11th lecture autoimmune ds
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This document discusses connective tissue diseases, specifically lupus erythematosus and scleroderma. It describes the different types of lupus, including discoid lupus erythematosus and systemic lupus erythematosus, and their characteristic manifestations, histology, and treatment. It also discusses scleroderma, differentiating between morphoea, systemic sclerosis, and chemically induced scleroderma. Systemic sclerosis is described in more detail, outlining its cutaneous features, systemic involvement affecting organs like the lungs and kidneys, and current limited treatment options.
11th lecture autoimmune ds
- 2. Lupus erythematosus: • an autoimmune disorder • two main forms: (DLE), (SLE), (SCLE) • small proportion of patients with DLE may subsequently develop SLE. • The pathogenesis :a combination of genetic factors predisposing to autoimmunity and environmental agents, such as viral and bacterial antigens, acting as triggering factors • Drug‐induced SLE.
- 3. Discoid lupus erythematosus (DLE): • affects light‐exposed areas, the face and neck, dorsa of the hands and the arms. • Individual lesions : scaling, erythematous plaques, with prominent follicular plugging. Lesions heal with hypopigmented, atrophic scar. • The scalp may be involved: scarring alopecia • Occasionally, the buccal or nasal mucosae are affected. • Investigations: diagnosis confirmed by skin biopsy.
- 4. DLE: • Histology : • a lymphocytic infiltrate around blood vessels, follicles and sweat glands • damage to the basal layer of the epidermis • follicular plugging and hyperkeratosis. • Direct immunofluorescence (DIF) of lesional skin • Treatment: • Steroid: topical steroids , intralesional • oral therapy with the antimalarial hydroxychloroquine • severe extensive: oral steroids, azathioprine and retinoids • sunscreen with a high sun protection factor (SPF), cosmetic camouflage
- 5. DLE
- 6. Discoid lupus erythematosus: typical lesion on the buccal mucosa Discrete red and white plaque on the hard palate in a patient with discoid lupus erythematosus.
- 7. Systemic lupus erythematosus (SLE): • multisystem disorder • Typically, the disease affects women, particularly of childbearing age • series of exacerbations and remissions.
- 8. Manifestations of systemic lupus erythematosus: • Pyrexia, weight loss and general malaise • facial erythema in a ‘butterfly’ distribution • photosensitivity. • diffuse alopecia • Mucocutaneous lesions include oropharyngeal ulceration. • Polyserositis: Arthralgia and arthritis ,Pericarditis, Pleurisy with effusions. • Nephritis • CNS:Psychosis and convulsions. • Haematopoietic abnormalities:Haemolytic anaemia, Leukopenia, Thrombocytopenia. • Raynaud’s phenomenon, vasculitis
- 9. facial erythema in a ‘butterfly’ distribution
- 10. facial erythema in a ‘butterfly’ distribution Oral hematomas Oral ulcers
- 11. Investigations: • full blood count and biochemistry • complement levels • Urinalysis • ECG and chest radiograph. • autoantibodies : (ANAs) ,(anti‐dsDNAs) • +ve RF and biological false‐positive serological tests for syphilis may also be found. • DIF.
- 12. Treatment: • Systemic steroids and immunosuppressive agents are the mainstay of treatment. • Light‐exposed areas of skin should be protected by sunscreens with a high SPF.
- 13. Scleroderma
- 14. • Scleroderma = thickening of the skin • Classification of scleroderma: • Morphoea: sclerosis of the skin without systemic involvement. • Systemic sclerosis: cutaneous sclerosis + MSO. • Chemically induced scleroderma: sclerosis of the skin as a manifestation of the toxic effects of certain chemicals. • Pseudoscleroderma: sclerosis of the skin associated with a number of diseases other than morphoea or systemic sclerosis.
- 15. Systemic sclerosis: • an autoimmune connective tissue disease of unknown aetiology. • Sclerotic changes in the skin • multisystem disorder associated with a vasculopathy of small arteries. In the most common form skin changes affect • predominantly the face and hands.
- 16. • Cutaneous features of systemic sclerosis: • Face: • a tight, shiny appearance, with loss of facial wrinkles • a beaked nose and restriction of mouth opening. • Perioral furrowing (‘purse‐string mouth’). • Facial telangiectasia. • Loss of lip vermilion. • Hands • Raynaud’s phenomenon. • sclerodactyly. • Finger pulp small, painful ulcers and progressive pulp atrophy and resorption of the underlying terminal phalanges. • Calcinosis.
- 17. Cutaneous features of systemic sclerosis facial appearance perioral telangiectases finger pulp uclers and scars
- 18. Systemic involvement: • Gastrointestinal: Dysphagia is the result of oesophageal involvement • Pulmonary: An inflammatory alveolitis , pulmonary fibrosis, pulmonary hypertension and cor Pulmonale • Renal: with proteinuria and hypertension, renal failure. • Nervous system • Cardiac: ECG abnormalities have been described. • Hepatic • Musculoskeletal: Arthralgia and arthritis occur in some patients, and myopathy and inflammatory myositis may also occur
- 19. •Treatment: •No therapy is known to alter the overall course of systemic sclerosis •Prognosis: •Severe pulmonary or renal involvement is a poor prognostic factor, although treatment is improving, but most patients with systemic sclerosis live for many years.