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Cutaneous Manifestations of Internal Disease Adam O. Goldstein, MD, MPH Associate Professor Family Medicine University of North Carolina at Chapel Hill.

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Presentation on theme: "Cutaneous Manifestations of Internal Disease Adam O. Goldstein, MD, MPH Associate Professor Family Medicine University of North Carolina at Chapel Hill."— Presentation transcript:

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

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

3 I. Vasculitis l 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 l Clinical Presentation »livedo reticularis »subcutaneous nodules (malleoli/post calf) »papulonecrotic lesions »digital infarctions »ulcers »ecchymosis

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

6 Small Vessel Vasculitis l Morphology »palpable purpura »urticaria »hemorrhagic pustules »nodules, bullae or ulcers l 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 l frequently confused for vasculitis but benign l progresssive pigmented purpuric dermatosis l nonpalpable, brown staining l petechiae/cayenne pepper- like l no necrosis of vessels l often lower extremities

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

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

12 Scenario 1- l 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- l 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- l 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) l SPEP/IEP l Hepatitis B/C screening l CH 50 l Cryoglobulins l Rheumatoid Factor

17 Patient Evaluation (cont’d) l 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 l CXR

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

19 Rheumatologic Diseases l Lupus erythematosus l Dermatomyositis l Scleroderma l Sjogren’s Syndrome

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

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

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

23 Specific findings l discoid l malar rash l photosensitivity l oral ulcers

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

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

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

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

28 SLE l malar rash l erythematous macules/patches/plaques l butterfly distribution l photo induced l asymptomatic l NLF sparing l 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 l Rosacea »IP/pustules/telang »other triggers »stinging/burning »common

31 SLE Ddx l seborrhea »paranasal scaling »look for scalp/eyebrow involvement

32 SLE Ddx l Contact dermatitis »pruritic, well-demarcated »look for other sites

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

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

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

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

37 SCLE l 10% of all SLE l older l milder disease

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

39 SCLE Ddx l psoriasis l erythema multiforme l polymorphous light eruption

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

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

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

43 Lupus Erythematosus Tx l topical corticosteroids »higher potencies for discoid LE l antimalarials »G6PD

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

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

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

47 Dermatomyositis l Diffuse flat erythema l photosensitive

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

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

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

51 Dermatomyositis Overlap l Systemic lupus erythematosus l Rheumatoid arthritis l Sjögren's syndrome l Dermatomyositis sine myositis or amyopathic dermatomyositis

52 Dermatomyositis l Muscle enzymes — CK,LDH, AST,ALT,aldolase l ANA 80% l anti-RNP - overlap=mixed connective tissue l anti-Jo Ab 30% l anti-Mi-2 Ab

53 Dermatomyositis l EMG - directing biopsy site biopsy l Muscle biopsy - definitive test l MR imaging and P-31 MR spectroscopy (MRS)

54 Thickened, sclerotic skin lesions l musculoskeletal,vasculature l renal, pulmonary, cardiac, gi l 4 to 253 / 1,000,000

55 Scleroderma l variable sclerosis l hands and face

56 CREST syndrome l Calcinosis l Raynaud's l Esoph dysmotility l Sclerodactyly l Telangiectasia

57 Sjogren’s Syndrome l xerosis with pruritus l hyperpigmentation

58 Sarcoidosis l sarco-flesh l eidos-like l osis-condition

59 Sarcoidosis l multisystem disease of unknown etiology l lymphocytes/mononuclear phagocytes form noncaseating granulomas l >2yrs = chronic

60 Sarcoidosis l highest incidence AA US females 30-39 y/o l 107/100,000 l high in Sweden

61 Sarcoidosis l 25% present with skin findings l Erythema nodosum-good prognosis

62 Sarcoidosis l asymptomatic l waxy red/brown papules, macules, plaques, nodules l sub q nodules, infiltrative scars

63 Sarcoidosis l face l lips nape of neck l upper back l extremities

64 Sarcoidosis-Lupus pernio l AA l fibrotic disease l nose, lips, cheeks, ears l respiratory tract involvement l uveitis l bone cysts

65 Sarcoidosis l acquired ichthyosis-DDX »lumphoma »solid malignancy »HIV »mycobacterial inf »medically induced CTD »thryoid/parathyroid »malnutrition

66 Sarcoidosis-Diff Dx l syphilis

67 Sarcoidosis l Lofgren’s Syndrome »erythema nodosum »bilat hilar adenopathy »+/- pulmonary fibrosis »migratory polyarthritis »fever »uveitis

68 Sarcoidosis-Tx l Potent topical corticosteroids l Intralesional corticosteroids l Prednisone l Antimalarials l Methotrexate

69 II. Hypersensitivity- Erythema Nodosum l Defn: Hypersensitivity reaction involving subcutaneous fat l Morphology: Erythematous nodules Location: Extensor surfaces l Epidemiology: Females l Natural history: Individual lesions last ~ 2 wks.

