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Dermatologic Manifestations of Internal Disease Photo 4/10/2012 LUPUS Acute – “butterfly” malar rash, transient, follows sun exposure Lesions on hands often spare knuckles DERMATOLOGIC Evaluate for internal MANIFESTATIONS OF INTERNAL disease (ANA/ENA) +/- oral ulcers DISEASE Kim Sanders MPAS, PA-C Nephritis concerning OHSU Department of Dermatology complication (usually dsDNA+) PHOTO CREDITS: LUPUS SCLE – photosensitive, Lynne Morrison M.D. photodistributed Bolognia et al. Dermtext.com Lesions may appear The internet annular, eczematous, psoriasiform. Not scarring 15-50% progress to SLE Med induced: HCTZ, NSAIDs, diltiazem, and terbenafine Usually anti-Ro + LUPUS ERYTHEMATOSUS LUPUS Multisystem, Discoid Lupus – face, autoimmune disorder scalp, and ears Female > male 6:1 Can occur on mucosa SLE prevalence 4 fold Can scar, also higher in AA females follicular plugging and SLE criteria –rash scarring alopecia photosensitivity, oral ulcers, arthritis, Long standing lesions serositis, renal can dvp SCC disorder, neurologic Only 5-10% dvp SLE disorders, hematologic disorder, +ANA. 1 4/10/2012 LUPUS DERMATOMYOSITIS More rare types – ¼ of adult pts have Tumid – no epidermal change. Dermal assoc malignancy process. Can appear like urticarial plaques on Females 2x> males face and trunk can overlap with other Lupus panniculitis – intense inflammation in collagen vascular the fat with indurated and depressed areas. diseases Lupus pernio – red/dusky/purple papules on Poikiloderma, fingers, toes, elbows, and lower legs. Brought violaceous color on by cold. (elbows, knees with fine scale) Photodistribution Periungal telangiectasia and dilated capillary loops. Ragged cuticles LUPUS DERMATOMYOSITIS Heliotrope sign - Diagnosis – Clinical, skin biopsy and DIF eyelids violaceous and puffy Eval for SLE – diffuse non-scarring alopecia, periungal telangiectasia, Raynauds, livedo retic, Gottrons sign – vasculitis, lymphadenopathy, ANA (and ENA), violaceous UA, CBC with diff, platelets, CMP, ESR. poikiloderma over knuckles, elbows, and Rheumatology referral knees TX: topical/intralesional steroids, SUN Gottrons papules – PROTECTION,hydroxychloroquine, dapsone, over knuckles methotrexate. With SLE - prednisone azathiaprine, cyclosporine, … Erosions and bullae are a bad sign Calcinosis cutis DERMATOMYOSITIS DERMATOMYOSITIS Malaise and fatigue Connective tissue- Muscle soreness/pain vascular disease and decreased Autoimmune strength Symmetric proximal, Can be assoc with extensor, interstitial lung dz inflammatory and cardiac dz myopathy and Rarely drug-induced characteristic skin DX: skin biopsy, eruption. ANA, ENA, muscle Polymyositis – muscle enzymes, MRI, LFT’s, only, spares the skin LDH, 24 hour Amyopathic – skin creatinine, MI2 only, no muscle Malignancy eval 2 4/10/2012 DERMATOMYOSITIS SCLERODERMA Skin findings: Diffuse hyperpigmentation, can have leukoderma except Characteristics of subtypes: perifollicular (“salt and Juvenile – Calcinosis cutis is common, usually pepper”) no malignancy Matted telangiectasias of Adult – Common malignancies ovarian, breast, face, lips and palms colon, lung, and pancreas Sclerodactyly Amyopathic – no myopathy, skin only. Can have late onset myopathy after 15mo-6 years. Calcinosis cutis – distal 14% have malignancy, 13% have ILD. Raynaud’s and ulcers on fingers common Treatment: sunscreen, topical steroids, Dry skin hydroxycholorquine, methotrexate, azathioprine, IVIG Pruritus SYSTEMIC SCLEROSIS (SCLERODERMA) SCLERODERMA Collagen vascular dz Labs: primarily affecting ANA +, and Scl-70 (diffuse) or anti-centromere females (30-50yo) (limited) Symmetric induration of the skin with TX: CCB’s for Raynauds (nifedipine). Avoid cold involvement of distal and tobacco. areas ACE inhibitors (for renal involvement?) Systemic involvement ?Prednisone, ?methotrexate, imatinib in trials – esophagus, lungs, heart, and kidneys May be fatal (lung involvement) ERYTHEMA SYSTEMIC SCLEROSIS MULTIFORME Strongly assoc. with a preceding infection Limited vs Diffuse Recurrent cases likely CREST: Calcinosis HSV. cutis, Raynaud’s Can also be drug phenomenon, induced, radiation esphogeal dysmotility, induced, and sclerodactyly, and idiopathic telangiectasia Appears targetoid (matted). with dusky center. Can be bullous. Common on the palms and soles, +/- oral involvement, acral distribution. 