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Cutaneous Manifestations of Internal Disease

Cutaneous Manifestations of Internal Disease

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Cutaneous Manifestations of Internal Disease

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  1. Cutaneous Manifestations of Internal Disease Adam O. Goldstein, MD, MPH Associate Professor Family Medicine University of North Carolina at Chapel Hill aog@med.unc.edu

  2. Cutaneous Manifestations of Internal Disease • Objectives: Recognize and evaluate patients with • I. Vasculitis • II. Rheumatologic disease • III. Hypersensitivity syndromes • IV. Pruritus

  3. I. Vasculitis • Definition: Inflammation of the vessel wall by immune complex deposition • type of inflammatory cell (neutrophil, lymphocyte, histiocyte) • size of vessel (venule, arteriole, artery or vein)

  4. Large/medium Vessel Vasculitis • Clinical Presentation • livedo reticularis • subcutaneous nodules (malleoli/post calf) • papulonecrotic lesions • digital infarctions • ulcers • ecchymosis

  5. Differential Diagnosis • Polyarteritis nodosa • Wegener’s Granulomatosis • Churg-Strauss Syndrome

  6. Small Vessel Vasculitis • Morphology • palpable purpura • urticaria • hemorrhagic pustules • nodules, bullae or ulcers • Features • fever, malaise, myalgia, • arthralgia, lower ext. edema

  7. Differential Diagnosis/ Etiologies • Infections: Hepatitis B/C, acute Strep, viral URI • ANCA • Henoch-Schonlein Purpura • Connective Tissue Disease • Neoplasms

  8. Differential Dx & Etiologies • Cryoglobulinemia • Hypersensitivity • Medications: • penicillin, thiazides, aspirin, phenothiazines, sulfonamides, iodides • Unknown

  9. Schamberg’s Disease • frequently confused for vasculitis butbenign • progresssive pigmented purpuric dermatosis • nonpalpable, brown staining • petechiae/cayenne pepper-like • no necrosis of vessels • often lower extremities

  10. Small Vessel Vasculitis: Signs/Symptoms • Renal-mild to severe • Peripheral neuropathy • GI -abd.pain,N/V,diarrhea,melena • Pulmonary-hemoptysis, cough, SOB, CXR only • Joint • Heart-arrthymia/CHF

  11. Small vessel vasculitis • Perform a skin biopsy -routine and DIF from a palpable purpuric papule 24 hours old. • Obtain an esr, cbc, u/a, liver/renal functions.

  12. Scenario 1- • Skin biopsy results are conclusive • In addition to vasculitis of the dermal vessels on routine histology, IgA deposition is noted in and around the vessels on the 4 mm punch specimen sent for DIF. The diagnosis is Henoch Schonelin Purpura.

  13. Scenario 2- • Skin biopsy results are nonspecific • Vasculitis is seen in the dermal vessels. RF, ANA, anti ds-DNA, Ro and La, complement and cyroglobulin levels, ANCA, SPEP, UPEP, CXR

  14. Scenario 3- • Skin biopsy results are pending and patient is seriously ill • combine entire work up while for waiting for the results of the skin biopsy.

  15. Patient Evaluation • Identify and confirm source of offending antigen (drug/infection) • throat culture/ASO titer • ESR/CBC w/plt • Creatinine • U/A • ANA

  16. Patient Evaluation (cont’d) • SPEP/IEP • Hepatitis B/C screening • CH50 • Cryoglobulins • Rheumatoid Factor

  17. Patient Evaluation (cont’d) • Skin biopsy from new lesions (<24-48 hrs. old) • lumph predominate -CTD/ eos-Churg-Strauss • Immunofluorescent studies if concerned about HSP(IgA) • special media required • CXR

  18. Small Vessel Vasculitis-Treatment • Removal of offending etiology • Symptomatic relief • Prednisone 40-60 mg/day x 2-3 weeks • Colchicine 0.6 mg bid for 7-10 days then taper • Azathioprine/Dapsone

  19. Rheumatologic Diseases • Lupus erythematosus • Dermatomyositis • Scleroderma • Sjogren’s Syndrome

  20. Systemic lupus erythematosus (SLE) • chronic inflammatory disease of unknown cause skin, joints, kidneys, lungs, nervous system, serous membranes and/or other organs of the body.

