Dermatomyositis.pptx Presenation For medical students lectures

Dermatomyositis presentation

1. Dermatomyositis Medicine III Lecture

2. Introduction Dermatomyositis (DM) is an autoimmune disorder affecting predominantly, the skin and skeletal muscle Alongside polymyositis in the idiopathic infl-ammatory myopathies (IIMs ) In DM, autoimmune inflammation damages the skin, muscle and, in certain cases, internal organs such as the lungs, joints, oro ‐pharynx and heart

3. Epidemiology Dermatomyositis is a relatively rare disease that occurs throughout the world The incidence of dermatomyositis ranges from 2 to 9 per million amongst various populations The prevalence of IIM is in the region of 5.1–22 per 100 000 The incidence of DM is greater in African Americans than in white Americans Most patients are either children or adults older than 40 years of age

4. In adults, dermatomyositis affects women two to three times more often than men

5. Pathogenesis Dermatomyositis is believed to result from an immune-mediated process Triggered by “outside” factors (e.g. malignancy, drugs, infectious agents ) Genetically predisposed individuals

6. Clinical features Cutaneous Heliotrope rash Gottron’s sign Gottron’s papules Photo-distributed eruption Poikiloderma V-neck sign or shawl sign

7. Heliotrope Rash

8. Heliotrope Rash

9. Gottron’s Gottron’s papules

10. Gottrons Sign

11. Shawl Sign

12. Shawl Sign

13. Poikiloderma

14. Clinical features ( cont ʼ d ) Raynaud’s phenomenon Nail-fold changes Calcinosis cutis Holster sign Scalp erythema and scale

15. Nail Fold Changes

16. Pitting Pulp Ulcers

17. Calcinosis Cutis

18. Calcinosis Cutis

19. Clinical features ( cont ʼ d ) Systemic Musculoskeletal - Symmetric, inflammatory myopathy - Triceps and quadriceps usually affected first and may be tender - Inflammatory polyarthritis Gastrointestinal - Dysphagia (upper esophagus), - Gastroesophageal reflux (lower esophagus) - In children, vasculopathy of the GI tract

20. Clinical features ( cont ʼ d ) Cardiac - Arrhythmias Pulmonary - Interstitial lung disease

21. Associated diseases There is a strong association between internal malignancy and adult DM The reported frequency of an internal malignancy in adults with dermatomyositis varies from <10% to > 50% The malignancy association occurs with adult dermatomyositis (both classic and amyopathic )

22. Associated cancers Ovarian cancer Colon cancer Nasopharyngeal carcinoma Breast cancer Lung cancer Gastric cancer Pancreatic cancer Lymphomas (including Non- Hodgkin ʼ s )

23. Differential diagnosis Lupus erythematosus Systemic sclerosis Psoriasis Cutaneous T cell lymphoma Atopic eczema Rosacea

24. Clinical variants Overlap syndromes/Mixed connective tissue disease Juvenile dermatomyositis Amyopathic dermatomyositis Drug‐induced dermatomyositis

25. Evaluation History and physical examination - History of potential triggers - Previous malignancies - Review of systems

26. Evaluation( cont ʼ d ) Physical examination - S kin - Muscle - Complete general examination - In adults, breast and pelvic (women) - Testicular and prostate (men) - Rectal examination (both sexes) - In Southeast-Asian patients, consider full ENT examination

27. Skin biopsy Serum creatine kinase Serum aldolase LDH AST/ALT Electromyography ( EMG) Muscle biopsy MRI

28. Pulmonary function tests (PFTs) with CO diffusion High-resolution chest CT (HRCT) Electrocardiogram (EKG) Holter monitor Barium swallow or manometry

29. Complete blood count Fasting blood glucose Fasting lipids Directed autoantibody panel (Anti- Mi 2 antibody)

30. Urinalysis Stool occult blood testing Serum prostate-specific antigen (PSA) Serum CA125 [women] Serum CA19-9 [men and women] Mammogram Papanicolaou smear Transvaginal pelvic USS [women]

31. CT of chest, abdomen and pelvis Colonoscopy Upper endoscopy

32. Treatment Photoprotection including sunscreens (high SPF including protection against UVA) and clothing Topical corticosteroids Topical T acrolimus Hydroxychloroquine (200 mg po twice daily or chloroquine (250 mg po daily ) Hydroxychloroquine (200 mg twice daily) or chloroquine (250 mg po daily) plus quinacrine (100 mg po daily) Methotrexate (5–15 mg weekly po , sc , IM) Mycophenolate mofetil

33. Treatment( cont ʼ d ) High-dose IVIg (2 g/kg/month) Retinoids Dapsone Thalidomide Leflunomide Anti-estrogens (e.g. tamoxifen, anastrazole) TNF- α inhibitors (e.g. infliximab, etanercept) Rituximab Oral tacrolimus Tofacitinib

