1 / 89

Cutaneous Manifestations of Internal Disease

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. Cutaneous Manifestations of Internal Disease. Objectives: Recognize and evaluate patients with I. Vasculitis

perrin
Download Presentation

Cutaneous Manifestations of Internal Disease

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  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

More Related