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Duke PA Cutaneous Manifestations of Systemic Disease

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Answer
Autoimmune disorders with cutaneous signs   SLE, dermatomyositis, scleroderma, vasculitides  
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endocrine disorders with cutaneous signs   diabetes  
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immune disorders with cutaneous signs   dermatitis herpetiformis, urticaria/angioedema, sarcoidosis  
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metabolic disorders with cutaneous signs   xanthelasma  
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>__% of patients with SLE have skin findings   85  
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subset of patients with __ have cutaneous, but not systemic disease   lupus erythematosus  
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3 categories of skin manifestations of SLE   acute cutaneous, subacute cutaneous, chronic cutaneous (discoid lupus)  
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aka discoid lupus   chronic cutaneous lupus  
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other dermatologic manifestations of SLE   alopecia and oral ulcers  
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__ is very common in those with lupus, sun exposure may trigger acute lesions   photosensitivity  
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localized or generalized face, scalp, upper extremities (malar or butterfly rash), papules, papular urticaria, scaly plaques, discoid lesions, bullae, palmar erythema   acute cutaneous LE (ACLE)  
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scaly plaques are more commonly associated with __LE   subacute cutaneous LE  
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discoid lesions are more commonly associated with __ LE   chronic cutaneous LE  
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__% of patients with SCLE have SLE   50  
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typically start as small erythematous papules with scale, associated with anti-Ro and anti-La antibodies,   annular or papular (psoriaform) lesions associated with SCLE  
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__% of SLE patients have discoid lesions   25  
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non-specific lesions/rashes associated with SLE   lupus profundus (lupus panniculitis), vasculitic lesions (purpura), livedo reticularis, urticaria  
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one of the idiopathic inflammatory myopathies, heliotrop is classic cutaneous lesion (erythematous or violaceous macular rash of eyelides, peri-orbital area, often accompanied by edema   Dermatomyosistis  
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considered pathognomonic of dermatomyositis   Gottron's papules  
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slightly raised pink, dusky red or violaceous papules over the dorsal sides of the MCP/PIP and or DIP joints, can occur over wrists elbows or knees (pathognomonic for dermatomyositis)   Gottron's papules  
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macular rash over posterior shoulders/neck. seen in dermatomyositis   shawl sign  
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macular photosensitivity rash over anterior neck, can aslo occur on face or scalp. associated with dermatomyositis   V sign  
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nail findings of dermatomyositis   periungual erythema, telangiectasias, cuticle overgrowth  
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systemic sclerosis   scleroderma  
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localized scleroderma   morphea, linear scleroderma  
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localized plaques or linear, band-like distribution, most common in women, peak age of onset 20-50, rarely progresses to systemic disease   localized scleroderma  
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sclerodactyly, Raynaud's phenom, sclerosis of face, scalp, trunk, periungual and mat-like telangiectasia, pigmentation abnormalities, calcinosis cutis   systemic scleroderma  
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the hallmark of vasculitis   palpable purpura  
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well defined raised petechiae and macules, may have a central area of hemorrhage, can become confluent, primarily on lower extremities but can extend to buttocks. can become ulcerative, necrotic in severe cases. Associated with vasculitis   palpable purpura  
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cutaneous disorders associated with diabetes   acanthosis nigricans, diabetic dermaopathy, diabetic bullae, necrobiosis lipoidica, infections, lesions secondary to peripheral neuropathy, lesions secondary to peripheral vascular disease  
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skin infections associated with diabetes   abscesses, furuncles, and carbuncles, cellulitis, erythrasma, candidiasis, malignant otitis externa, mucormycosis  
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thickened hyperpigmented velvety plaques that develop on neck, axillae, other body folds. associated with obesity and insulin resistance   acanthosis nigricans  
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considered by many to be most common cutaneous manifestations of DM. Pathophysiology:microangiography. Characterized by atrophic, small (<1 cm), brown lesions on lower extremities   diabetic dermopathy  
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appear spontaneously, usually on hands or feet, patho-unclear but they appear to be associated with peripheral neuropathy in diabetics   diabetic bullae  
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three types of diabetic bullae   sterile (heals w/o scarring), hemorrhagic (heals with scarring), non-scarring (triggered by sun exposure)  
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flesh colored or reddish brown papules that evolve into waxy plaques (center becomes yellow and atrophic, telangiectasis my appear, mostly seen on shins usually bilateral)   Necrobiosis Lipodica Diabeticorum  
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pathophysiology of necrobiosis lipodica diabeticorum   degeneration of collegen in dermis and subutaneous fat  
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treatement for necrobiosis lipodica diabeticorum   topical or intralesional steroid  
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can result from neuropathic or ischemic causes from diabetes. often surrounded by callus formation, may extend through subcutaneous tissue to bone-->osteomyelitis   diabetic ulcers  
