1. Cutaneous Manifestations of Systemic Diseases Hayden H. Franks, MD June 13, 2013

2. Who Is This Guy? • Private practice Dermatologist • Clinics in Little Rock and Texarkana • Fellow of the American Academy of Dermatology • Diplomate of the American Board of Dermatology • Assistant Clinical Professor of Dermatology, UAMS AHEC SW • Honorary Member of the Arkansas Academy of Family Physicians

3. Disease Categories • Autoimmune Diseases • Endocrine Diseases • Cardiopulmonary Diseases • Gastrointestinal Diseases • Neurological Diseases • Diseases not Otherwise Specifiied

4. Cutaneous Manifestations of Systemic Diseases • Frequently encountered • May be the initial sign of internal disease • May occur late in the course of the disease • May assist in making the diagnosis • May be obvious or subtle • Overlap of Family Practice and Dermatology

5. AUTOIMMUNE DISEASES • Systemic Lupus Erythematosus • Scleroderma • Dermatomyositis • Rheumatoid Arthritis

6. Systemic Lupus Erythematosus • Autoimmune, systemic disease affecting multiple organ systems • The most common connective tissue disease • Especially prevalent in black women – Prevalence 1/250 • Cutaneous lesions present in 85% of patients • Of the 11 Classic Criteria for diagnosing SLE, 4 involve the skin or mucus membranes

7. Systemic Lupus Erythematosus • Malar (Butterfly) rash is the “classic presentation” • May be distinct or subtle

8. Systemic Lupus Erythematosus • Fixed erythema, flat or raised, over malar eminences • Spares the Nasolabial Folds

9. Systemic Lupus Erythematosus • Discoid Rash is “classic” as well

10. Systemic Lupus Erythematosus • Erythematous, patches and plaques, with adherent scales, follicular plugging and atrophic scarring

11. Systemic Lupus Erythematosus • Photosensitivity – rash as an unusual reaction to sunlight

12. Systemic Lupus Erythematosus • Oral Ulcers – usually painless and may be nasopharyngeal

13. Systemic Lupus Erythematosus • Presentation isn’t always “classic” • High index of suspicion • Alopecia and rash may be anywhere on skin

14. Systemic Lupus Erythematosus • Diagnosis is based on presence of multisystem disease and presence of antinuclear antibodies • Treatment is multifactorial with corticosteroids being the mainstay still • Sunscreen • Antimalarials, methotrexate, dapsone and biologics now are commonly used

15. Scleroderma • Chronic autoimmune disease of unknown cause that affects the microvasculature and loose connective tissue • Characterized by fibrosis and obliteration of vessels in skin, lungs, GI tract, kidneys and heart • May be localized (Morphea) or systemic (Systemic Scleroderma)

16. Scleroderma (Morphea) • Morphea – benign and self limited • Usually single or few in number • Red, then white, atrophic, indurated with alopecia

17. Scleroderma (Morphea) • Treatment is unsatisfactory • Topical or intralesional steroids, PUVA

18. Systemic Scleroderma (SSc) • Four times more common in women • 10 year survival rate of 21-71% • Clinical manifestations depend on the sites involved • Initial complaints are usually Raynaud’s phenomenon or chronic, non pitting edema of hands and fingers or migratory polyarthritis • Disease may extend to involve upper extremities, trunk, face and finally the lower extremities

19. Systemic Scleroderma (SSc)

20. Systemic Scleroderma (SSc)

21. Systemic Scleroderma (SSc)

22. Systemic Scleroderma (SSc) Diagnosis • Autoantibodies to Fibrillin 1, Rheumatoid Factor, Anti SS DNA, Anti RNA Polymerase 3, Antitopoisomerase 1, Anticentromere Antibodies • Skin Biopsy

23. Systemic Scleroderma (SSc) Treatment • Treatment is unsatisfactory • Immunosuppressive Drugs of numerous types • Methotrexate, Cyclosporine, Imuran • Biologics

