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Mimics Test Taking Tip Test Taking 10 – Syndromes that Masquerade as Infections Speaker: Karen C Bloch, MD, MPH, FIDSA, FACP Disclosures of Financial Relationships with Relevant Commercial Interests 10 •None Syndromes that Masquerade as Infections With Special Thanks to Dr. Bennett Lorber! Karen C. Bloch, MD, MPH, FIDSA, FACP Associate Professor, Division of Infectious Diseases Vanderbilt University Medical Center 7/1/2019 Mimics 2018 ID Board Content Medical Content Category % of exam • Many conditions masquerade as infections. Bacterial Diseases 27% HIV Infection 15% • Often with fever Antimicrobial therapy 9% • Sometimes focal abnormality Viral Diseases 7% Travel and Tropical Medicine 5% – Cellulitis Fungi 5% – Pneumonia Immunocompromised Host (non HIV) 5% Vaccinations 4% – Lymphadenopathy Infection Prevention and Control 5% –Etc General Internal Medicine, Critical Care & Surgery 18% Total 100% Test taking tip Test taking tip • Just as for infections, look for “buzz words” • For infections: and “hooks” If I say “rabbit”, you say….. • For infections: If I say “rabbit”, you say….. TULAREMIA ©2019 Infectious Disease Board Review, LLC 10 – Syndromes that Masquerade as Infections Speaker: Karen C Bloch, MD, MPH, FIDSA, FACP Test taking tip Test taking tip I say ”Chitlins” I say ”Chitlins” You say….. You say….. YERSINIA Test taking tip Test taking tip I say ”Bull’s-eye rash” I say ”Bull’s-eye rash” You say….. You say….. Lyme disease (or Erythema migrans or STARI) My Approach to Mimics Warm Up! • Think like an internist! • I’ll use cases to illustrate what kinds of diseases and questions you might encounter on the Board Exam. • Goal is to cover lots of cases rather than in-depth discourse ©2019 Infectious Disease Board Review, LLC 10 – Syndromes that Masquerade as Infections Speaker: Karen C Bloch, MD, MPH, FIDSA, FACP Question 1 Question 2 A young man has oral and genital ulcers. You Sweet Syndrome is most likely to occur in a suspect Behçet’s disease. Which one of the patient with which one of the following illnesses? following findings is most consistent with that diagnosis? A. Ulcerative colitis A. Evanescent, salmon-colored rash B. Adult onset Still’s Disease B. High ferritin C. Acute leukemia C. Saddle nose deformity D. Systemic lupus D. Pustule at site of venipuncture E. Ankylosing spondylitis E. Posterior cervical adenopathy Question 3 Now on to our discussion, A patient has a slowly enlarging ulcerated skin lesions on starting with a case. his shin after being hit by a soccer ball. Which one of the following is the most likely diagnosis? A. Pyoderma gangrenosum B. Ecthyma gangrenosum C. Erythema nodosum D. Sweet Syndrome E. Behçet’s disease Case 1 Case 1 • 26yo man with presents with a 1-month h/o • Exam: T=38.4⁰C, HR=118bpm, no fever, night sweats and fatigue. He was lymphadenopathy, palpable spleen tip evaluated by his PCP with unrevealing appreciated. bloodwork, and a negative monospot test. He was diagnosed with a viral illness, but symptoms • Labs: 13 ALT=38 have persisted. He lives in Indiana with his wife AST=200 2.7 53 and 2 yo son. They have 2 cats. 0.79 Alk Phos=494 39 Bili total=1.9 Ferritin=35,148 mg/ml ©2019 Infectious Disease Board Review, LLC 10 – Syndromes that Masquerade as Infections Speaker: Karen C Bloch, MD, MPH, FIDSA, FACP Question 4 Hemophagocytic Lymphohistiocytosis • What is the most appropriate next study? • Immune activation syndrome – Primary: Familial due to genetic mutation A. Flow cytometry of whole blood B. ANA profile – Secondary: Most commonly triggered by C. EBV serologies infections (EBV or other herpes group viruses, D. Soluble IL-2 receptor alpha level HIV, histoplasmosis, Ehrlichia, etc) or E. Toxoplasma titer malignancy (lymphoma, leukemia) HLH: Diagnostic Criteria HLH Clues • Requires >=5/8 • EBV or other infection with progressive – Fever symptoms – Splenomegaly – Cytopenias (any line) – Hypertriglyceridemia – Ferritin >500 mg/mL • Massively elevated ferritin – Elevated soluble IL-2 receptor alpha (aka CD25) – Low NK cell activity – Hemophagocytosis on pathology • Cytopenia with negative infectious evaluation Case 2 • Exam: T=104.2⁰ F. Tonsilar swelling and erythema is present, • A 39-year-old woman is seen on day 4 of with tender cervical LN. Spleen hospitalization for high fever and leukocytosis. tip is palpable. The R wrist is The fever had been present for 3 ½ weeks and swollen and painful. A rash is was accompanied by severe arthralgias of the noted on the trunk and knees, wrists and ankles as well as myalgias. A extremities, most prominently severe sore throat was present during the first under the breasts and in the area week of the illness. of her underwear waistband. ©2019 Infectious Disease Board Review, LLC 10 – Syndromes that Masquerade as Infections Speaker: Karen C Bloch, MD, MPH, FIDSA, FACP • Labs: Question 5 Ferritin 3600 ng/ml (nl 40-200) WBC 32,200 (89% neutrophils) AST and ALT 3x normal • The most likely diagnosis is? ESR and CRP 5x normal A. Lymphoma ANA and RF negative