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Connective Tissue Diseases of the Skin

Connective Tissue Diseases of the Skin. MBChB IV. Outcomes for this lecture. After this lecture the student should be able to: Recognise and describe scleroderma Recognise and describe the different types of scleroderma

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Connective Tissue Diseases of the Skin

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  1. Connective Tissue Diseases of the Skin MBChB IV

  2. Outcomes for this lecture • After this lecture the student should be able to: • Recognise and describe scleroderma • Recognise and describe the different types of scleroderma • Recognise and describe the symptoms and signs of systemic sclerosis (cutaneous and systemic) • Name and apply the investigations that has to be done in cases of scleroderma (clinical, side room tests, imaging and laboratory tests) • Broadly name the management of scleroderma • Recognise and describe in detail the clinical presentation of chronic discoid lupus erythematosus (DLE) • Broadly describe the management of DLE • Be aware of the condition named subacute cutaneous lupus erythematosus • Recognise and describe in detail the skin signs of acute systemic lupus erythematosus (SLE) • Name the systemic involvement of SLE and recognise and describe the symptoms and signs of systemic involvement • Name the serological tests that may be done to further investigate SLE and discuss the implications thereof • Name the special investigations (imaging and laboratory tests) that have to be done to investigate SLE • Describe in broad terms the management of SLE • Recognise and describe the clinical presentation of dermatomyositis (skin and muscle) • Recognise and describe the clinical presentation of cutaneous vasculitis and rheumatoid nodules

  3. Connective Tissue Diseases with Skin Lesions • Lupus erythematosus • Scleroderma • Dermatomyositis • Rheumatoid arthritis • Reiter’s syndrome

  4. Scleroderma • Variants • In common: Fibrosis / sclerosis of dermis Cutaneous vascular insufficiency • Sometimes systemic fibrosis

  5. Variants of Scleroderma • Systemic sclerosis • CREST • Morphoea • Pseudoscleroderma – porphyria lipodermatosclerosis

  6. Systemic Sclerosis • Raynaud’s phenomenon • Sclerodactyly • Diffuse scleroderma • Small oral orifice • Sharp tip of nose • Telangiectasia • Calcification Plus: Arthritis, esophageal dysmotility, lung fibrosis, hypertension, etc

  7. CREST • Calcifications (forearms) • Raynaud’s phenomenon • Esophageal dysmotility • Sclerodactyly • Telangiectasia By definition: No other systemic involvement

  8. Morphoea • Localised plaques of scleroderma • Seldom systemic involvement • Begins as pink-purple macule • Later central induration / hardening • Violaceus edge persists • Epidermis sometimes shiny, atrophic, depigmented, spotty • Sometimes deep atrophy

  9. Variants of Morphoea • Usual plaques (one or many) • Liniar / segmental • Central forehead (en coup de sabre) • Hemifacial involvement

  10. Further Examination • Skin lesions – helps to predict prognosis • Signs of arthritis • Blood pressure • Signs of lung fibrosis • Urine microscopy

  11. Special Investigations • Serology: ANF, ENAs Anti-Scl-70 • FBC • Renal functions • Chest X-rays • Lung functions • Barium swallow • Skin biopsy

  12. Management: Therapeutic • Vasodilators – prazocin nifedipine • Corticosteroids systemically – early • PUVA • NSAIDS • Cimetidine / PPIs • Chloroquine • Methotrexate • Cyclosporine A

  13. Management: Referral • Rheumatology • Nephrology

  14. Management: Advice / Information • Warn that blood pressure control is crucial • Minimize cold exposure • Soft diet if dysphagia present

  15. Management: Follow-up • Regularly – 3 monthly • Ask about arthralgia, dysphagia, dyspnea • Measure blood pressure • Tests urine • Lung functions

  16. Lupus Erythematosus • Chronic (Discoid LE) • Subacute LE • Systemic LE

  17. Discoid LE • Most common • Extremely chronic and resistant, mutilating • Photo areas • 5% Systemic involvement • Early diagnosis crucial

  18. Discoid LE • Erythema • Deep induration / firmness • Hyperkeratotic scaling • Follicular plugs • Atrophy • Edge of hyperpigmentation (grey, black) • Hair loss (scarring) • Cheilitis

  19. Discoid LE: Management • Sun protection • Topical steroids (potent) • Chloroquine • Exclude systemic involvement

  20. Subacute Cutaneous LE • Photosensitive disease • Face, shoulders, back, arms • Annular / psoriasiforme • Little / no atrophy • ENAs positive (anti-Ro, anti-La) • Arthritis • Brain -, lung involvement, not nephropathy

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