KERRIE TIDWELL – MS 3 RED MR

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

Cocaine-Induced Pseudovasculitis

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]

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]

What is the diagnosis? Case Report 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 [Khoo & Ng, 1998]

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

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 Epidemiology

M. S Daoud et al, 1997 Cutaneous PANSystemic PAN Normal BPElevated BP Leukocytosis normal to moderateSevere leukocytosis Small and medium arteries and arterioles Localized involvementMulti-organ involvement Hep B and C negativeHep B and C association Immunological testing equivocalSmall ANCA association Chronic, relapsing, benign diseaseFatal in 2 years without Rx [Khoo & Ng, 1998]

Study by M.S. Daoud et al, 1997 Non-ulcerative cutaneous PANUlcerative cutaneous PAN Patients found to have indurated plaques on lower extremities Painful ulcerations in legs Edema, swelling of lower extremities (60%) Edema (54%) Low grade fever, arthralgias, myalgias, malasie, and lethargy (25%) Low grade fever, fatigue, arthralgias, myalgias (< 20%) Sensory disturbances Elevated ESR (60%)Elevated ESR (59%) Negative Hep B and Hep C Steroids symptomatically effective

Cutaneous PAN [Brandt, HRC, 2009]

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

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]

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

References Bhinder S and Majithia V. Cocaine use and its rheumatic manifestations: a case report and disccusion. Clin Rheumatol (2007) 26: Brandt HRC, Arnone M, Valente NYS, Sotto MN, Criado PR. An Bras Dermatol. 2009;84(1): Brewer J, Meves A, Bostwick M, Hamacher K and Pittelkow M. Cocaine abuse : Dermatologic manifestations and therapeutic approaches. J Am Acad Dermatol 2008; 59(3): Carlson J and Chen K. Cutaneous Pseudovasculitis. Am J Dermatopathol 2007; 29: Daoud M, Hutton K, and Gibson L. Cutaneous periarteritis nodosa: a clinicopathological study of 70 cases. British Journal of Dermatoloty 1997; 136: Diaz-Perez J, Lagran Z, Diaz-Ramon J, Winkelmann R. Cutaneous Polyarteritis Nodosa. Semin Cutan Med Surg 2007; 36:77-88 Fiorentino D. Cutaneous vasculitis. J Am Acod Dermatol 2003; 48: Friedman D and Wolfsthal S. Concin-Induced Pseudovasculitis. Mayo Clin Proc. 2005; 80(5): Khoo BP, Ng SK, Cutaneous Polyarteritis Nodosa: A Case Report and Literature Review. Ann Acad Med Singapore 1998; 27: Ribi C, Cohen P, Pagnoux C, et al. Treatment of polyangitis nodosa and microscopic polyangiitis without poor prognosis factors: A prospective randomized study of one hundred twenty-four patients. Arthritis Rheum 2010; 62:1186.