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Derm Systemic Dz
Cutaneous Manifestations of Systemic Disease
| Question | Answer |
|---|---|
| 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) |