Cutaneous Manifestations of Systemic Disease
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| Autoimmune disorders | Rheumatic Dz:SLE, dermatomyositis, scleroderma, vasculitides,
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| Endocrine Disorders | Diabetes
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| Immune Disorders | Dermatitis herpetiformis, urticaria/angioedema, sarcoidosis
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| Urticaria should make you think of | connective tissue disease or systemic dz
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| Miscellaneous disorders | Erythema multiforme, erythema nodusum
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| Which inflammatory arthritis are associated with skin manifestations? | Reider's and psoriatic
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| Criteria for systemic lupus | alopecia, oral ulcers, photosensitivity, discoid lesions
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| Acute cutaneous SLE | most have systemic dz.
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| Subacute cutaneous SLE | 50% have systemic SLE
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| chronic cutaneous SLE | rare to have systemic SLE
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| Acute Cutaneous LE (ACLE) Presentation | localized or generalized: face, scalp, upper extremities. Malar or butterfly rash (seen in 70% of SLE), papules, bullae, palmar erythema
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| Women who have anti-Ro and anti-La antibodies | tend to have babies with SLE.
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| Subacute cutaneous LE (SCLE) presentation | mostly on upper trunk, face, neck. Scaly plaques common. Not as generalized as acute and chronic. annular or papular lesions.
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| Chronic Cutaneous LE (CCLE) Presentation | localized or generalized (like ACLE). Discoid lesions are classic. lesions start as well defined scaling plaques that extend into hair follicles (follicular plugging); expand slowly and heal with scarring, dyspigmentation and/or atrophy
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| Classic discoid lesions are associated with | chronic cutaneous LE
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| ____ is the classic cutaneous dermatomyositis lesion | heliotrope; violaceous macular rash or eyelids or periorbital area
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| ________ is considered pathognomonic of dermatomyositis | Gottron's papules: slightly raised pink, dusky red or violaceous papules over the dorsal sides of the MCP/PIP and/or DIP joints. Can also occur over wrists, elbows or knees
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| cutaneous findings of dermatomyositis | poikiloderma/mottling, calcifications, linear erythema over extensor surfaces of joints, nail findings: periungual erythema, telangectasia, cuticle overgrowth
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| Morphea and linear scleroderma are associated with | localized scleroderma. Linear plaques, most common in women, onset 20-50 years, rarely progresses to systemic dz
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| Common Presentations of Systemic Scleroderma | Sclerodactyly, Raynaud's Phenomenon, sclerosis of face, scalp, trunk, periungual and mat-like telangiectasia, pigementation abnormalities, calcinosis cutis
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| Hallmark of vasculitis (think: blood oozing out of vessels) | palpable purpura (nothing else looks like this) Doesn't blanch. Usually on lower extremities, stasis can worsen this and coalescing occurs
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| Anyone who presents with vasculitis, check for | hematuria. Very common.
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| Most common cause of vasculitis | Infection (GABS, Hep B, Hep C) and then drugs (sulfonamides, penicillin, others). Also connective tissue disease
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| Acanthosis Nigricans is associated with | insulin resistance. Thickened, hyperpigmented, velvety plaques that develop on neck, axillae, other body folds. Associated with obesity and insulin resistance.
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| Considered by many to be the most common cutaneous manifestation of Diabetes Mellitus | Diabetic dermopathy (DD). AKA shin spots. Pathophysiology: microangiography. Characterized by atrophic, small (<1cm), brown lesions on lower extremities. Asymptomatic; lesions stay 18-24 months, fade
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| lesions that are likely associated with Diabetic peripheral neuropathy | Diabetic Bullae. Appear spontaneously usually on hands or feet. Three types: sterile, heals without scarring; hemorrhagic, heals with scarring; non-scarring, triggered by sun exposure
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| Degredation of collagen in dermis and subcutaneous fat associated with Diabetes. | Necrobiosis Lipodica Diabeticorum (NLD). Flesh-colored or reddish-brown papules that evolve into waxy papules. Center of lesion becomes yellow and atrophic, telangectasis may appear. usually seen on shins bilaterally. Painful. may be pruritic
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| Diabetic Ulcers | can result from neuropathic or ischemic causes. Can go through subQ tissue, even to the bone, causing osteomyelitis.
