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RED MR

RED MR. Kerrie Tidwell – MS 3. What is the diagnosis?. Case Report 1 35 yo AAF c/o new rashes on extremities PE: Diffuse palpable purpura in reticular pattern on bilateral lower ext, buttocks, and arms Labs: Elevated LFTs

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RED MR

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  1. RED MR Kerrie Tidwell – MS 3

  2. What is the diagnosis? • Case Report 1 • 35 yo AAF c/o new rashes on extremities • PE: Diffuse palpable purpura in reticular pattern on bilateral lower ext, buttocks, and arms • Labs: • Elevated LFTs • Neg  ANA, ANCAs, antiphospholipid Ab, lups anticoag, cryoglobulins, C3/C4, hepatitis panel, HIV Ab and hypercoag panel • Biopsy: Fibrin thrombi occluding vessels, extensive hemorrhage • Outcome: Improved on oral prednisone

  3. Cocaine-Induced Pseudovasculitis

  4. Pseudovasculitis • Disorders that mimic vasculitis by not revealing the expected diagnostic histopathologic findings. • Consider when vasculitis is not supported or data is inconsistent [Friedman, 2005]

  5. Cocaine- Induced Pseudovasculitis • Characteristics • Biopsy: No granulomas or leukocytoclasia • Found in Wegner’s • Labs: inconsistent ANCA and target Ab pattern • Localized disease, NOT systemic • Treatment: Abstaining from cocaine use is best [Bhinder S, 2007 and Friedman D, 2005]

  6. Case Report 2 51 yo chinese man presented with erythematous erysipeloid-like plaque on lower extremity Treated for bacterial infection with antibiotics Treated with Prednisolone after negative cultures Presented with plaques and nodules over BLE and thighs 1 yr later. No other symptoms. Biopsy: Fibrinoid necrosis of medium-size artery with neutrophilic infiltrate. LFTs, CK, aldolase, ANA, ANCA, Hep panels, CXR, and EKG normal Relief of symptoms with Prednisolone What is the diagnosis? [Khoo & Ng, 1998]

  7. Cutaneous Periarteritis Nodosa

  8. Cutaneous Periarteritis Nodosa • Benign, chronic, relapsing course • NO systemic involvement, mostly localized • Primary lesion • Painful subcutaneous nodules in lower extremities • Peripheral neuropathy • Numbness, burning and rarely foot drop • Medium size vessels in deep dermis and panniculus • Not associated with Hep B or C • Favorable prognosis factor • Rare involvement with c-ANCA or p-ANCA

  9. Epidemiology 33 cases Diaz-Peres and Winkelmann 79 cases Daoud, Hutton, and Gibson • 1 F/ M • Age: Variable onset • 1.7 F/M • Age: Variable onset

  10. M. S Daoud et al, 1997 [Khoo & Ng, 1998]

  11. Study by M.S. Daoud et al, 1997

  12. Cutaneous PAN [Brandt, HRC, 2009]

  13. Histopathology of Cutaneous PAN • Medium sized vessels • Inflammatory changes in deep dermis • Necrotizing leukocytoclasticvasulitis of capillaries • Superficial dermis • Microscopic changes do not correlate with severity of disease [Diaz-Perez, 2007 and Daoud, 1997]

  14. Treatment • Prednisone • Initial: 1mg/kg/d with max 60 to 80 mg/d • Long term: • Continue high dose for 4 weeks or significant improvement • Taper • 5 to 10 mg every 7 days till 20 mg/day is reached • 1 mg/day every 7 days till finished • Total: 9 months • Reduction in prednisone dose • Associated with flare of disease [ Ribi, 2010; Daoud, 1997]

  15. Summary • Cocaine-Induced pseudovasculitis • Consider when biopsy and lab data are inconsistent • High level of suspicion in cocaine users • Cutaneous PAN • Consider when: • Medium-vessel vasculitis in deep dermis • Localized normally to lower extremities • Labs are normal or negative • Improves with Prednisone

  16. References Bhinder S and Majithia V. Cocaine use and its rheumatic manifestations: a case report and disccusion. ClinRheumatol (2007) 26: 1192-1194 Brandt HRC, Arnone M, Valente NYS, Sotto MN, Criado PR. An Bras Dermatol. 2009;84(1):57-67. Brewer J, Meves A, Bostwick M, Hamacher K and Pittelkow M. Cocaine abuse : Dermatologic manifestations and therapeutic approaches. J Am AcadDermatol 2008; 59(3): 483-487 Carlson J and Chen K. CutaneousPseudovasculitis. Am J Dermatopathol 2007; 29: 44-55 Daoud M, Hutton K, and Gibson L. Cutaneousperiarteritisnodosa: a clinicopathological study of 70 cases. British Journal of Dermatoloty 1997; 136: 706-713 Diaz-Perez J, Lagran Z, Diaz-Ramon J, Winkelmann R. CutaneousPolyarteritisNodosa. SeminCutan Med Surg 2007; 36:77-88 Fiorentino D. Cutaneousvasculitis. J Am AcodDermatol 2003; 48: 311-331 Friedman D and Wolfsthal S. Concin-Induced Pseudovasculitis. Mayo Clin Proc. 2005; 80(5): 671-673 Khoo BP, Ng SK, CutaneousPolyarteritisNodosa: A Case Report and Literature Review. Ann Acad Med Singapore 1998; 27: 868-72 Ribi C, Cohen P, Pagnoux C, et al. Treatment of polyangitisnodosa and microscopic polyangiitis without poor prognosis factors: A prospective randomized study of one hundred twenty-four patients. Arthritis Rheum 2010; 62:1186.

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