Lupus

Question 1

Definition & Pathogenesis

Answer 1

Autoimmune disease due to susceptible genes + environmantal factors
cells undergo apoptosis and cell DNA, histones and other proteins become targets for autoantibodies like antinuclear ab + less clearance of these apoptotic molecules
they form complexes and travel throughout the body causing local inflammatory reaction, they also activate complement systems (Type III reaction)
Can present with fecer and arthritis, malaise, weight loss & rash

Question 2

Triggers

Answer 2

Genes, Uv radiation, medications, estrogen

Question 3

Criteria (DOPMAINE RASH)

Answer 3

need 4 or more:

• skin: 1) malar rash 2) discoid rash 3) photosensitivity
• Mucosa: 4) ulcers: mouth/nose 5) serositis (pleuritis, pericarditis, libman sacks endocarditis, …)
• joints: 6) arthritis (2 or >)
• kidney: 7) renal disorders (proteinuria, DPGN, ..)
• Brain: 8) neurologic sympotms :seizure psychosis
• hematologic: 9) anemia, thrombocytopenia, leukopenia
• Antibodies: ANcA, Anti-smith, Anti-dsDNA, Anti-phopholipid (cardiolipin, lupus ab, anti B2GP1)

Question 4

Acute cutaneous lupus erythematosus

Answer 4

Most commonly associated with SLE
predict the recurrence of systemic disease or prognosis of the disease 
ANCA
systemic + 7% butterfly malar rash
sun exposure

Question 5

Subacute cutaneous lupus

Answer 5

related to medications (CCB, Phenytoin, INH, NSAIDS, ACEI, tetracyclines and sun exposure (Photo aggravated dermatoses)
doesn’t scar
50% chance of having SLE but not severe
epidermis and upper dermis
anti-Ro/SSA autoantibodies (ab against cytoplasmic proteins )

Question 6

Discoid

Answer 6

scars & need early treatment
epidermis, upper and lower dermis, and adnexal structures (chronic)
limited to skin with IgG, IgM deposited at the dermal-epidermal junction and no ab
paresthesia
may be found on the ears, face, trunk, and extremities

Question 7

Type of rash in subacute LE

Answer 7

Erythematous papules and scaly, hyperkeratotic, annular/ polycyclic plaques, bright red annular
appears suddenly
mainly affects the upper trunk and dorsi of the hands

Question 8

Type of rash in discoid LE (early & late)

Answer 8

early lesions are sharply demarcated erythematous scale papules and plaque, follicular plugging, and early scarring
Late lesions: atrophic plaques with central scarring, telangiectasis, and hypopigmentation
CDLE on the scalp: oval, scarring area of alopecia due to fibrosis. Active erythematous margin and a white atrophic inactive center

Question 9

Diagnosis

Answer 9

Antinuclear antibodies, Anti DNA antibodies
CBC + urinalysis
Skin histology: epidermal atrophy, hyper/paraceratosis, edema, vaculisation, inflammatory infiltrate
direct immunofluorescence (DIF): IgG deposits on basal layer

Question 10

Local Treatment

Answer 10

• Sun protection
• Topical and intralesional corticosteroids
• Topical calcineurin inhibitors
• Topical retinoids

Question 11

Systemic treatment

Answer 11

• Low dose corticosteroids
• NSAIDs
• Azathiopine
• Hydroxychloroquine (po qd–bid)- antimalarial
• Chloroquine (po qd)- antimalarial drug
• Quinacrine (po qd)-combination of the 2
If there is obvious renal and CNS involvement or significant systemic upset, then high-dose pulsed
methylprednisolone may be required