SLE Flashcards
What is the lupus band test
Immunohistology of the skin. IgG, IgM, and C3 deposition is seen along the dermal-epidermal junction.
Chronic discoid lupus
- test is positive in active lesions, negative in burned out lesions and normal skin.
Systemic Lupus erythematosus
- test is positive in all skin, with or without lesions.
Treatments for chronic discoid lupus
Prophylactic sunscreen
Topical corticosteroids
Systemic antimalarial drugs- Hydroxychloroquine or Quinacrine
Systemic Acitretin for hyperkeratotic lesions
Disseminated, resistant forms: Azathioprine, systemic corticosteroids.
Categories of Lupus erythematosus
Systemic LE
Cutaneous LE
- Acute
- Subacute
- Chronic Cutaneous LE
Types of chronic cutaneous LE- discoid hypertrophi/verrucous Profundus Panniculitis Mucosal discoid LE Lupus timidus Chilbain LE Lichenoid LE
Characteristics of Chronic discoid lupus lesions
Slowly evolving
Papules evolving into oval or round plaques with sharp borders
Adherent scaling
Hyperkeratotic border with central atrophy and burned out scarring
Scarring alopecia due to follicular hyperkeratosis and pulgging.
Located on face and and sun-exposed regions.
Lesions last for months to years and may or may not itch
Histology characteristics of discoid lesions
Marked basal cell layer degeneration and vacuolization.
Perivascular Lymphocytic cell infiltration.
Mild hyperkeratosis.
Follicular hyperkeratosis and plugging.
Positive lupus band test in active lesions, not in burned out lesions.
Gross characteristics of Subacute cutaneous lupus rash
Acute onset rash usually in spring or summer.
Provoked by UV light
More widespread than the chronic discoid type.
Plaques are more inflammed, and there is no atrophy.
Plaques can be one of two types
1) Psaoriasiform
2) Annular
both are bright red, but annular lesions ahve central regression and little scaling.
Treatment for Subacute cutaneous lupus erythematosus
Topical glucocorticoids partially effective
Topical Tacrolimus and pimecrolimus partially effective
Systemic thalidomide very effective for skin lesions
Systemic Hydroxychloroquine, and Quinacrine
For systemic LE symptoms: Prednisone plus other immunosuppressants as involvement becomes more severe.
Methotrexate for joint pain and flares Severe flares: I.v. cyclophosphamide and high dose prednisolone Mycophenolate Rituximab Azathioprine
How is SLE diagnosed?
Patient has at least 4 of the clinical or laboratory criteria, and at least one of each type
or
Patient has biopsy-proven lupus nephritis as well as positive anti-nuclear or anti-DNA antibodies.
What are the clinical criteria for SLE?
11
the SLICC classification criteria
1) Acute cutaneous lupus - light sensitive erythematous malar rash
2) Chronic cutaneous lupus - discoid chronic rash with lesions in three stages, erethematous, keratotic, and scarred. Face, scalp, and chest.
3) Non-scarring alopecia, body hair loss
4) Oral or nasal mucocutaneous ulcers
5) Serositis - pericarditis or pleuritis/pleural effusion.
6) Synovitis - joint pain in two or more joints, or multiple tender and stiff joints in the morning lasting for 30 minutes.
7) Urine - Proteinuria or RBC casts in the urine
8) Neurologic SLE symptoms: CNS- seizures, psychosis, myelitis (CNS inflammation) or PNS- Cranial or peripheral neuropathy
9) Hemolytic anemia
10) Leukopenia at least once
11) Thrombocytopenia at least once.
What are the Immunologic criteria for SLE?
1) ANA’s Anti-nuclear antibodies. Very sensitive, in 95% of SLE. But not specific, seen in normal individuals as well.
2) Anti-dsDNA
3) Anti-Smith srn-RNA antibodies
4) Anti-phospholipid abs
5) low complement levels - indicating complement activation
6) Positive direct Coombs test.
SLE typical patient
A woman of childbearing age
9:1 ratio female to males.
More common in aftrican-carribeans, and asians.
Goes through Relapsing, Remitting phases, flares of symptoms of the disease
Drug induced lupus, what causes it
what is the hallmark antibody of DIL
Many drugs can induce it.
Most frequent: Hydralazine, Isoniazid, and Procainamide
(these drugs are acetylated in the liver)
It is typically reversible with drug removal.
95% will have anti-Histone antibodies.
Other symptoms of SLE not mentioned in the clinical criteria
Generalized symptoms: Fatigue Fever Weight loss Chronically weak immune system and frequent infections.
Skin symptoms:
ACLE, SCLE, CCLE: acute, subacute, and chronic skin rash.
Erythema exsudativum multiforme - exudating skin rash, face and mouth.
Rowell s yndrome, annular/ringed-rimmed rash all over body.
Cardiac symptoms:
In addition to pericarditis and effusion-
Libman-Sacks endocarditis of the Mitral valve.
- non infective vegetations of fibrin and immune cells
Myocarditis - life threatening, arrythmia possibility
Vasculitides:
Raynaud’s syndrome and cold agglutinins
Vasculitis and petechial/purpural rashes.
Livedo reticularis
Antiphospholipid syndrome: CLOTS Coagulable state Livedo reticularis Obstetric miscarriages Thrombocytopenia
GI symptoms:
Nausea, vomitting
Peptic/gastric ulcers
Eye symptoms:
Conjunctivitis
SKIN LESIONS of acute SLE
Butterfly/malar rash
Generalized papular or urticarial rash on the sun exposed skin.
Bullae - can be hemorrhagic
Discoid type plaques as in SCLE and CCLE
Palmar erythema on fingertips - pathognomonic for acute SLE
Dorsal scaly erythematous plaques on the hands.
Nailfold telangiectasia - most heavily seen in scleroderma, but also in SLE and dermatomyositis.
Urticarial lesions
Palpable purpural lesions - vasculitis.
Discoid lesions causing patchy allopecia.
Mucous membrane ulcers, from necrotic purpural lesions.
Describe the butterfly rash in derma terms.
Butterfly rash - confluent, erythematous, macular, with fine scaling.
