Chronic cutaneous lupus erythematosus (CCLE) Flashcards
______ accounts for the majority of CCLE.
- Discoid lupus
What percent of discoid lupus patients prgress to SLE?
- 5-20%
- but 40-70% of SLE patients will have discoid lesions
If there is only head involvment of DLE, what percent will progress to SLE?
5% (if diffuse involvement then 20%)
If there is diffuse involvement of DLE, what % of patients will progress to SLE?
20%
What are the triggers/risk factors for CCLE?
- UV radiation is important trigger
- Tobacco smoking is risk factor (stopping may help treat recalcitrant lesions)
- Genetics
What are some of the cells involved in pathogenesis of CCLE?
- Type I interferon with CD4 + Th1 cells and CD8 + recruitment and activation
What are the clinical subtypes of CCLE?
- Discoid LE
- Hypertophic (verrucous) LE
- Chilblain LE
- Tumid LE
- LE panniculitis/profundus
- Discoid lupus/lichen planus overlap
- Mucosal LE
Describe the lesions of discoid lupus:
- start as red macules or plaques–> develop scale, atrophy and scarring w/ central hypopigmentation and periphral hyperpigmentation
What findings can you see on the scale of DLE lesions?
- Langue du chat= carpet “tack-like” spines on undersurface of scale
What are the typical locations DLE lesions present?
- face, scalp, conchal bowl
Childhood DLE has a (higher or lower) chance to progress to SLE?
- higher
Hypertrophic LE is a subtype of CCLE that presents as:
- thick, hyperkeratotic and verrucous scaling plaques w/ indurated border
similar to hypertrophic LP, hypertrophic LE patients have increased risk of ____
SCC
Hypertrophic LE favors (upper/lower half) of the body, while hypertrophic LP favors (upper/lower half)
- Hypertrophic LE upper half
- HLP lower half
Chilblain lupus erythematosus presents as:
- painful, red or dusky purple papules/plaques on fingertips, rims of ears, calves and heels
Chilblain LE is precipitated by cold, but unlike non-LE chilblains tends to_____
- persist year round
Tumid LE presents with:
- edematous, indurated, erythematous, often annular plaques without epidermal involvement
- typical location is face and trunk
Tumid LE is considered to be on a spectrum with what two other diseases?
- Jessner’s
- Reticular erythematous mucinosis
Lupus erythematosus panniculitis/profundus presents as:
- indurated, non-tender, subcutaneous nodules or plaques that heal with subcutanous atrophy (depressions in skin)
What percent of lupus panniculitis is a/w SLE?
Mucosal LE presents as:
- classic plaque w/ central erythema and surrounding white keratotic border usually on hard palate.
Histopath of discoid lupus?
- 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
Hypertrophic LE histopath:
- similar to DLE but greater orthohyperkeratosis, endophytic buds of hyperplastic follicular epithelium
Chilblain LE histopath:
- features of Chilblains (papillary edema, perivascular and dermal lymphohistiocytic infiltration) and DLE
Tumid lupus histopath:
LE panniculitis histopath:
Treatment for CCLE if localized disease:
- sunscreen!
- topical CS
- if this does not control, then you can go for systemic agents like HCQ
Treatment for CCLE with w/ severe and widespread skin manifestations:
-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)
