Jess Dreicer, MD Internal Medicine, PGY-3
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1 Jess Dreicer, MD Internal Medicine, PGY-3
2 Problem List Myelodysplastic syndrome
3 Problem List Myelodysplastic syndrome Giant cell arteritis Treatments Prednisone 60 mg -6 weeks
4 Problem List Myelodysplastic syndrome Giant cell arteritis Treatments Prednisone 60 mg -6 weeks -4 weeks Dx of GCA
5 Problem List Myelodysplastic syndrome Giant cell arteritis Treatments Prednisone 60 mg -6 weeks -4 weeks Dx of GCA
6 Problem List Myelodysplastic syndrome Giant cell arteritis Treatments Prednisone 60 mg -6 weeks -4 weeks -2 weeks Dx of GCA Cut finger
7 Problem List Myelodysplastic syndrome Giant cell arteritis Bilateral necrotic skin lesions Left fourth finger necrosis Maxillary mucosal necrosis Fever Treatments Prednisone 60 mg Vancomycin and piperacillin/tazobactam -6 weeks -4 weeks -2 weeks Presents to OSH Dx of GCA Cut finger Eye pain
8 Problem List Myelodysplastic syndrome Giant cell arteritis Bilateral necrotic skin lesions Left fourth finger necrosis Maxillary mucosal necrosis Fever Treatments Prednisone 60 mg 40 mg Vancomycin and piperacillin/tazobactam Voriconazole Daptomycin and meropenem -6 weeks -4 weeks -2 weeks Presents to OSH Dx of GCA Cut finger Eye pain Broad abx First admit
9 Initial work-up WBC 6.5x10 9 /L Hepatitis A, B, and C Negative Hemoglobin 8.5 g/dl HIV Negative Platelets 132,000/ml Rheumatoid factor POSITIVE Ferritin 1555 ng/ml Hemoglobin A1c 6% Vitamin B pg/ml Beta-2 glycoprotein IgM POSITIVE Absolute reticulocyte count Cardiolipin IgM Negative Multiple sets of blood cultures No growth ESR 115 mm/hr Total IgG WNL RPR Negative Cryoglobulins Negative Liver function tests WNL ANCAs Negative Histoplasma antibody Negative ANA Negative Aspergillus galactomannan Negative Bone marrow biopsy Hypocellular Protein C and S activity WNL Herpes simplex virus IgM POSITIVE HSV culture of mouth lesions Negative
10 Problem List Myelodysplastic syndrome Giant cell arteritis Bilateral necrotic skin lesions Left fourth finger necrosis Maxillary mucosal necrosis Fever Treatments Prednisone 60 mg Vancomycin and piperacillin/tazobactam Voriconazole Daptomycin and meropenem Heparin drip Valacyclovir -6 weeks -4 weeks -2 weeks Presents to OSH Dx of GCA Cut finger Eye pain Broad abx First admit
11 Problem List Myelodysplastic syndrome Giant cell arteritis Bilateral necrotic skin lesions Left fourth finger necrosis Maxillary mucosal necrosis Fever Treatments Prednisone 60 mg Vancomycin and piperacillin/tazobactam Voriconazole Daptomycin and meropenem Heparin drip Valacyclovir -6 weeks -4 weeks -2 weeks Presents to OSH Dx of GCA Cut finger Eye pain Broad abx First admit Heparin Hospital transfer
12
13 Problem List Myelodysplastic syndrome Giant cell arteritis Left fourth finger necrosis Bilateral necrotic skin lesions Maxillary mucosal necrosis Fever Left conjunctival erosion Neutrophilic dermatitis Treatments Prednisone 60 mg Vancomycin and piperacillin/tazobactam Voriconazole Daptomycin and meropenem Heparin drip Valacyclovir -6 weeks -4 weeks -2 weeks Presents to OSH First admit Hospital transfer Second admit Dx of GCA Cut finger Eye pain Broad abx Heparin Rash worsened
14 Problem List PMHx MDS GCA Despite steroids, antimicrobials, and anticoagulation
15 + Malignancies Sweet syndrome Leukemia cutis Paraneoplastic syndromes Atypical Infections infections Rheumatologic conditions Sporotrichosis Medium and large Leishmaniasis vessel vasculitides Atypical mycobacteria M. tuberculosis Cutaneous anthrax Nocardia Tularemia
16 + Malignancies Sweet syndrome Leukemia cutis Paraneoplastic syndromes Atypical infections Rheumatologic conditions Sporotrichosis Medium and large Leishmaniasis vessel vasculitides Atypical mycobacteria M. tuberculosis Cutaneous anthrax Nocardia Tularemia
17 Additional work-up Computed tomography angiography (CTA) head and neck Positive emission tomography (PET scan) Computed tomography (CT) of chest, abdomen, and pelvis Universal polymerase chain reaction (PCR) for bacterial and viral infections SPEP ESR MMA LDH Rapid plasma reagin Repeat blood cultures CRP Zinc Vitamin D, 25-hydroxy Ferritin Cryptococcus antigen Normal 116 mm/hr 0.26 umol/l 402 U/L Negative No growth 134 mg/l 62 ug/mdl 31.8 ng/ml 1980 ng/ml Negative
18 Malignancies Sweet syndrome Leukemia cutis Paraneoplastic syndromes? + Atypical infections Rheumatologic conditions Sporotrichosis Medium and large Leishmaniasis vessel vasculitides Atypical mycobacteria M. tuberculosis Cutaneous anthrax Nocardia Tularemia
![A Lesson Learned from [We] puzzled, and puzzled, till [our]](/docs-images/85/92214858/images/19-0.jpg "puzzlers were sore.")
