herald patch seen Histopathologically: psoriasiform and lichenoid dermatitis with perivascular and interstitial infiltrate with lymphocytes, histocytes and plasma cells, the infiltrate may be granulomatous Swollen, prominent endothelial cells are common ' **Other eczematous eruptions** **(contact dermatitis, nummular dermatitis, seborrheic dermatitis**): May be indistinguishable histopathologically and clinical correlation is required, Although a few eosinophils may be present in pityriasis rosea, large numbers are uncommon **Drug eruptions:** Variable clinical presentation Histopathology is variable; combination of interface pattern with spongiosis and eosinophil rich infiltrates are common **Pityriasis lichenoides:** Clinically, may have similar features; however, tends to have a chronic course, no herald patch present Histopathologically*_: an interface dermatitis and perivascular lymphocytic infiltrate is present with erythrocyte extravasates_* Presence of neutrophils in the parakeratosis
28
DDx of PR
if presence of split papules/ condyloma → syphilis (also confirm hx of primary chancre) 2ndry syph patients have more complaints, peripheral LAD Drug eruptions can mimic PR (ACE inhibitors, b-blockers, metronidazole, isotretinoin, barbiturates, NSAIDs, vaccines The herald patch can resemble tinea corporis, versicolor, nummular dermatitis
29
Type 1 pityriasis rotunda associations versus type 2 associations What does the Path look like?
Type 1 is seen primarily in blacks/ asians and appears as large \>10cm scaly, annular plaques/patch and is a/w internal malignancy (particularly hepatocellular/ gastric cancer) but no family history Type 2 is seen in caucasians and is typically \>30 smaller lesions with no fam history or a/w internal malignancy Both are seen in adults 24-45 yo and there is slight female predominance Most common in Mediterranean *May be a/w malnutrition, genetics* ***Path:*** **looks like ichthyosis vulgaris, absent granular layer, moderate hyperkeratosis without parakeratosis, increased pigmentation of basal layer, epidermal atrophy**
30
Granular parakeratosis pruritic? location? male vs female? lesion? Path:
yes pruritic involves all intertriginous sites female exclusively in adult forms Thoughts on pathogenesis: *potential failure to degrade keratohyalin granules and keratin filaments leading to hyperkeratosis* ***Lesion:*** **keratotic, brown-red papules (some with cone shape), some coalescing into larger, well-demarcated plaques with maceration/ fissures causing pain/ pruritus** Path: *stratum corneum is thickened with increased eosinophilic staining, retained nuclei in the keratin layer (parakeratosis), retained keratohyalin granules*
31
Common causes of intertrigo (4 on this card)
seb derm candidiasis inverse psoriasis erythrasma - Erythrasma is **a bacterial infection of the skin typically caused by Corynebacterium minutissimum**
32
Talk about the epidemiology of parasporiatic disorders
Onset age 40ish Males:Females 3:1
33
PLEVA and PLC have strict absence of what type of cell histologically?
Eosinophils
34
This variation of large plaque parapsoriasis has a rate of MF transformation of \_\_\_?
100% (possibly) Retiform parapsoriasis - variant of large plaque para *widespread, ill-defined patches in net-like pattern*
35
Ddx for small plaque parapsoriasis?
- Upper left- more common in children, often leaves behind PI hypopigmentation - upper right- itchy!! more common in older population - lower left- sudden onset of rash - middle lower: pink, scaly papules and small plaques - lower right- great mimicker, may have palm/sole involvement
36
Large plaque parapsoriasis Ddx :
37
Describe
Widespread papules with crust, some ulcerated, sometimes vesicles/ pustules
38
Which gene is associated with PRP?
CARD14 (PSORS2) ## Footnote **autosomal dominant**
39
PRP most common at which age
first and second decade and again in 6th decade
40
Do steroids help PRP?
NO Retinoids
41
Which infection is associated with LP? Those with this ifxn associated LP have likely involvement of ?
HCV Most common sites of involvement ionclude tongue, labial mucosa, gingiva
42
characteristic nail findings of LP include:
The characteristic nail abnormalities include lateral thinning (Fig. 11.15A), longitudinal ridging, and fissuring
43
Which two variants of LP have highest risk of malignancy?
Oral and cutaneous ## Footnote **in the Cutaneous we think of Hypertrophic and vulvovaginal LP**
44
Ddx for annular LP
Granuloma annulare (except LP has scale) Porokeratosis Tinea
45
Lichen striatus time course
- usually appears suddenly, develops fully over days to weeks, and after several months to a year or more, undergoes spontaneous resolution. - usually does not recur - Treatment of lichen striatus is usually not needed because it is self-limited, usually resolving within 1 to 2 years. Topical corticosteroids under occlusion can be used to hasten spontaneous resolution. - Left: **can have nail involvement** - Right: appearance on less pigmented skin types, where it appears more pink
46
Which two grey-brown rashes come to mind with LP variants
47
EDP which skin types describe morphology Path? treatment?
48
Talk about the differential for PLEVA
drug eruptions, viral exanthems, 2ndry syphillis, arthropod bite rxn, polymorphous light eruption, connective tissue disease, erythema multiforme *drug eruptions/ viral exanthem → **not as much keratinocyte necrosis/ less inflammation*** ***PLEVA/ PLC*** Do not contain eosinophils
49
PLC Histology
Perakeratosis, mild basal layer vacuolization, predominantly superficial perivascular infiltrate , extravasated RBCs (less than pleva)
50
DDx of PLC
Small plaque Parapsoriasis, LP, Pr, drug eruptions, PRP, early MF, subacute spongiotic dermatitis, guttate psoriasis, 2ndry syphilis
51
PLEVA acronym for path
Parakeratosis (focal) Lichenoid interface dermatitis Extravasation of rBCS Vasculitis \**lymphocytic* *Apoptotic keratinocytes* Top-heavy wedge shaped infiltrate , rbcs, scale crust, focal epidermal cell death, vasculitis?, Infiltrate = T cell often monoclonal