42- Lupus erythematosus

Question 1

What is the strongest risk factor that affects systemic lupus erythematosus?

Answer 1

• gender : women outnumber men by 6:1
• more common in childbearing ears.
• ethnicity is also a very strong risk factor.
• African americans develop disease at an earlier rate and have a higher frequency of nephritis, pneumonitis and discoid lesions.

Question 2

What factors are involved in the pathogenesis of lupus erythematosus

Answer 2

• ultraviolet radiation
• medications
• cigarette smoke
• maybe viruses

Question 3

What genes are the most strongly associated with SLE?

Answer 3

HLA-DR
PTPN22
STAT4
IRF5
TREX1
STAT4
C1Q

Question 4

What are the clinical manifestations of discoid luplus?

Answer 4

• lesions found on the face, scalp and ears.
• can be widespread (but unusual to be e on the body without being on the head/neck.
• patients may have lesions on sunprotected sites.
• dyspigmentatino is a common feature may look like vitiligo with hyperpigmentation around a depigmented area.

Question 5

What drugs are commonly affected with subacute cutaneous lupus erythematosus?

Answer 5

• terbinafine
• TNF inhibitors
• anti-epilepitcs
• proton pump inhibitors

Question 6

what are the clinical features of acute cutenous lupus?

Answer 6

• bialteral mara erythema
• follows sun exposure
• need to evaulate for evidence of internal disease
• may have telangiectasia, erosions, dyspigmentation and epidermal atrophy.
• knuckles are spared

Question 7

What is Rowell syndrome

Answer 7

• acute eruption of EM like lesionsin patients with lupus

Question 8

What are the features of lupus erythematosus tumidus?

Answer 8

• firm erythematous plaques that lack scale or follicular plugging.
• usually on the face or trunk.
• may have central clearing.

Question 9

what is the distribution of lupus panniulitis?

Answer 9

face, scalp,upper arms, upper trunk, breast, buttocks and thighs.

Question 10

What is the criteria for the diagnosis of SLE?

Answer 10

1. Serositis- pleuritis/pericarditis
2. Oral ulcerations
3. Arthritis
4. Photosensitivity
5. Blood abnormalities including leuopenia, lymphopenia, haemophytic anaemia, thrombocytopenia.
6. Renal involvement
7. ANA 1:160
8. Immunologic disorder: Anti-DNA antibody, Anti-Sm.
9. Neurological manifestations - seizures/psychosis
10. Malar rash
11. Discoid rash

Question 11

what are some non specific cutaneous lesions?

Answer 11

• vascular lesions: livedo reticularis, palmar erythema, nail fold telangiectasia. Vasculitis
• Raynauds

Question 12

What is the treatment ladder for Lupups

Answer 12

• topical or intralesiona; corticosteroids
  (repeated monthly while lesions are active).
• hydroxychloroquine. 200 mg once daily to twice daily.
• quinacrine if the patient is not responsive.
• may take 2-3 months for the efficacy to be appreciated
• oral retinoids, thalidomide or MMF, AZA, or MTX can work.
• Dapsone works well in bullous SLE.

Question 13

what are the histologic features of cutaneous LE?

Answer 13

DLE:
- basal cell damage
- lymphohistocytic inflammatory infiltrtes
- periadnexal inflammation
-follicular plugging
SCLE:
-- little no no hyperkeratosis

LE tumidus:
- prominent dermal mucin deposition and lympho

Question 14

what is the characteristic DIF finding in cutaneous lupus erythematosus?

Answer 14

• antibody deposition at the dermal-epidermal junction and around hair follicles.

usualy IgG or IgM.

in SCLE there may be depositions in the epidermis.