3 4/10/2012 ERYTHEMA MULTIFORME URTICARIA Minor – self limited, recurrent, usually in Vascular rxn young adults (spring characterized by the and fall) and lasts 1-4 appearance of wheals, weeks. generally surrounded Major – more by a red halo or flare extensive w/ mucosal Severe itching, involvement. stinging, or prickling Previously thought to sensation. be a precursor to SJS. May be annular or Treatment is polycyclic. supportive Individual wheals (prednisone may rarely last >12 hours. provoke recurrences). ERYTHEMA NODOSUM URTICARIA Bilateral, symmetric Acute – resolves in panniculitis with days to weeks, with deep, tender, bruise- complete resolution of like nodules symptoms within 6 Pre-tibial weeks. Can have malaise, leg Chronic - >6 weeks. edema, arthralgias, Females 2x more fever, HA, affected than males. conjuctivitis, GI More common in complaints adults ERYTHEMA NODOSUM URTICARIA Causes: strep Triggers: drugs are infection, TB, most common intestinal infection, Acute >50% idiopathic fungal infection, followed by URI, sarcoidosis, IBD, drugs, and foods drugs Chronic 60% “ordinary” Tx: identify trigger, (autoimmune, rest, elevation, anti- pseudoallergic, inflammatories infection related, and idiopathic) then physical and vasculitic causes 4 4/10/2012 URTICARIA ACANTHOSIS NIGRICANS Type II – familial, present at birth Physicial urticarias: dermatographism, cold, Type III – neck, axillae, heat, cholinergic, aquagenic, solar, vibratory, and groin. Occurs in obesity, exercise induced. acromegaly and +/- allergic rhinitis, asthma, nasal polyps and gigantism, Cushing’s, food induced anaphylaxis DM, hypothyroidism, Food: acute. Addison’s, hyperandrogenic states. Can do food diary Drugs: OCP’s, Infections: URI, strep, Hep B/C, mono testosterone, and Emotional stress, menthol, neoplasms, pollen, glucocorticoids. and alcohol URTICARIA ACANTOSIS NIGRACANS D/DX: urticarial vasculitis, BP, EM, GA, Tripe palms – Sarcoidosis, CTCL which would all last >24 hours thickened velvety TX: antihistamines. Consider Singulair. Cold palms with baths, sarna, phototherapy, nifedipine, dapsone, pronounced gold, methotrexate. dermatoglyphics. 95% occur in pt’s with cancer. Lung CA most common if only palms affected. Tripe palms + AN = gastric cancer. ACANTHOSIS NIGRICANS ACANTHOSIS NIGRACANS Grey, brown, or black Treatment – remove malignancy, treat DM, hyperpigmentation weight loss, Oral retinoids, metformin, tretinoin, and velvety textured calcipotriol, urea, sal acid, CO2 laser and pulsed plaques. dye laser may be of benefit. Symmetric distribution. Type I – Rare. Assoc with malignancy. Increased extent of involvement. Increased suspicion >40yo, not obese with rapid onset 5 4/10/2012 LIVEDO RETICULARIS ERUPTIVE XANTHOMA Netlike mottled or Small yellowish reticulated pink or orange to red-brown reddish blue papules that appear in discoloration. crops over entire body Markedly elevated Exposure to cold can triglycerides. accentuate. Also seen with DM, +/- coldness, obesity, pancreatits, numbness, chronic renal failure, paresthesia. hypothyroidism, estrogens, corticosteriods, or systemic retinoids. LIVEDO RETICULARIS “XANTHELASMA” PALPEBRARUM Most not assoc with systemic disease, but may be Most common. Soft a manifestation of lupus, dermatomyositis, yellowish orange scleroderma, rheumatic fever, rheumatoid oblong plaques on arthritis, Hep C, parvovirus B19, syphilis, eyelids meningococcemia, pneumococcal sepsis, TB, Assoc with other types pancreatitis, breast CA, and on and on…… of xanthoma, but are Cholesterol emboli can cause. May be uni or typically present bilateral. Usu with cyanosis, purpura, nodules, without any other ulceration or gangrene. disease >1/2 pt’s with normal lipid profile XANTHOMATOSIS PEUTZ-JEGHER SYNDROME Cutaneous manifestation of lipidosis in which AD. Mucocutaneous plasma lipoproteins and free fatty acids are pigmented macules changed quantitatively. and hamartomatous Names based on clinical morphology polyps of the GI tract. Can be associated with genetic diseases Dark brown to black round to oval macules Morphologies fairly specific for the associated elevated lipid on central face, lips and oral mucosa. Can be seen on hands, feet, tongue, and periumbilical. 6 4/10/2012 PEUTZ-JEGHERS SYNDROME THANKS FOR YOUR ATTENTION! Polyps in GI tract usually in jejunum and ileum. Questions??? +/- abdominal pain, obstruction, hemorrhage, and anemia. Increased risk for malignancy Tx: check stool for blood, colonoscopy, upper endoscopy DERMATITIS HERPETIFORMIS Symmetric distribution favoring elbows, extensor forearms, upper back, buttocks and knees. Urticarial papules, vesicles and blisters. INTENSE pruritis, burning or stinging. May only see crusting DERMATITIS HERPETIFORMIS Cutaneous manifestation of celiac disease Only about 20% have intestinal symptoms Males > females 2:1 Uncommon in AA and Asian populations Can be associated with Hashimoto’s thyroiditis Dx: biopsy and DIF (perilesional), anti- endomysial antibioties, tissue transglutaminase TX: gluten free diet!!! Dapsone, topical steriods for itching. GI eval?? Probably 7.
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