19 A Lesson Learned from [We] puzzled, and puzzled, till [our] puzzlers were sore. And then, [we] thought of something [we] hadn t before
20 Problem List PMHx MDS GCA Despite steroids, antimicrobials, and anticoagulation
21 Pyodermatitis-Pyostomatitis Vegetans (PD-PSV) Neutrophilic dermatosis subtype Characterized by rash involving the skin and mucosa First described in 1898 Strong association with inflammatory bowel disease (IBD) Clinical dx supported by histology High dose steroids are first-line tx
22 Pyodermatitis-Pyostomatitis Vegetans Pyo=pus (neutrophils)
23 Pyodermatitis-Pyostomatitis Vegetans Pyo=pus (neutrophils) Dermatitis=inflammation of skin
24 Pyodermatitis-Pyostomatitis Vegetans Pyo=pus (neutrophils) Dermatitis=inflammation of skin Stomatitis=inflammation of mucosa
25 Pyodermatitis-Pyostomatitis Vegetans Pyo=pus (neutrophils) Dermatitis=inflammation of skin Stomatitis=inflammation of mucosa Vegetans=proliferative growths
26 Pyodermatitis-Pyostomatitis Vegetans Pyo=pus (neutrophils) Dermatitis=inflammation of skin Stomatitis=inflammation of mucosa Vegetans=proliferative growths
27 A Brief Primer on Neutrophilic Dermatoses Sweet syndrome Pyoderma gangrenosum To name a few. Pustular psoriasis Drug-induced/acute generalized exanthematous pustulosis Keratoderma blennorrhagicum Sheddon-Wilkinson disease IgA pemphigus Amicrobial pustulosis of the hands Infantile acropustulosis Neutrophilic dermatosis of the dorsal hands DIRA syndrome Behcet s disease
28 What happened to our patient? Prednisone was increased to 1 mg/kg (prednisone 70 mg) Denied any symptoms of inflammatory bowel disease Fecal calprotectin was positive Referred to gastroenterology?
29
30 Lessons to take away Puzzle and puzzle, till your puzzler is sore If you don t have enough of the right treatment, it doesn t work If there s neutrophilic inflammation without infection, think neutrophilic dermatoses PD-PSV involves skin and mucosa and is highly associated with IBD
31 Thank You to Our patient, who graciously gave permission for his story and pictures to be shared. Our consulting teams: dermatology, ophthalmology, rheumatology, oral maxillofacial surgery, plastic surgery, and infectious diseases. Everyone who contributed to the care of the patient during his hospitalizations and follow-up.
32 References Clark, L.G. et al., Pyostomatitis vegetans (PSV)-pyodermatitis vegetans (PDV): A clinicopathologic study of 7 cases at a tertiary referral center. J Am Acad Dematol. 2016;75(3): doi: /j.jaad Hallopeau H., Pyodermite végétante, Arch. f. Dermat. 1898; 43: 289. Kim, T. H. And Kim S. Ann Dermatol. 2015; 27(5): Moschella S. L. And Daivs, M. DP, (2012) Neutrophilic Dermatosis. Bolognia, J.L., Jorizzo, J.L., Schaffer, J.V.. Dermatology ( ). Online: Elsevier Limited. Storwick, G.S. et al., Pyodermatitis-pyostomatitis vegetans: A specific marker for inflammatory bowel disease. J Am Acad Dermatol. 1994; 21: Rosmaninho, A. Carvalho, S. Neurophilic Dermatoses Revisited. EMJ Dermatol. 2014;2:77-85.
33 References for Outside Images Dr. Seuss letter to the children of Troy, MI. Sweet Syndrome (Acute Febrile Neutrophilic Dermatosis) Associated With Pulmonary Coccidioidomycosis. Arch Dermatol. 2005;141(7): Neutrophilic Dermatosis (Pustular Vasculitis) of the Dorsal Hands A Report of 7 Cases and Review of the Literature. Arch Dermatol. 2002;138(3): Pyoderma gangrenosum. Mayo Clinic. Accessed 10/23/17.
34 Clip Art References Fotosearch.com, image k ole-christmas-screencap Illustrationsof.com, #
35 Another Case of PD-PSV
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