24. Dermatomyositis • The most common idiopathic inflammatory myopathy • May occur at any age • Unknown etiology • Autoimmune Disease • Progressive weakness of trunk and major limb muscles • Difficulty in rising from a chair or climbing stairs • Impaired mobility and some muscle tenderness

25. Dermatomyositis • Bilateral muscle weakness that is progressive • Skin lesions are almost always present from the onset • Maculopapularerythema over bony prominences such as the knuckles, elbows and knees • Red to violaceous plaques with telangiectasias and scales • Gottron’s Papules – polymorphic, erythematous and atrophic plaques

26. Dermatomyositis

27. Dermatomyositis

28. Dermatomyositis • Heliotrope Rash – Periorbitalerythema • Nail Margin Telangiectasias

29. Dermatomyositis Diagnosis • Elevated serum muscle enzymes (CK) and Aldolase • Antinuclear Antibodies • Muscle biopsy – segmental muscle fiber fibrosis, interstitial inflammation and vasculopathy • Skin biopsy – Focal vacuolar degeneration of basal cells, basement membrane degeneration and epidermal atrophy

30. Dermatomyositis Treatment • Primary treatment remains Prednisone 1mg/kg/day • Plasmapheresis • Cyclosporine • Dapsone • ?Biologics • Physical Therapy

31. Rheumatoid Arthritis • Disease affects up to 2% of adult women • Onset is sudden or insidious • Symmetric polyarthritis that affects the proximal interphalangeal and metacarpophalangeal joints, the wrists, ankles, knees and cervical spine • Stiffness, painful, warm and tender joints • Fever, weight loss and anemia are prominent

32. Rheumatoid Arthritis • Rheumatoid Nodules – discrete, non tender subcutaneous tumors

33. Rheumatoid Arthritis • Vascular Lesions – erythema of palms and digital infarcts

34. Rheumatoid Arthritis

35. Rheumatoid Arthritis • Gravitational ulcers – most common • Arteritic ulcers – actually rare until advanced disease

36. Rheumatoid Arthritis • Laboratory Workup – Rheumatoid Factor and ANA • Treatment – Prednisone, Methotrexate, Biologics

37. ENDOCRINE DISEASES • Diabetes Mellitus • Thyroid Disease

38. Diabetes Mellitus • The skin shares both in the effects of acute metabolic derangements and in the chronic degenerative complications of diabetes.

39. Diabetes Mellitus • Infection • Diabetic Dermopathy • Thickened skin, stiff joints and ScleredemaAdultorum • NecrobiosisLipoidicaDiabeticorum • Vitiligo • AcanthosisNigricans • Kyrle’s Disease (Reactive Perforating Collagenosis)

40. Diabetes Mellitus • Bacterial and fungal infections • Furunculosis, Cellulitis, Erythrasma, and Candidiasis • Hyperglycemia leads to abnormalities in leukocyte function including diminished chemotaxis and phagocytosis

41. Diabetes Mellitus • Bacterial Infections

42. Diabetes Mellitus • Cellulitis

43. Diabetes Mellitus • Erythrasma

44. Diabetes Mellitus • Candidiasis

45. Diabetes Mellitus • Diabetic Dermopathy – atrophic, circumscribed brownish lesions usually on the lower extremities • They resemble post traumatic scarring

46. Diabetes Mellitus • Thickened Skin, Stiff Joints and ScleredemaAdultorum • 33% of Diabetics have tight, indurated and waxy skin over the dorsa of the hands • ScleredemaAdultorum is strongly correlated with IDDM • Consists of induration of the skin beginning on the posterior and lateral aspect of the neck, is painless and may be progressive

47. Diabetes Mellitus

48. Diabetes Mellitus • ScleredemaAdultorum

49. Diabetes Mellitus • NecrobiosisLipoidicaDiabeticorum • Occurs in 0.3% of IDDM Patients • Very distinct • Asymptomatic, atrophic, yellow to brown patches classically on the lower extremities • Telangiectasias are prominent

50. Diabetes Mellitus • NecrobiosisLipoidicaDiabeticorum