Great Cases from the Cleveland Clinic. Melissa Piliang, MD

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1 Great Cases from the Cleveland Clinic Melissa Piliang, MD

2 DISCLOSURE OF RELATIONSHIPS WITH INDUSTRY Melissa Piliang, MD Scarring Alopecia Diagnostic Challenges DISCLOSURES I do not have any relevant relationships with industry. Investigator for Samumed, Kythera, Incyte, Concert, Allergan Advisory Board/Consultant: Samumed, Castle Biosciences, Proctor and Gamble

3 Case 1

4 Case Presentation 48 year old woman Diabetes mellitus Pancreas transplant 6 months ago CC: Rash X 2 months Asymptomatic Centrifugal spread

5 Hospital Readmission Increased CMV Viral load Shortness of breath, fevers Increased Xerosis

6 PMH: Diabetes mellitus Pancreatic Transplant CMV Small Bowel Obstructions TPN Gastric tube Asthma ROS: Abdominal pain Cough No Fevers Allergies: Sulfa FMH: No skin cancer

7 Medications Metoclopramide 10mg QID PRN Colace 100mg TID Nebupent 300mg inhalation q28 days Hycodan 5/1.5mg q 3-4 hours PRN Prograf 3mg BID Ondansteron 4 mg q 4-6 hours PRN Mycophenolate mofetil 250mg 4 tabs BID Wellbutrin SR 150mg 2 tabs daily Xanax 0.5mg QID PRN Albuterol 90 mcg PRN Flovent 44mcg 1 puff BID

8 Graft-versus-Host Disease Biopsy: Early evolving interface dermatitis Vacuolar Type Increased skin involvement Increased xerosis Hospital Admission

9 Graft-versus-Host Disease = High fatality Weinsein A, Dexter D, KuKuruga, Philosophe B, Hess J, Klassen D. Acute Graft-versus-Host Disease in Pancreas Transplantation: A Comparison of Two Case Presentation and a Review of the Literature. Transplantation. 2006;82(1): Meves A, et al. Acute graft-versus-host disease after liver transplantation diagnosed by fluorescent in situ hybridization testing of skin biopsy specimens. J Am Acad Dermatol 2006;55:642-6.

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23 Day 21

24 Work-Up Mild eosinphilia at admission, but in normal range most of hospital course Strongyloides in gastric aspirate and stool BAL negative for strongyloides ELISA negative

25 Hospital Course: Ivermectin 12 mg/day for a 16 day course Repeat Gastric Aspirates/Stool negative CMV viremia Undetectable DNA Valcyte 900mg PO BID

26 Discharge - Day 32

27 Disseminated Strongyloidiasis

28 Immunosuppressed Medications Diabetes Systemic Lupus Erythematosus Pemphigus vulgaris Malignancy lymphoma Malnutrition

29 Cutaneous Manifestations Pruritus Urticaria (Acute, Chronic) Buttocks, thighs Petechia, purpura Thumbprint sign

30 Pathophysiology Strongyloides stercoralis

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32 Clinical Course Fatalitiy rate 50-89% Cure rates 70% and 59% Respiratory failure Gram negative septicemia Meningitis

33 Hospital Course Symptomatic treatment Repeat biopsy obtained CMV viremia Gancyclovir 300mg IV q 12 hrs X 4 weeks Discharged home

34 Strongyloides work-up History - West Virginia ELISA negative Cytogam 11/24/2006 Gastric aspirate + Stool + Sputum Candidia albicans Eosinophilia (at admission) 52, (at diagnosis) 6.4 Treatment Ivermectin 12mg/day

35 Pulmonary Work-up Sputum remained negative Bronchoalveolar lavage negative Chest CT Bilateral pleural effusions Ground glass infiltrates Bronchoscopy diffuse alveolar damage Congestive heart failure Thoracentesis - Transudative

36 GI Work-up Gastric aspirate + Stool + CT Abdomen Mild biliary duct dilatation

37 Secondary Infection Work-up Fungal battery negative Pneumocystic jeroveci, legionella, HSV, Acid fast bacilli negative HTLV-I/II negative Cryptococcal antigen negative CMV viremia DNA: (11/17)233,977 (12/3)229,294 Foscarnat Klebsiella Urinary Tract Infection Zosyn

38 Hospital Course: Ivermectin 16 day course completed Repeat Gastric Aspirates/Stool negative CMV viremia Undetectable DNA Valcyte 900mg PO BID

39 Discharge - Day 32

40 Disseminated Strongyloidiasis

41 Epidemiology Cochin China diarrhea 1876 French troops million affected Endemic Tropical, Subtropical Southeast Asia, Latin America, sub-saharan Africa, Southeastern US

42 Immunosuppressed Medications Diabetes Systemic Lupus Erythematosus Pemphigus vulgaris Malignancy lymphoma Malnutrition

43 Pathophysiology Strongyloides stercoralis, fuelleborni

44 Symptoms Asymptomatic Fever GI Pain, nausea, diarrhea, constipation, vomiting Pulmonary Cough, dyspnea, spasms, hemoptysis

45 Cutaneous Manifestations Pruritus Larva currens Urticaria (Acute, Chronic) Buttocks, thighs Petechia, purpura Thumbprint sign Papules/vesicles - feet

46 Diagnosis Stool - Repeat exams Sputum Tracheal apirates Bronchoalveolar lavage Gastric, Intestinal - aspirate/biopsies Serum ELISA Eosinophilia unreliable Anemia Chest X-ray

47 Treatment Thiabendazole 50 mg/kg BID x 2 days 25mg/kg BID X 7-10 days Ivermectin 200 mcg/kg/day x 2 doses Ivomec 1% 200mcg/kg SQ Ceftazidime IV Cefoperazone X 1 week Mebendazole 100mg BID Albendazole 400mg/day

48 Clinical Course Fatalitiy rate 50-89% Cure rates 70% and 59% Respiratory failure Gram negative septicemia Meningitis

49 Case 2

50 64 year old man Ulcer New lung nodule Presumed lung ca Weight loss (80 lbs), fevers, nights sweats Nose bleeds

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52 Elevated erythrocyte sedimentation rate (ESR) Outside imaging: 7 cm mass with large central cavitation Medistinal LAD R

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54 Palisading extravascular granuloma

55 Skin Biopsy: Mid Back Excision

56 Labs Elevated ESR No evidence of infection or malignancy Transbronchial bx: bronchial wall with acute and necrotizing granulomatous inflammation TB gamma negative Positive PR3-ANCA/c-ANCA

57 Granulomatosis with polyangiitis (GPA) Formerly Wegener s Granulomatosis Rare autoimmune disease Widespread granulomatous inflammation Sinopulmonary Renal Necrotizing small to medium vessel vasculitis

58 GPA Necrotizing small to medium vessel Leukocytoclastic vasculitis Most PR3-ANCA/c-ANCA Sinopulmonary and renal disease PG Dense neutrophilic infiltrate Vasculitis not primary event Occasional granulomas in later lesions ANCA s negative Rare cases of malignant pyoderma

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60 Summary GPA may present as PG-like cutaneous ulcers Be aware of co-existing clinical findings Cavitary lung mass +/- hemoptysis Sinusitis Anorexia/weight loss Recognizing this presentation can lead to Proper workup of broad differential diagnosis Initiation of life-saving therapy

61 Thank You!