34. Treatment( cont ʼ d ) Oral prednisone: 1 mg/kg/day tapered to 50% over 6 months and to zero, over 2–3 years Option to use pulse, split-dose, or alternate-day Methotrexate: 5–25 mg weekly po , sc , IM Azathioprine: 2–3 mg/kg/day po

35. Treatment( cont ʼ d ) High-dose IVIg (2 g/kg/month ) Mycophenolate mofetil (1 g po twice daily) Pulse cyclophosphamide (0.5–1.0 g/m2 IV monthly ) Chlorambucil (4 mg/day po ) Cyclosporine (3–5 mg/kg/day po ) Rituximab (375 mg/m2/infusion for 4 weekly IV infusions)

36. Treatment( cont ʼ d ) Tacrolimus (0.12 mg/kg/day po ) Sirolimus (5 mg/day × 2 weeks, 2 mg/day × 2 weeks, then 1 mg/day po ) TNF- α inhibitors (e.g. infliximab, etanercept) Fludarabine Hematopoietic stem cell transplantation Plasmapheresis

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38. Eczema Medicine III Lecture

39. The word eczema, derived from the Greek ‘to break out or boil over ’ Refers to the vesicles (bubbles) seen in acute eczema Eczema and dermatitis tend to be used synonymously Dermatitis means inflammation of the skin Eczema may be endogenous ( commonest) or exogenous

40. Eczema is described as a reaction pattern of the skin with diverse etiology Is an itch that rashes (when scratched) Chronic itching leads to scratching and scratching leads to secondary changes in the skin

41. Epidemiology Eczematous dermatoses account for a large proportion of all skin diseases The point prevalence of all forms of eczema was 18 per 1000 population Most cases of eczema in infants and young children are atopic Hand eczema is common in atopic children and uncommon in non‐atopic children

42. Pompholyx and atopic eczema are less common in elderly people Eczematous dermatoses are reported in all ethnic groups

43. Genetics Filaggrin mutations are linked to hand eczema, irritant contact dermatitis and allergic contact dermatitis

44. Pathogenesis The interaction of trigger factors, keratinocytes and T lymphocytes seems particularly important in most eczema types Allergic contact dermatitis represents a reproducible model of eczema development Irritant contact dermatitis is provoked in a non‐allergic manner Irritant contact dermatitis is characterized by disturbed barrier function, epidermal cell change and release of inflammatory mediators and cytokines

45. Clinical features A spectrum from acute to subacute to chronic Acute eczema - Itching - Erythema - Swelling - Oozing - Vesicles - Bullae - Crusting

46. Acute Eczema

47. Acute Eczema

48. Subacute eczema - I tching - Erythema - Scaling - Less oedema - Minimal vesiculation - Excoriation

49. Subacute Eczema

50. Subacute Eczema

51. Subacute Eczema

52. Subacute Eczema

53. Subacute Eczema

54. Chronic eczema - Scaling - Fissuring - Lichenification - Mild erythema - Excoriations - Hyperpigmentation

55. Chronic Eczema

56. Chronic Eczema

57. Chronic Eczema

58. Chronic Eczema

59. Chronic Eczema

60. Classifications Endogenous eczemas Atopic eczema Asteatotic eczema Seborrhoeic eczema Nummular dermatitis Hand eczema/Foot eczema Dishidrotic eczema Juvenile plantar dermatosis Gravitational eczema/Venous eczema

61. Exogenous eczemas Allergic contact eczema Irritant contact eczema Eczematous polymorphic light eruption Infective dermatitis Photoallergic contact eczema

62. Diagnosis Clinical Skin swab test Patch testing Dermatoscopy Skin biopsy

63. Treatment Bed rest Sedation Wet dressings and soaks Wet wrap bandaging Paste bandages Emollients Pharmacologic agents

64. Acute eczema Adequate rest Limb elevation Astringent - Potassium permanganate or Burrow’s solution Bland emollients Topical corticosteroids Topical calcineurin Inhibitors - Tacrolimus and P imecrolimus Topical antibiotics Antihistamines

65. Subacute eczema Paste bandaging/wet dressing Topical steroids under occlusion Topical calcineurin inhibitors – Tacrolimus and Pimecrolimus Doxepin 5% cream Antibiotics Tar

66. Wet Wrap

67. Chronic eczema Avoidance of irritants Frequent emollients Topical corticosteroids Topical calcineurin inhibitors Coal tar Phototherapy - Narrow‐band UVB - P soralen and UVA (PUVA) - UVA

68. Systemic steroids Immunosupressives Oral retinoids

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