SKIN MANIFESTATIONS IN RHEUMATIC DISEASES AROMA 2011

1. SKIN MANIFESTATIONS INRHEUMATIC DISEASESAROMA 2011 • TarekMahmoud El Ghandour, MD • Professor of Dermatology, Andrology & STD’s. • Faculty of Medicine • Ain shams University

2. Systemic Lupus Erythematosus

3. Raynaud’s phenomenonis commonly found in lupus. It lack specificity. (a triphasic reaction of distal digits to cold or emotion, in which the skin colour changes from white to blue to red)

4. Dermatological involvement • Up to 85% of SLE • Butterfly rash • Maculopapular eruption • Discoid lupus • Relapsing nodular non-suppurativepanniculitis • Vasculitic skin lesin • Livedoreticularis • Purpuric lesions • Alopecia • Oral ulcer

5. Malar rash: This is a "butterfly-shaped" red rash over the cheeks below the eyes and across the bridge of the nose. It may be a flat or a raised rash.The rashes are made worse by sun exposure.

7. Systemic Lupus Erythematosus

8. Discoid lupus These are red, raised patches with scaling of the overlying skin.

10. Oral ulcer: Painless sores in the nose or mouth need to be observed and documented by a doctor.

11. Systemic Lupus Erythematosus • Discoid Lupus: Cutaneous manifestations • Scar upon healing

12. Maculopapular eruption

13. Vasculitic skin lesin

14. Alopecia

15. Systemic Lupus Erythematosus • Criteria for the Classification of SLE • Malar rash • Fixed erythema • Malar eminences • Sparing of the nasolabial fold • Discoid Rash • Erythematous plaques • Scaling, follicular plugging • Atrophic scarring

16. Systemic Lupus Erythematosus • Serositis • Pleuritis • Pericarditis • Oral ulcers • Arthritis • Nonerosive • Photosensitivity

17. Systemic Lupus Erythematosus • Blood/Hematologic • Hemolytic anemia • Leukopenia • Lymphopenia • Thrombocytopenia • Renal disorder • Persistent proteinuria >0.5 gm/day • Cellular casts • (+) ANA

18. Systemic Lupus Erythematosus • Immunologic • Anti-native DNA OR • Anti-Smith OR • Antiphospholipid antibody (ACA, B2GPI) • Lupus anticoagulant (DRVVT) • False (+) serologic test for syphilis • Neurologic • Seizures • Psychosis • Focal deficits

19. Systemic Lupus ErythematosusUnconventional Criteria • Multi-organ systemic inflammatory disease • AND • +ANA

20. Systemic Lupus Erythematosus • Other organ system involvement not part of the criteria: • GI • Peritonitis • Elevation of LFTs • Pancreatitis • Pulmonary • Acute lupus pneumonitis • Pulmonary hemorrhage • Pulmonary HTN • Shrinking lung syndrome

21. Systemic Lupus Erythematosus • ANA • + in >90% of patients • + in 25-33% of healthy normal population • Anti-dsDNA • Nephritis • Associated with flares • Anti-SSA/Ro and Anti-SSB/La • SLE, Sjogren’s syndrome • Neonatal lupus • SubacuteCutaneous Lupus

22. Systemic Lupus Erythematosus • Anti-Smith • More specific for a diagnosis of SLE • Anti-RNP • SLE • Mixed Connective Tissue Disease

23. Systemic Lupus Erythematosus • Treatment • Corticosteroids • Anti-malarials (i.ePlaquenil) • Azathioprine • Cellcept • Methotrexate, Cyclosporine • Cyclophosphamide • Plasmapheresis • IVIG • Rituxan

24. Drug-Induced Lupus • Drugs • Chlorpromazine • Hydralazine • Isoniazid • Methyldopa • Minocycline • Procainamide • Quinidine

25. Drug-Induced Lupus • Weeks to months after starting drug • Presentations • Constitutional symptoms • Joints • Pleuropulmonary • RARE – cutaneous, renal, neurologic • Rapid resolution of clinical features with d/c of drug, ANA may persist longer • Anti-histone antibodies

26. Systemic Sclerosis (SS) • Subsets • Diffuse SS • Limited SS • CREST syndrome – calcinosis, raynaud’s, esophageal dysmotility, sclerodactyly, telangiectasias • Overlap syndromes • Mixed Connective Tissue Disease • Localized scleroderma

27. Diffuse SclerodermaClinical Features

28. Diffuse SclerodermaClinical Features • Other features of diffuse SS: • Cardiac: diastolic dysfunction, arrhythmia • Renal crisis

29. Systemic Sclerosis (SS) • CREST syndrome

30. Systemic Sclerosis (SS)

31. Localised scleroderma(Morphea) • Localised injury • Tick bites – it has been associated withLyme diseasedue toBorreliaburgdorferiinfection • Pregnancy • Measlesand other viral infections • Autoimmune diseases includinglichen sclerosusandlichen planus

32. Localised SclerodermaClinical Features Plaques

33. Localised SclerodermaClinical Features • Plaques: most common pattern of morphoea. • Superficial Morphoea: • Middle-aged women • In the skin folds, particularly the groin, armpits and under the breasts. • Linear scleroderma: • On the limb of a child. • A long and narrow plaque may be associated with underlying contractures. • En coup de sabre.

34. Localised SclerodermaClinical Features En coup de sabre

35. Localised SclerodermaClinical Features • Generalisedmorphoea. • Pansclerotic disabling morphoea: • children • extensive hardening of skin and underlying muscle • bone growth may be affected. • Atrophoderma of Pierini and Pasini severe loss of subcutaneous tissue with depression.

36. Localised SclerodermaClinical Features Subcutaneous atrophy

37. SCLERODERMA TREATMENT • Skin – symptomatic • Topical calcipotriol • Methotrexate • Systemic steroids • Intralesionalsteroid injections • Photochemotherapy or phototherapy with UVA1 • Long courses of oral penicillin or tetracyclines

38. SCLERODERMA TREATMENT • GI – acid reduction • Raynaud's – vasodilators • Pulmonary alveolitis – Cytoxan, steroids(?) • Pulmonary hypertension – ca+channel blockers, sildenafil, bosentan, epoprostenol, lung transplantation

39. Idiopathic Inflammatory Myopathies • Dermatomyositis • Juvenile • Adult • Polymyositis • Inclusion Body Myositis • Overlap syndromes • Myositis associated with neoplasia

40. Idiopathic Inflammatory Myopathies • Clinical features • Symmetrical proximal weakness • Elevation of skeletal muscle enzymes • Myopathic changes on EMG • Presence of inflammation of skeletal muscles on biopsy

41. Idiopathic Inflammatory Myopathies

42. Idiopathic Inflammatory Myopathies

43. Idiopathic Inflammatory Myopathies

44. Myositis Antibodies

45. Dermatomyositis

46. Dermatomyositis

47. Dermatomyositis

48. Dermatomyositis Gottron’sPapules

49. Dermatomyositis

50. Dermatomyositis