41 - Lupus eritematoso

Question 1

Manifestações extra-cutâneos do LE neonatal?

Answer 1

• bloqueio cardíaco congénito (com ou sem MCP),
• doença hepatobiliar (varia desde falência hepática na gestação ou neonatal; hiperBrb conjugada nas primeiras semanas; ou elevação das transamineses nos 2-3 meses de vida),
• citopenia (+++ trombocitopenia).

Question 2

Critérios SLICC LE Sistémico?

Answer 2

Clinical Criteria:
1. Acute cutaneous lupus or Subacute
2. Chronic cutaneous lupus
3. Oral ulcers: palate
4. Nonscarring alopecia (diffuse thinning or hair fragility with visible broken hairs)
5. Synovitis involving two or more joints, characterized by swelling or effusion OR
tenderness in two or more joints and thirty minutes or more of morning stiffness.
6. Serositis
7. Renal (proteina/albumina >500mg/24h ou cilindros de eritrócitos).
8. Neurologic
9. Hemolytic anemia
10. Leukopenia (< 4000/mm3 at least once) or Lymphopenia (< 1000/mm3 at least once)
11. Thrombocytopenia (<100,000/mm3) at least once.

Immunological Criteria

1. ANA above laboratory reference range
2. Anti-dsDNA above laboratory reference range, except ELISA (twice above normal level)
3. Anti-Sm
4. Antiphospholipid antibody: any of the following
   - anticoagulante lupico, falso positivo RPR, anti-cardiolipinas, anti-B2-glicoproteina.
5. Low complement (C3, C4, CH50).
6. Direct Coombs test in the absence of hemolytic anemia

Question 3

DD de LE agudo/ rash malar?

Answer 3

• Rosacea (including corticosteroid-induced)
• Seborrheic dermatitis (especially photosensitive form)
• Sunburn
• Drug-induced photosensitivity (phototoxic and photoallergic)
• Dermatomyositis
• Erysipelas
• Pemphigus erythematosus and foliaceus
• Dermatitis – atopic, contact and photocontact

Question 4

DD de LE subagudo?

Answer 4

• Psoriasis
• Dermatophytosis, including tinea incognito
• Photolichenoid drug eruption
• Polymorphous light eruption
• Granuloma annulare
• Figurate erythemas (e.g. erythema annulare centrifugum)
• Dermatitis – atopic, contact and photocontact
• Pemphigus foliaceus

Question 5

DD de LE discoide?

Answer 5

• Facial discoid dermatosis
• Tinea faciei, tinea capitis, tinea corporis, tinea (pityriasis) versicolor
• Lichen planus and lichen planopilaris
• Sarcoidosis
• Granuloma faciale
• Dimorphic fungal infections, lupus vulgaris
• Follicular mucinosis (alopecia mucinosa)
• When single lesion, non-melanoma skin cancer

Question 6

DD de LE túmido?

Answer 6

• Lymphocytic infiltrate of Jessner
• Polymorphous light eruption
• Cutaneous lymphoid hyperplasia (pseudolymphoma of the skin)
• Reticular erythematous mucinosis

Question 7

Exames laboratoriais no LE?

Answer 7

EVALUATION FOR SYSTEMIC LUPUS ERYTHEMATOSUS
1) History and review of systems

2) Physical examination
•Specific cutaneous lesions (see Tables 41.4 and 41.5)
•Nonspecific cutaneous lesions (see Table 41.6)
•Lymphadenopathy, arthritis

3) Laboratory tests
•ANA with profile (anti-dsDNA, -Sm)
•Urinalysis
•CBC with differential, platelet count
•Chemistries (BUN, creatinine)
•ESR, CRP
•Complement levels (C3, C4)
•Anti-phospholipid antibodies

Question 8

ANAs no LE?

Answer 8

Autoantibodies to dsDNA, Sm and ribosomal P are relatively specific for SLE, and are, therefore, helpful indicators of a high likelihood of systemic disease.

Autoantibodies to SSA/Ro, SSB/La, U1RNP, histones and ssDNA are common in patients with SLE, but they are not disease-specific.

An ANA is helpful if negative, as it is quite unusual for patients with SLE to have a negative ANA. It is common for the ANA to be positive in patients with cutaneous lesions, but a positive ANA is neither an indicator of systemic disease nor of LE.

Positive ANAs may be found in patients with many other diseases and even in apparently normal individuals. Approximately a third of apparently normal individuals have a positive ANA at a dilution of 1 : 40, 13% at a dilution of 1 : 80, and 5% at a dilution of 1 : 160.