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Derm Systemic Dz

Cutaneous Manifestations of Systemic Disease

QuestionAnswer
Autoimmune disorders Rheumatic Dz:SLE, dermatomyositis, scleroderma, vasculitides,
Endocrine Disorders Diabetes
Immune Disorders Dermatitis herpetiformis, urticaria/angioedema, sarcoidosis
Urticaria should make you think of connective tissue disease or systemic dz
Miscellaneous disorders Erythema multiforme, erythema nodusum
Which inflammatory arthritis are associated with skin manifestations? Reider's and psoriatic
Criteria for systemic lupus alopecia, oral ulcers, photosensitivity, discoid lesions
Acute cutaneous SLE most have systemic dz.
Subacute cutaneous SLE 50% have systemic SLE
chronic cutaneous SLE rare to have systemic SLE
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
Women who have anti-Ro and anti-La antibodies tend to have babies with SLE.
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.
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
Classic discoid lesions are associated with chronic cutaneous LE
____ is the classic cutaneous dermatomyositis lesion heliotrope; violaceous macular rash or eyelids or periorbital area
________ 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
cutaneous findings of dermatomyositis poikiloderma/mottling, calcifications, linear erythema over extensor surfaces of joints, nail findings: periungual erythema, telangectasia, cuticle overgrowth
Morphea and linear scleroderma are associated with localized scleroderma. Linear plaques, most common in women, onset 20-50 years, rarely progresses to systemic dz
Common Presentations of Systemic Scleroderma Sclerodactyly, Raynaud's Phenomenon, sclerosis of face, scalp, trunk, periungual and mat-like telangiectasia, pigementation abnormalities, calcinosis cutis
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
Anyone who presents with vasculitis, check for hematuria. Very common.
Most common cause of vasculitis Infection (GABS, Hep B, Hep C) and then drugs (sulfonamides, penicillin, others). Also connective tissue disease
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.
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
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
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
Diabetic Ulcers can result from neuropathic or ischemic causes. Can go through subQ tissue, even to the bone, causing osteomyelitis.
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
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
Angioedema urticaria that extends into subcutaneous tissue. Also, doesn't turn erythematous like urticaria, usually flesh colored.
What percentage of acute urticaria patients will go on to develop chronic urticaria? 25%
Wheel and Flare Center white portion is the wheel, surrounding red is the flare
Question to ask with urticaria migratory, transient nature. Normal to migrate, usually worse at pressure points. Itch terribly.
Is urticaria a systemic reaction? Yes, not uncommon to have malaise and fever as well.
What is important to rule out with chronic urticaria? Connective tissue dz and malignancy
Tx for urticaria H1 and H2 blockers, doxepin (sedating, histamine blockers, for chronic), glucocorticoids (for chronic), epi-pen. CAN GIVE IM BENEDRYL IN CLINIC
Tx for angioedema epi pen
order an ANA if there is a family hx of connective tissue disease
Chronic skin disorder associated with celiac disease Dermatitis Herpetiformis. Physiology: IgA deposits in skin; these are antibodies made in response to glutens
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
Signs of venous insufficiency pitting edema (sometimes the first sign), varicose veins, stasis dermatitis (appears eczematous, +/- papules, excoriations), hyperpigmentation, skin fibrosis, venous ulcers
Characteristic of varicose veins that is worrisome if they are painful.
Stasis dermatitis often mistaken for cellulitis. Occurs on lower legs, ankles, pruritic, erythematous, pitting edema may also be present.
End stage for people with venous insufficiency venous ulcers. 1/3 of pts with venous insufficiency will develop these.
Common location of venous ulcers above medial malleolus. exquisitely painful, well demarcated, shallow erosion, irregular shape, base often necrotic
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
plaque specific to sarcoidosis lupus pernio: infiltrating violaceous plaque; occurs on nose, cheeks, ears, lips
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)
Erythema Multiforme causes Infections (especially HSV 1&2), Drugs (especially sulfonamides), systemic diseases
Target lesions can be found on skin and on mucous membrains (major EM)
Target lesions are a hallmark of Erythema multiforme. Symmetric and bilateral
widespread bullae on trunk, face + mucous membrane involvement with epidermal detachment < or = 10% of TBSA (SJS), > or = 30% of TBSA (TEN)
SJS is always a medical emergency
Lesions similar to Erythema multiforme, but likely to occur in 18-30 year olds Erythema nodosum. May precede diagnosis of lymphoma by many months
Which systemic diseases is Erythema Nodosum associated with? IBD: UC and Crohn's, malignancy, sarcoid
Anti-Ro and Anti-La antibodies are associated with which form of LE? Subacute cutaneous LE (SCLE)
Created by: ltm12
 

 



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