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| Uncommon benign skin disorder of papules and plaques in annular distribution; self-limiting | Granuloma Annulare (GA). Generalized should be a tip off for Diabetes Screen
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| Yellow plaques occurring near medial canthus of eyelid; upper lid >lower lid | Xanthelasma. Associated with lipid problems. Tx: reduction of serum lipids: diet, pharmacotherapy. Surgical excision
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| Angioedema | urticaria that extends into subcutaneous tissue. Also, doesn't turn erythematous like urticaria, usually flesh colored.
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| What percentage of acute urticaria patients will go on to develop chronic urticaria? | 25%
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| Wheel and Flare | Center white portion is the wheel, surrounding red is the flare
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| Question to ask with urticaria | migratory, transient nature. Normal to migrate, usually worse at pressure points. Itch terribly.
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| Is urticaria a systemic reaction? | Yes, not uncommon to have malaise and fever as well.
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| What is important to rule out with chronic urticaria? | Connective tissue dz and malignancy
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| Tx for urticaria | H1 and H2 blockers, doxepin (sedating, histamine blockers, for chronic), glucocorticoids (for chronic), epi-pen. CAN GIVE IM BENEDRYL IN CLINIC
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| Tx for angioedema | epi pen
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| order an ANA if there is a family hx of | connective tissue disease
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| Chronic skin disorder associated with celiac disease | Dermatitis Herpetiformis. Physiology: IgA deposits in skin; these are antibodies made in response to glutens
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| What does dermatitis herpetiformis look like? | Lesions: erythematous papules/plaques studded with vesicles. location: classic: extensor surfaces of elbows, knees, also buttocks, scapular areas, scalp; symmetricintensely pruritic
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| Signs of venous insufficiency | pitting edema (sometimes the first sign), varicose veins, stasis dermatitis (appears eczematous, +/- papules, excoriations), hyperpigmentation, skin fibrosis, venous ulcers
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| Characteristic of varicose veins that is worrisome | if they are painful.
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| Stasis dermatitis | often mistaken for cellulitis. Occurs on lower legs, ankles, pruritic, erythematous, pitting edema may also be present.
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| End stage for people with venous insufficiency | venous ulcers. 1/3 of pts with venous insufficiency will develop these.
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| Common location of venous ulcers | above medial malleolus. exquisitely painful, well demarcated, shallow erosion, irregular shape, base often necrotic
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| Chronic multisystem granulomatous disease | Sarcoidosis. Common in AA females: 10-15x greater risk of disease. Skin involvement: 25% of pts. Lesions: nodules, plaques, asymptomatic, violaceous. Tends to develop in scars, ex: tatoos
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| plaque specific to sarcoidosis | lupus pernio: infiltrating violaceous plaque; occurs on nose, cheeks, ears, lips
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| Cutaneous immunologic response to varied antigens. Characteristic lesion: macule → papule with vesicle or bulla in center; this is known as a TARGET (or iris) lesion | Erythema mulitforme (EM)
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| Erythema Multiforme causes | Infections (especially HSV 1&2), Drugs (especially sulfonamides), systemic diseases
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| Target lesions can be found on skin and | on mucous membrains (major EM)
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| Target lesions are a hallmark of | Erythema multiforme. Symmetric and bilateral
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| widespread bullae on trunk, face + mucous membrane involvement with epidermal detachment | < or = 10% of TBSA (SJS), > or = 30% of TBSA (TEN)
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| SJS is always | a medical emergency
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| Lesions similar to Erythema multiforme, but likely to occur in 18-30 year olds | Erythema nodosum. May precede diagnosis of lymphoma by many months
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| Which systemic diseases is Erythema Nodosum associated with? | IBD: UC and Crohn's, malignancy, sarcoid
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| Anti-Ro and Anti-La antibodies are associated with which form of LE? | Subacute cutaneous LE (SCLE)
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