Chronic cutaneous lupus erythematosus (CCLE)

Question 1

______ accounts for the majority of CCLE.

Answer 1

• Discoid lupus

Question 2

What percent of discoid lupus patients prgress to SLE?

Answer 2

- 5-20%

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

Question 3

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

Answer 3

5% (if diffuse involvement then 20%)

Question 4

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

Answer 4

20%

Question 5

What are the triggers/risk factors for CCLE?

Answer 5

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

Question 6

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

Answer 6

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

Question 7

What are the clinical subtypes of CCLE?

Answer 7

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

Question 8

Describe the lesions of discoid lupus:

Answer 8

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

Question 9

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

Answer 9

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

Question 10

What are the typical locations DLE lesions present?

Answer 10

• face, scalp, conchal bowl

Question 11

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

Answer 11

• higher

Question 12

Hypertrophic LE is a subtype of CCLE that presents as:

Answer 12

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

Question 13

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

Answer 13

SCC

Question 14

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

Answer 14

• Hypertrophic LE upper half
• HLP lower half

Question 15

Chilblain lupus erythematosus presents as:

Answer 15

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

Question 16

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

Answer 16

• persist year round

Question 17

Tumid LE presents with:

Answer 17

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

Question 18

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

Answer 18

• Jessner’s
• Reticular erythematous mucinosis

Question 19

Lupus erythematosus panniculitis/profundus presents as:

Answer 19

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

Question 20

What percent of lupus panniculitis is a/w SLE?

Question 21

Mucosal LE presents as:

Answer 21

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

Question 22

Histopath of discoid lupus?

Answer 22

• 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

Question 23

Hypertrophic LE histopath:

Answer 23

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

Question 24

Chilblain LE histopath:

Answer 24

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

Question 25

Tumid lupus histopath:

Question 26

LE panniculitis histopath:

Question 27

Treatment for CCLE if localized disease:

Answer 27

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

Question 28

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

Answer 28

-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)