2. EL MODELO DE LAS INTELIGENCIAS MÚLTIPLES (IM) DE HOWARD
2.2. ANTECEDENTES TEÓRICOS DE REFERENCIA EN LA ESCUELA
2.2.2. Autores y propuestas relevantes para el enfoque de las IM
SLE a multisystem disorder, primarily affects skin, joints and vascular system. The age of onset is usually between 16 and 42 years occurring more frequently in females (F: M:: 8:1).
Etiology
• Exact cause is unknown but there is evidence to suggest the role of genetic, immune and various environmental factors.
• It has been postulated that four or more genes are involved in predisposing an individual to SLE.
Pathogenesis
LE is a multifactorial disease with genetic and immunopathologic abnormalities. The release of nuclear antigens because of enhanced apoptosis is a key factor.
Important predisposing factors are genetic predisposition (HLA-B8, DR2, DR3, DQwl, DRB1), complement defects, exogenous factors (UV radiation, and medications), and individual factors (hormone status, altered immune status).
The 1982 Revised Criteria for Diagnosis of SLE are:
1. Malar rash: Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds (Fig. 14.7).
2. Discoid rash: Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions.
Fig. 14.7: Systemic lupus erythematosus—malar and photosensitive rash over the face
3. Photosensitivity: Skin rash as a result of unusual reaction to sunlight, by patient history or physician observation.
4. Oral ulcers: Oral or nasopharyngeal ulceration, usually painless, observed by a physician.
5. Arthritis: Nonerosive arthritis involving two or more peripheral joints, characterized by tenderness, swelling, or effusion. or
6. Serositis:
a. Pleuritis—convincing history of pleuritic pain or rub heard by a physician or evidence of pleural effusion.
b. Pericarditis—documented by ECG or rub or evidence of pericardial effusion.
7. Renal disorder:
a. Persistent proteinuria > 0.5 g/day or greater than 3+ if quantitation not performed or
b. Cellular casts—may be red cell, hemoglobin, granular, tubular, or mixed.
8. Neurologic disorder:
a. Seizures—in the absence of offending drugs or known metabolic derangements, e.g. uremia, ketoacidosis, or electrolyte imbalance or
b. Psychosis—in the absence of offending drugs or known metabolic derangements, e.g. uremia, ketoacidosis, or electrolyte imbalance.
9. Hematologic disorder:
a. Hemolytic anemia—with reticulocytosis b. Leukopenia < 4000/mL on two or moreor
occasions or
c. Lymphopenia < 1500/mL on two or more occasions or
d. Thrombocytopenia—<100,000/mL in the absence of offending drugs.
10. Immunologic disorder:
a. Anti-DNA—antibody to native DNA in abnormal titer or
b. Anti-Sm—presence of antibody to Sm nuclear antigen or
c. Positive finding of antiphospholipid antibodies based on (1) an abnormal serum level of IgG or IgM anticardiolipin antibodies, (2) a positive test result for lupus anticoagulant using a standard method, or (3) a false-positive serologic test for syphilis known to be positive for at least 6 months and confirmed by Treponema pallidum immobilization or fluorescent treponemal antibody absorption test.
11. Antinuclear antibody (ANA): An abnormal titer of antinuclear antibody by immuno-fluorescence or an equivalent assay at any point in time and in the absence of drugs known to be associated with “drug-induced lupus” syndrome.
Note: The proposed classification is based on 11 criteria. For the purpose of identifying patients in clinical studies, a person shall be said to have SLE if any 4 or more of the 11 criteria are present, serially or simultaneously, during any interval of observation.
Chief Cutaneous Features
• Butterfly rash.
• Photosensitivity.
• Raynaud’s phenomenon.
• Non-scarring alopecia-short hairs in the frontal region are referred to as lupus hairs.
• Urticarial vasculitis.
• Mouth ulceration.
• Bullous lesions.
• Chronic discoid LE lesion.
• Cutaneous vasculitis.
• Other features are vasculopathy, periungual telangiectasia, leg ulcers, erythema multi-forme, thrombophlebitis, peripheral gangrene, etc.
Investigations
• Complete blood count, ESR.
• Urine analysis for microscopic hematuria and proteinuria.
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• Skin biopsy- There is no single diagnostic pathological feature in the skin, but a combination of features aids diagnosis. Some changes are similar to DLE. The primary pathological lesions of SLE are fibrinoid necrosis, collagen sclerosis, necrosis and basophilic body formation and vascular endothelial thickening.
• LE cell test to demonstrate LE cell (a neutrophil containing engulfed nuclear material) or rosette phenomenon (neutrophils surroun-ding nuclear debris, trying to engulf it).
• Lupus band test- deposits of IgG and C3 along the basement membrane on normal, non-sun exposed skin suggest SLE but is no longer an accepted criterion. It positively correlates with the presence of anti-ds DNA antibodies and with risk for developing LE nephritis.
• C3, C4, CH50 levels.
• Serum globulins are frequently raised especially gamma globulin.