70 Erythema Nodosum- Features l Preceding uri l Low grade fever l Malaise l Arthralgias 50%

71 Erythema Nodosum-Etiology l Infections »URI/Strep »TB »Coccidiodomycosis »Yersiniosis »others l Lymphoma/leukemia l 30-50% Idiopathic

72 Erythema Nodosum-Etiology l Drugs »Sulfonamides »Oral contraceptives l Pregnancy l Inflammatory Bowel Disease l Sarcoidosis

73 Erythema Nodosum-Patient Evaluation l CXR-hilar lymphadenopathy may be seen in EN w/o sarcoidosis l Throat culture/ASO titer l PPD skin test l ESR l Stool culture if gi symptoms l Excisional biopsy rarely indicated

74 Erythema Nodosum-Differential Diagnosis l Weber-Christian panniculitis: suppurate/heal abnormally l Thrombophlebitis l Erysipelas l Pancreatic induced panniculitis l Erythema induratum-TB associated/ calves

75 Erythema Nodosum-Treatment l Naproxen/indomethacin/salicylates l Bed rest l Prednisone l Potassium iodide

76 Pyoderma Gangrenosum l Defn: Ulcerating skin disease l Population: Adults l Distribution: Lower legs, but anywhere l Morphology: »Tender red papules and pustules dusky red/indurated nodules necrotic ulcer »with undermined purple margin »Cribiform scarring

77 Pyoderma Gangrenosum- Etiology l Inflammatory Bowel Disease l Malignancy l Rheumatoid arthritis l IgA monoclonal gammopathy l Lymphoreticular malignancy l 40-50% unknown

78 Pyoderma Gangrenosum-Diagnosis l Clinical appearance l Diagnosis of exclusion l Skin biopsy for routine and cultures »Fungal, AFB, mycobacteria, bacterial l SPEP/IEP

79 Pyoderma Gangrenosum-Treatment l Avoid trauma l Intralesional triamcinolone: 10-25mg/cc l Clobetasol proprionate 0.05% ointment under occlusion l Prednisone l Minocycline l Dapsone l Local care

80 Pruritus of Unknown Origin l Rashless

81 Pruritus of unknown origin: a retrospective study Zirwas MJ, J Am Acad Dermatol 2001;45(6) 892-6 University dermatology department 50 chart reviews 11 systemic cause 7 initial symptom of systemic disease Hypothyroidism Gastric adenocarcinoma Hepatitis C HIV Laryngeal carcinoma GVH CLL

82 Initial Evaluation l Any close contacts affected? »Yes>>>>scabies »NO?-Review of systems/medication review l 2 weeks of mild skin care regimen

83 Initial Evaluation l Medication review »opiates, aspirin, quinidine, phenothiazines, hormones, antimalarials l ROS l Social history- exposure to fiberglass, chemicals l Full cutaneous exam

84 Initial Evaluation l Mild Skin Care Regimen »Unscented white Dove soap »Avoid prolonged, hot showers/baths »Emollients applied immediately after shower or bath –Cetaphil, Eucerin, Lubriderm, Moisturel

85 Initial Evaluation l Mild Skin Care Regimen (cont’d) »Cotton clothing washed in mild laundry detergent –Whisk Free, Ivory Snow Flakes, etc. –avoid any fabric softeners, anti-wrinkle sheets »Triamcinolone ointment or cream 0.1% one pound jar –educate to apply over emollients bid/tid, avoiding face and groin

86 F/U Evaluation l Repeat ROS/Medication review l Pruritus work up »cbc »u/a »liver and kidney panel »thyroid functions »chest x ray »age and/or symptom appropriate cancer screening

87 Special Testing l Secondary tests »HIV »SPEP/IEP »stool for ova and parasites »skin biopsy -routine and DIF

88 Bibliography l Patel P, Werth. Cutaneous lupus erythematosus: a review. Dermatologic Clinics 2002; 20;3: 373-85 l McCauliffe, DP, Sontheimer, RD. Cutaneous Lupus Erythematosus. In: The Clinical Management of Systemic Lupus Erythematosus, 2d ed, Schur, PH (Ed), Lippincott, Philadelphia 1996 l Requena L - Panniculitis. Part I. Mostly septal panniculitis.J Am Acad Dermatol 2001; 45(2): 163-83

89 Bibliography l Sontheimer RD Dermatomyositis: an overview of recent progress with emphasis on dermatologic aspects. Dermatol Clin - 2002; 20(3): 387- 408 l Stonecipher, MR, Jorizzo, JL, White, WL, et al. Cutaneous changes of dermatomyositis in patients with normal muscle enzymes: dermatomyositis sine myositis? J Am Acad Dermatol 1993; 28:951. l English JC, Patel PJ, Greer KE. Sarcoidosis. J Am Acad Dermatol 2001; 44: 725-43. l Etter L, Myers SA. Pruritus in systemic disease: mechanisms and management. Dermatologic Clinics 2002;20(3): 459-72 l Srivastava M - Drug-induced, Ro/SSA-positive cutaneous lupus erythematosus. Arch Dermatol - 2003; 139(1): 45-9

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