  21. Lupus Erythematosus • acute: SLE/Bullous LE • subacute: SCLE/Neonatal/Drug-induced • chronic: discoid/ panniculitis

  22. Diagnosis of SLE • 4/11ARA criteria are cutaneous • 50% present w/skin finding • 85% have skin findings at some point

  23. Specific findings • discoid • malar rash • photosensitivity • oral ulcers

  24. Nonspecific findings • vasculitis • urticarial vasculitis • alopecia • calcinosis cutis • rhematoid nodules • Raynaud’s

  25. Discoid LE • distribution: sun-exposed • face and scalp • papules, plaques • erythema, pigmentary alteration, scarring, follicular plugging

  26. Discoid LE: Ddx • tinea facei/capitis • scarring alopecia • cicatricial pemphigoid • hypertrophic lichen planus • actinic keratoses

  27. Discoid LE: Dx • biopsy • ANA • CBC w/diff esr, BUN/Cr/u/a • <5% have SLE

  28. SLE • malar rash • erythematous macules/patches/plaques • butterfly distribution • photo induced • asymptomatic • NLF sparing • 90% arthritis

  29. Lupus Profundus • 3% w/ chronic cutaneous lupus • More frequent in women • Upper arms, shoulders, face, and buttocks • Often trauma related • Dx-biopsy

  30. SLE Ddx • Rosacea • IP/pustules/telang • other triggers • stinging/burning • common

  31. SLE Ddx • seborrhea • paranasal scaling • look for scalp/eyebrow involvement

  32. SLE Ddx • Contact dermatitis • pruritic, well-demarcated • look for other sites

  33. SLE Dx • ANA • cbc/esr/cr/bun/u/a • +/- biopsy if other unclear

  34. SLE Lab Dx • ANA + 95-8% • DS DNA + 40% • ENA + 30% • RNP/Sm + • SSA/Ro + 25%

  35. Photosensitivity • UVA and UVB • r/o drug photosensitivity • same as malar for w/u

  36. Oral Ulcers • ddx: lichen planus/aphthae/pemphigus • w/u same as malar rash

  37. SCLE • 10% of all SLE • older • milder disease

  38. SCLE • face/neck/upper torso/forearms • erythematous scaling papules >>>>polycyclic/arcuate plaques • no scarring/alopecia or follicular plugging • photosensitive eruption • 70% SSA/B + • 85% ANA +

  39. SCLE Ddx • psoriasis • erythema multiforme • polymorphous light eruption

  40. SLE: Drug-induced • 90% anti histone Ab • 20% skin lesions • <8% SLE caused by drugs

  41. Ca++ channel blockers PUVA ACE inhibitors Hyrdralazine Procainamide Isoniazid Chlorpromazine Minocycline HCTZ Lamisil D-penicillamine Interferon -Statins Sulfonylurea Griseofulvin Piroxicam Beta blockers Carbamazepine Estrogens Lithium Methyldopa Minoxidil Phenytoin PTU Quinidine Testosterone SLE: Drug-induced/aggravated/un-masked

  42. Lupus Erythematosus Tx • broad spectrum sun protection • sunblock for lips • sun avoidance • physical protection • monitor for systemic involvement

  43. Lupus Erythematosus Tx • topical corticosteroids • higher potencies for discoid LE • antimalarials • G6PD

  44. Dermatomyositis • idiopathic inflammatory myopathy • 1/100,000 • 2:1 female to male • malignancy assoc. 15-25% • immune complex deposition in vessels

  45. Five diagnostic criteria • Sym proxl muscle wknss • Typical rash • ^ muscle enzymes • EMG abn • Muscle bx abn

  46. Gottron's sign • symmetric, • nonscaling • violaceous erythematous eruption • extensor surfaces MCP/IP/elbows/knee joints

  47. Dermatomyositis • Diffuse flat erythema • photosensitive

  48. Dermatomyositis • Heliotrope rash • is a reddish–violaceous eruption on the upper eyelids • often accompanied by swelling of the eyelid

  49. Dermatomyositis • Periungual erythema • Painful roughening and cracking fingers termed "mechanic's hands”

  50. Juvenile dermatomyositis: a retrospective review Pelero TM J Am Acad Dermatol 200l; 45 of a 30-year experience