12:50 - 1:50pm Disclosures Can't Miss Dermatology Diagnoses: The following relationships exist related to this presentation: Cutaneous Manifestations of ► Daniela Kroshinsky, MD MPH: No financial relationships to disclose. Systemic Disease SPEAKER Daniela Kroshinsky, MD MPH Off-Label/Investigational Discussion ► In accordance with pmiCME policy, faculty have been asked to disclose discussion of unlabeled or unapproved use(s) of drugs or devices during the course of their presentations. Overview • Identify cutaneous manifestations of systemic disease and their associated risk factors 58 yo woman referred for unresponsive drug rash • Review treatment options • Learn other mimicking cutaneous conditions Syllabi/Slides for this program are a supplement to the live CME session and are not intended for other purposes. Dermatomyositis • Ragged cuticles, nail fold telangiectasias • Extensor limb rash, including knuckles • Shawl‐distribution poikiloderma with extension into scalp • Periorbital edema, heliotrope rash • Diffuse facial erythema, malar erythema • Holster sign • More violaceous and pruritic than lupus • Erosions, ulcerations Syllabi/Slides for this program are a supplement to the live CME session and are not intended for other purposes. Forms • Resembles polymyositis; symmetric proximal muscles usually • Skin findings precede muscle in most cases • Classic (with muscle disease) • Amyopathic (myositis may evolve over time) • Hypomyopathic dermatomyositis (no clinical muscle weakness, but myositis present on radiographic or laboratory testing) Other systems Malignancy Association • Estimated 10% to over 50% of patients • Pulmonary disease occurs in approximately 15–30% • Generally presents as a diffuse interstitial fibrosis • Genitourinary (esp ovarian) and colon malignancies may be overrepresented, nasopharyngeal in some SE Asian populations • Cardiac disease is not commonly symptomatic • Common others: breast, lung, gastric, pancreatic, lymphomas • Usually presents as arrhythmias or as conduction defects (including non‐Hodgkin) • The risk of malignancy may normalize after 2–5 y • Frequent and thorough medical histories, repeated ROS, complete physical exams, and screening labs Syllabi/Slides for this program are a supplement to the live CME session and are not intended for other purposes. Malignancy Screening Mimickers • Urinalysis, stool occult blood testing • Serum prostate‐specific antigen • Serum CA125 • Mammogram and transvaginal pelvic U/S • CT of chest, abdomen and pelvis • Colonoscopy‐ if age‐appropriate, iron deficiency anemia, fecal occult blood, or symptoms • Upper endoscopy – if colonoscopy negative in the setting of iron deficiency anemia, fecal occult blood, or symptoms 31 yo W with anxiety, palpitations, and recurrent cellulitis Syllabi/Slides for this program are a supplement to the live CME session and are not intended for other purposes. • Three weeks prior to presentation, she developed redness, pain and swelling of her left leg • 31yo W h/o bipolar disorder, anxiety, hypothyroidism • U/S negative, pelvic MRI negative and deep vein thrombosis on warfarin • Course of clindamycin • Presents to ED with anxiety, palpitations, and left calf • Two weeks later, develops same symptoms on edema, pain, and erythema right leg, started on levofloxacin 500mg qd and furosemide with improvement by day 5 • New left leg pain, swelling and erythema Chart Review • 10/2013 had similar incident and was found to have L leg DVT in absence of immobility or smoking, + OCP which was discontinued • Warfarin for 3 months and then stopped • 4/2014 pt has similar episode and found to have L leg DVT • Warfarin restarted • Normal workup per patient Syllabi/Slides for this program are a supplement to the live CME session and are not intended for other purposes. • Concern for thrombophlebitis, DVT •No personal or family h/o miscarriages, PE, clotting disorders • Repeat U/S, coagulopathy workup, ANA, dsDNA, anti‐Ro and La, and anti‐Smith •ROS negative for weight loss, fatigue, malaise, fever, chills • More detailed history: photosensitivity but no malar or discoid rash, arthritis, serositis, kidney disease, neurologic •Afebrile, HR 128, SO2 100% on room air symptoms •Pertinent Labs: WBC 3.7, Hct 35, ESR 85, INR 3 Course •Hypercoagulability workup: •10/2013: Normal Factor V Leiden, Prothrombin •U/S demonstrates left popliteal vein thrombosis gene mutation, Protein C & S levels •Hematology increases INR goal to 3‐4, monitor •4/2014: +anticardiolipin Ab, lupus anticoagulant, •Repeat aCL IgG 38 and B2GP1 9934 beta‐2 glycoprotein, normal antithrombin III •ANA 1:5120, dsDNA 1:80, Anti‐Sm and Anti‐RNP positive •Diagnosis: Systemic Lupus Erythematosus with secondary : Antiphospholipid Antibody Syndrome •Hydroxychloroquine 200 mg BID