B. Adult Still’s Disease Throat and blood cultures negative C. Acute Rheumatic Fever • On afternoon rounds with the attending, the fever D. Cryoglobulinemia resolved with Tylenol and the rash is no longer present. E. Kikuchi’s Disease Adult Still’s Disease (Adult Onset JRA) Adult Still’s Yamaguchi Criteria: (5 features with 2 major criteria) • Buzz words and associations: evanescent, salmon-colored rash Major: Minor: • Other clues: 1. Fever >39⁰C for >week 1. Sore throat Multi-system illness 2. Arthritis/arthralgia >2 wks 2. Lymphadenopathy Elevated ferritin 3. Nonpruritic mac-pap rash 3. Lg Liver or spleen Pharyngitis during febrile episodes 4. Abnl LFTs or LDH Koebner phenomenon = rash elicited by 4. Leukocytosis >10K with 5. Negative ANA & RF >80% granulocytes. stroking skin or areas of pressure. Case 3 • Exam: afebrile. • A 24-year-old man is referred from the ER for ulcers of Left eye is inflamed and there is a hypopyon. the mouth and penis. Three months ago he came to the Numerous ulcers on the oral mucosa. U.S. from Japan to attend graduate school. No adenopathy. There is a 0.5cm ulcer on the penis. A 6mm papulo-pustular lesion is present in the right • He has a history of intermittent, painful oral ulcers for 3-4 antecubital fossa; the patient says that is where they years. Four days ago he developed a painful ulcer on drew blood yesterday in the ER. the penile shaft. Had a similar lesion 2 months earlier. • Rest of the exam is normal. Takes no medicines and denies sexual contact for the • Labs: Hb 12.1; WBC 13,750. HIV negative past 5 years. ©2019 Infectious Disease Board Review, LLC 10 – Syndromes that Masquerade as Infections Speaker: Karen C Bloch, MD, MPH, FIDSA, FACP Question 6 Behçet’s disease • Pleomorphic vasculitis with clinical diagnosis • The most likely diagnosis is? Recurrent oral ulcers (>3 per year) PLUS 2 of the following recurrent genital ulcers A. Syphilis eye (uveitis, retinitis, hypopion) or skin lesions (EN, papules) B. Behçet’s disease pathergy (red papule developing 24-48 hrs after needlestick) • Think “silk road” (Asia->Mediterranean) C. Herpes simplex virus infection • Less common manifestations D. Sarcoidosis – GI disease (abdo. Pain, bloody diarrhea) – CNS disease (aseptic meningitis) E. Cytomegalovirus infection – Arterial and venous thrombosis • Treatment: colchicine Behçet’s disease Case 4 • Buzz words and associations: • A 38-year-old woman with AML is admitted with fever. Mucosal ulcers on mouth and/or genitals She underwent induction chemotherapy 2 weeks prior, PLUS…. complicated by neutropenic fever. Following marrow recovery, she was d/c to home. The day of admit she GI symptoms (vs CMV) developed fever without localizing symptoms. CBC Aseptic meningitis (vs HSV) showed a white blood cell count of 12,250 with 20% bands. Visual changes • Exam: T 101.4; P 98; otherwise unremarkable. Pathergy (needle or IV site) • Blood cultures were sent, and she was admitted and Asian or Mediterranean ancestry started on broad spectrum empiric antibiotics. • HD 2: Fever persists, with interval development of raised, red-purple, tender, HD 3: Fever persists; some of the papules non-pruritic papules and nodules on her develop a plaque-like appearance face, neck and the dorsum of her hands. HD 4: biopsy: dense dermal perivascular infiltrates of neutrophils without evidence of vasculitis; stains for organisms negative. ©2019 Infectious Disease Board Review, LLC 10 – Syndromes that Masquerade as Infections Speaker: Karen C Bloch, MD, MPH, FIDSA, FACP Question 7 Sweet Syndrome • AKA acute febrile neutrophilic dermatosis • Which one of the following is the most • Three variants: – Idiopathic or “classical”->50% (IBD, post viral illness, preg, etc) likely diagnosis? – Malignancy associated~20% (may precede dx, AML most frequent) A. Ecthyma gangrenosum – Drug induced-G-CSF most common, usually 2 wk after exposure • Fever universally present B. Pyoderma gangrenosum • Rarely oral ulcers or extra-cutaneous disease characterized C. DRESS by neutrophilic infiltrate on path • Labs notable for leukocytosis with left shift, inc ESR & CRP D. Leukemic infiltrates • Path diagnostic—Neutrophilic infiltrate without vasculitis E. Sweet syndrome Sweet Syndrome Sweet Syndrome • Buzz words and associations: • Lesions appear abruptly and usually tender. Acute • May be single or multiple, Febrile often involving dorsum of Neutrophilic (periph and path) hand. Dermatosis • Red, violaceous, or yellow center Be suspicious in patients with malignancy (esp AML, past • Nodular or plaque-like or present), IBD, recent URI, vaccination, pregnancy, or • Central umbilification with colony stimulating factor use in preceding 2 weeks target appearance Case 5 •Exam: • A 33-year-old recent immigrant from Central T 100.2; skin lesion on leg (see image) America is seen for a chronic ulcer of the leg. Slight, diffuse abdominal tenderness. • The ulcer has progressively enlarged over 3 months. Otherwise unremarkable. after he bumped his leg on a table.
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