RED MR

K E R R I E T I D W E L L – M S 3

What is the diagnosis?

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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 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 [Khoo & Ng, 1998]

Cutaneous Periarteritis Nodosa

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

Epidemiology

33 cases Diaz-Peres and Winkelmann

  1 F/ M Age: Variable onset

79 cases Daoud, Hutton, and Gibson

  1.7 F/M Age: Variable onset

M. S Daoud et al, 1997

Cutaneous PAN

Normal BP Hep B and C negative Immunological testing equivocal

Systemic PAN

Elevated BP Leukocytosis normal to moderate Severe leukocytosis Small and medium arteries and arterioles Small and medium arteries and arterioles Localized involvement Multi-organ involvement Hep B and C association Small ANCA association Chronic, relapsing, benign disease Fatal in 2 years without Rx [Khoo & Ng, 1998]

Study by M.S. Daoud et al, 1997

Non-ulcerative cutaneous PAN

Patients found to have indurated plaques on lower extremities Edema, swelling of lower extremities (60%) Low grade fever, arthralgias, myalgias, malasie, and lethargy (25%) Sensory disturbances Elevated ESR (60%) Negative Hep B and Hep C Steroids symptomatically effective

Ulcerative cutaneous PAN

Painful ulcerations in legs Edema (54%) Low grade fever, fatigue, arthralgias, myalgias (< 20%) Sensory disturbances 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

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Bhinder S and Majithia V. Cocaine use and its rheumatic manifestations: a case report and disccusion. Clin Rheumatol (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 Acad Dermatol 2008; 59(3): 483-487 Carlson J and Chen K. Cutaneous Pseudovasculitis. Am J Dermatopathol 2007; 29: 44-55 Daoud M, Hutton K, and Gibson L. Cutaneous periarteritis nodosa: a clinicopathological study of 70 cases. British Journal of Dermatoloty 1997; 136: 706-713 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: 311-331 Friedman D and Wolfsthal S. Concin-Induced Pseudovasculitis. Mayo Clin Proc. 2005; 80(5): 671-673 Khoo BP, Ng SK, Cutaneous Polyarteritis Nodosa: A Case Report and Literature Review. Ann Acad Med Singapore 1998; 27: 868-72 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.