Chronic cutaneous lupus erythematosus (CCLE) Flashcards

1
Q

______ accounts for the majority of CCLE.

A
  • Discoid lupus
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2
Q

What percent of discoid lupus patients prgress to SLE?

A

- 5-20%

- but 40-70% of SLE patients will have discoid lesions

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3
Q

If there is only head involvment of DLE, what percent will progress to SLE?

A

5% (if diffuse involvement then 20%)

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4
Q

If there is diffuse involvement of DLE, what % of patients will progress to SLE?

A

20%

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5
Q

What are the triggers/risk factors for CCLE?

A
  • UV radiation is important trigger
  • Tobacco smoking is risk factor (stopping may help treat recalcitrant lesions)
  • Genetics
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6
Q

What are some of the cells involved in pathogenesis of CCLE?

A
  • Type I interferon with CD4 + Th1 cells and CD8 + recruitment and activation
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7
Q

What are the clinical subtypes of CCLE?

A
  • Discoid LE
  • Hypertophic (verrucous) LE
  • Chilblain LE
  • Tumid LE
  • LE panniculitis/profundus
  • Discoid lupus/lichen planus overlap
  • Mucosal LE
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8
Q

Describe the lesions of discoid lupus:

A
  • start as red macules or plaques–> develop scale, atrophy and scarring w/ central hypopigmentation and periphral hyperpigmentation
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9
Q

What findings can you see on the scale of DLE lesions?

A
  • Langue du chat= carpet “tack-like” spines on undersurface of scale
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10
Q

What are the typical locations DLE lesions present?

A
  • face, scalp, conchal bowl
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11
Q

Childhood DLE has a (higher or lower) chance to progress to SLE?

A
  • higher
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12
Q

Hypertrophic LE is a subtype of CCLE that presents as:

A
  • thick, hyperkeratotic and verrucous scaling plaques w/ indurated border
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13
Q

similar to hypertrophic LP, hypertrophic LE patients have increased risk of ____

A

SCC

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14
Q

Hypertrophic LE favors (upper/lower half) of the body, while hypertrophic LP favors (upper/lower half)

A
  • Hypertrophic LE upper half
  • HLP lower half
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15
Q

Chilblain lupus erythematosus presents as:

A
  • painful, red or dusky purple papules/plaques on fingertips, rims of ears, calves and heels
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16
Q

Chilblain LE is precipitated by cold, but unlike non-LE chilblains tends to_____

A
  • persist year round
17
Q

Tumid LE presents with:

A
  • edematous, indurated, erythematous, often annular plaques without epidermal involvement
  • typical location is face and trunk
18
Q

Tumid LE is considered to be on a spectrum with what two other diseases?

A
  • Jessner’s
  • Reticular erythematous mucinosis
19
Q

Lupus erythematosus panniculitis/profundus presents as:

A
  • indurated, non-tender, subcutaneous nodules or plaques that heal with subcutanous atrophy (depressions in skin)
20
Q

What percent of lupus panniculitis is a/w SLE?

A
21
Q

Mucosal LE presents as:

A
  • classic plaque w/ central erythema and surrounding white keratotic border usually on hard palate.
22
Q

Histopath of discoid lupus?

A
  • vacuolar interface dermatitis w/ necrotic keratinocytes and pigment incontinence
  • epidermal atrophy

- BMZ thickening

-follicular plugging

- superficial and deep perivascular lymphohistiocytic inflammation with plasma cells

- mucin deposition

23
Q

Hypertrophic LE histopath:

A
  • similar to DLE but greater orthohyperkeratosis, endophytic buds of hyperplastic follicular epithelium
24
Q

Chilblain LE histopath:

A
  • features of Chilblains (papillary edema, perivascular and dermal lymphohistiocytic infiltration) and DLE
25
Q

Tumid lupus histopath:

A
26
Q

LE panniculitis histopath:

A
27
Q

Treatment for CCLE if localized disease:

A
  • sunscreen!
  • topical CS
  • if this does not control, then you can go for systemic agents like HCQ
28
Q

Treatment for CCLE with w/ severe and widespread skin manifestations:

A

-sunscreen

+ topical CS

+ HCQ + systemic CS (when active disease)

  • if these do not work can add: Quinacrine, then MTX, then Retinoids or Dapsone (see treatment ladder on pg 103)