• Rheumatoid factor occurs in approximate 40%
cases.
• ANA (Commonest pattern is homogeneous;
peripheral pattern-predictor of renal involvement).
• ndsDNA.
• Antibodies-SSA (Ro), SSB (La), Sm, nRNP.
Differential Diagnosis
SLE must be differentiated from dermatomyositis, erythema multiforme, polyarteritis nodosa, acute rheumatic fever, rheumatoid arthritis, pellagra, pemphigus erythematosus, drug eruption, hyperglobulinemic purpura, Sjogren’s syndrome, necrotizing angiitis and myasthenia gravis. In SLE, there may be fever, arthralgia, weakness, lassitude, diagnostic skin lesions, an increased ESR, cytopenias, proteinuria, immunoglobulin deposition at dermoepidermal junction and a positive ANA test. Biopsies of skin lesions and involved kidney may also be diagnostic.
Treatment
SLE with only cutaneous lesions and arthritis:
• Photoprotection.
• NSAID’s, antimalarials, prednisolone ( 1-2 mg per kg body weight daily).
• Dapsone most effective in urticarial vasculitis and bullous SLE.
Severe Disease with End-organ Damage
• Steroid pulse therapy.
• Immunosuppressants such as azathioprine (100-150 mg daily), cyclophosphamide (50-100 mg daily), methotrexate (7.5-20 mg weekly), chlorambucil, mycophenolate mofetil (2 gm daily) and cyclosporine (5 mg/kg body weight daily).
Experimental Therapies
• Intravenous immunoglobulins, plasma-pheresis, anti CD20 (rituximab), -CD40 and – TNF alpha antibodies.
Course and Prognosis
The course of the SLE is very variable. Acute fulminating cases are much less common than subacute cases, which smoulder on for many years.
SCLERODERMA
Scleroderma (Gr. Skleros – hard, and derma – skin) is a connective tissue disorder characterized by generalized or localized sclerosis of the skin. The localized type is called morphea.
Classification
Morphea – Localized (Circumscribed plaque, morphea profundus, bullous, linear, en coup de sabre) and generalized.
Systemic sclerosis – LM (Limited cutaneous) SSc, DC (Diffuse cutaneous) SSc.
Occupational scleroderma – Polyvinylchloride, perchlorethylene, trichloroethylene, organic solvents, malathion, DDT, epoxy resins, silicosis.
Iatrogenic scleroderma – Bleomycin, carbidopa, pentazocine, cocaine, appetite suppressants, silicone or paraffin implants, GVHD.
Pseudoscleroderma – Scleroedema of Buschke, Scleromyxoedema, prophyria cutanea tarda, phenylketonuria.
Primary systemic amyloidosis, carcinoid syndrome, hypothyroidism.
Miscellaneous – Toxic oil syndrome, eosinophilic fascitis.
MORPHEA
Localized Morphea
• Most common form of morphea.
• Occurs most commonly in females than males and primarily in young adults.
• Seen commonly on the trunk.
• The lesion of morphea may begin as erythematous macule, evolve into
ivory-colored center and violaceous bordered plaque.
• Lesions slowly involute over 3 to 5 years period leaving permanent atrophic skin or normal appearing skin behind.
Generalized Morphea
• Lesions are more numerous and larger
• Often coalesce to involve extensive portions of the body.
• Muscle atrophy may be associated.
Pansclerotic Morphea (Morphea Profunda)
• Sclerosis involves dermis, panniculus, fascia, muscle and bones.
• There is disabling limitation of the joints.
En Coup De Sabre
• It is a variant of linear scleroderma involving scalp parasagitally on frontal scalp and forehead (Figs 14.8 and 14.9).
• Often has the configuration of the stroke of a saber (en coup de sabre).
Differential Diagnosis
1. Morphea-like lesions can occur in sarcoidosis and morpheic basal cell carcinoma.
Figs 14.8 and 14.9: En coup de sabre—linear indurated depressed lesion in the midline over the forehead
Fig. 14.8 Fig. 14.9
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Histopathology is required to differentiate between them.
2. Lichen sclerosus atrophicus (ivory white plaques with follicular delling and atrophy) and subcutaneous zygomycosis may resemble morphea. Fingers can be insinuated below the plaques of subcutaneous zygomycosis.
3. Pseudoscleroderma especially porphyria cutanea tarda and graft versus host disease.
4. Drug reaction (bleomycin induced sclerosis, atrophic morphoeic plaques from intra-muscular injection of vitamin K or subcutaneous corticosteroid injections).
Treatment
• Natural history is towards spontaneous resolution.
• Topical steroid, intralesional steroids and oral chloroquine.
• Topical calcipotriol.
• Bath PUVA or UVA1.
• For widespread or rapidly advancing disease consider therapy usually reserved for systemic sclerosis (corticosteroids, d-penicillamine, cyclopsporine, low dose methotrexate, etretinate, phenytoin, plasmapheresis).
• Physiotherapy may be helpful in preventing joint deformities.