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uncommon benign skin disorders of papules and plaques in annular distribution, self limiting (more common in women, hands, feet, knees and elbows) usually associated with diabetes   granuloma annulare  
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yellow plaques occuring near medial canthus of eyelid, upperlid>lower lid (50% associated with elevated lipid levels)   xanthelasma  
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localized swelling of the skin and mucous membranes   urticaria  
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urticaria that extends into subcutaneous tissues   angioedema  
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localized swelling of the skin and mucous membranes accompanied by pruritis. occurs once, individual lesions usually resolves in 24 hours. lasts days to 6 weeks, triggers:foods, drugs, infection, stress, latex, environmental agents   acute urticaria  
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localized swelling of the skin and mucous membranes accompanied by pruritis. recurrent or constant, 6 weeks or greater duration, trigger is undetermined in 85% of cases (may be due to autoimmune or chronic disease)   chronic urticaria  
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wheal is the characteristic lesion of   urticaria  
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edematous papule or plaque, transient, very pruritic   wheal (urticaria)  
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treatment of urticaria   H-1 and H-2 blockers ahve synergisitic effect, doxepin, glucocorticoids, epinephrine  
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__ generation H-1 blockers are more effective in acute urticaria   first  
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__ generation H-1 blockers are more effective in chronic urticaria   second  
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clinical evaluation of chronic urticaria   complete H&P, CBC, LFT's, TFT's, RFT's, ESR or CRP, biopsy in cases of vasculitis (referral to allergist/rheumatologist may be appropriate)  
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treatment principles of chronic urticaria   avoid use of systemic corticorsteroids, identify exacerbating/causitive factors, around the clock use of antihistamines  
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chronic skin disorder associated with celiac disease   dermatitis herpetiformis  
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physiology of dermatitis herpetiformis   IgA deposits in the skin, these are antibodies made in response to glutens  
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characteristics of __ lesions are - erythematous papules/plaques studded with vesicles (location is classicaly on extensor surfaces of elbows, knees, also buttocks, scapular areas, scalp. they are symmetric and intensely pruritic(celiac)   dermatitis herpetiformis  
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skin findings of __ are: pitting edema (usually dependant, improves overnight), varicose veins, stasis dermatitis, hyperpigmentation (mottled blue, purple), skin fibrosis, venous ulcers   venous insufficiency  
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occurs on lower legs, ankles (pitting edema also often present), often mistaken for cellulitis, lesions (erythematous papules, scale, erosions, excriations), can be pruritic, irritant contact dermatitis   stasis dermatitis  
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treatment for stasis dermatitis   compression, oral antibiotics, topical steroids  
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1/3 of patients with venous insufficiency will develop __   venous ulcers  
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venous ulcers are usually located __   above medial malleolus  
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characteristics of __ are : usually located above medial malleolus associated with venous insufficiency, exquisitely painful, well demarcated, irregular shape, begin as a shallow erosion but can become quite deep (base often necrotic),   venous ulcers  
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__ is always present with venous ulcers   bacterial superinfection  
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chronic multisystem granulomatous disease (more common in AA females)   sarcoidosis  
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with sarcoidosis skin involvement occures in __% of patients   25  
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most common lesions of sarcoidosis   macules/papules-brown yellow or purple, occur mostly on face, extremities  
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lesions of sarcoidosis   macules/papules-brown, yellow, or purple (face, extremities), nodules (brown purple, occur on face/trunk/extremities), plaques (annular or serpiginous, may be scaly. occur on buttocks/trunk/extremties) lupus pernio  
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classic sarcoid lesion, infiltrating violaceous plaque, occurs on nose, cheeks, ears, lips   lupus pernio  
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lesions tend to occur on old scars (tattoos)   sarcoid  
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characteristic lesion of __ is: macule--> papule with vesicle or bulla in center, this is known as a target (or iris) lesion. occurs on hands, forearms, feet, face, usually symmetric   erythema multiforme  
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mild erythema multiforme   no bullae, lesions on upper extremities, face  
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major/severe erythema multiforme   one or more mucous membranes involved, epidermal detachment of <10% of total body surface area  
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considered dermatologic emergencies   Steven-Jonhnson Syndrome, Toxic Epidermal Necrolysis  
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definition of __ is widespread bullae on trunk, face, and mucous membrane involvement with epidermal datachments   Steven-Jonhnson Syndrome, Toxic Epidermal Necrolysis  
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treatment for mild erythema multiforme   symptomatic analgesics, topical steroids  
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treatment for major erythema multiforme   often associated with drugs. discontinue the offending agent  
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treatment for Steven-Jonhnson Syndrome, Toxic Epidermal Necrolysis   medical emergency, monitor fluid/electrolytes, systemic steroids  
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