Syllabi/Slides for this program are a supplement to the live CME session and are not intended for other purposes. Pseudocellulitis •Dozens of clinical mimickers of cellulitis: ‘pseudocellulitis’, no ‘gold standard’ diagnostic tool •Estimated misdiagnosis rate as high as 30% • Most common mimicker: stasis dermatitis •Empiric use of aggressive antibiotics rising rates of resistance in soft tissue infections • ‘98 –’04: MRSA soft tissue infections 26.2 47.4% ‐ Moet GJ et al. Contemporary causes of skin and soft tissue infections in North America, Latin America, and Europe: report fromthe SENTRY Antimicrobial Surveillance Program (1998‐2004). Diagn Microbiol Infect Dis 2007;57:7‐13. ‐ David, C.V., et al., Diagnostic accuracy in patients admitted to hospitals with cellulitis. Dermatol Online J. 2011 Mar 15:17(3):1. 32 year old W admitted for diarrhea and weight loss with itchy plaques on the legs Syllabi/Slides for this program are a supplement to the live CME session and are not intended for other purposes. Localized (Pretibial) Myxedema • TSH <0.01, T4 = 25 • Graves disease • A/w hyperthyroidism (usually Graves), may appear w/ hypothyroidism that follows treatment • Found in 1–5% of patients with Graves, but up to 25% of those with exophthalmos • Rarely, in Hashimoto’s thyroiditis without thyrotoxicosis, and euthyroid patients • Rarely, face, shoulders, upper extremities, lower abdomen, scars or donor graft sites Cutaneous manifestations of hyperthyroidism • Warm, moist skin, with increased temperature • Diffuse non‐scarring alopecia 56 yo M admitted for pancytopenia, • Palmoplantar hyperhidrosis found to have leg rash • Facial flushing • Increased skin pigmentation • Onycholysis (aka Plummers nails) Syllabi/Slides for this program are a supplement to the live CME session and are not intended for other purposes. DDx • Pruritic Infectious • Dermatophyte • Present for 6 weeks • Subcutaneous mycoses (chromoblastomycosis) • Atypical mycobacteria • Noticed after leg • Less likely bacterial, sporothrix or systemic scratched on a bush mycoses Inflammatory • Not improved with • Contact dermatitis triamcinolone cream • Eczema • Reactive phenomena Diagnostic Studies KOH negative Tissue culture • Gram stain (-) • Culture grew CoNS • Acid fast smear (-) • Mycobacterial culture (-) • Fungal culture (-) Punch biopsy Syllabi/Slides for this program are a supplement to the live CME session and are not intended for other purposes. Necrolytic Acral Erythema Necrolytic Acral Erythema • Type of necrolytic erythema • Necrolytic Migratory Erythema (glucagonoma) • Nutritional Deficiency (zinc/acrodermatitis enteropathica, niacin/pellagra, biotin, essential fatty acids) • In chronic hepatitis, prevalence is 1.7% • Predilection for dorsal feet and lower extremities • First described in 1996 • Treatment • Case series of 7 patients from Egypt with hepatitis C • Oral zinc supplementation 440 mg/day, divided BID • Hepatitis C treatment • Patient’s Hepatitis Viral Load: 748,000 • Topical corticosteroids are NOT effective HCV & Lichen Planus Necrolytic Migratory Erythema • Up to 38% of patients with LP, especially associated with oral form • Glucagonoma • Inquire esophageal ROS, genital involvement • Analogous to acrodermatitis enteropathica Nejm.org Dermnz.net Syllabi/Slides for this program are a supplement to the live CME session and are not intended for other purposes. Necrobiosis Lipoidica Diabeticorum Necrobiosis Lipoidica Diabeticorum • Associated with diabetes, glucose intolerance • Decreased sensation to pinprick and fine touch, hypohidrosis and partial alopecia • Usually follows trauma • 82 yo M presented to OSH for evaluation of left hand lesion s/p dog bite 82 yo M with “Resistant Hand Lesions” • Started on vancomycin, piperacillin-tazobactam • Developed painful, hemorrhagic bullae over wound Syllabi/Slides for this program are a supplement to the live CME session and are not intended for other purposes. History: • Biopsy at OSH: “acute inflammatory cellular • PMH: Hidradenitis Suppurativa, COPD, HTN, exudate and granulation tissue” DM2, sinus bradycardia s/p PPM, MM • Debrided at OSH for presumptive necrotizing • SHx: Married, 60 pack year smoker, dog cellulitis • ROS: 5 lb unintentional wt loss/ 1 week, +Diarrhea • Left hand wound expands • Similar wound appears on right hand DDx: • Neutrophilic Dermatosis: • Pyoderma Gangrenosum, Sweet’s syndrome • Infectious: • Bacterial, mycobacterial, protozoal, fungal, viral • Vasculitis: • ANCA-associated or medium vessel vasculitis • Malignancy: • Lymphomatoid granulomatosis Syllabi/Slides for this program are a supplement to the live CME session and are not intended for other purposes. Pyoderma Gangrenosum Exam and Tests: • Classic: inflammatory pustule rapidly enlarging ulcer w/ undermined,