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
 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
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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
79 cases Daoud, Hutton,
and Gibson
 1 F/ M
 1.7 F/M
 Age: Variable onset
 Age: Variable onset
M. S Daoud et al, 1997
Cutaneous PAN
Systemic PAN
Normal BP
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 negative
Hep B and C association
Immunological testing equivocal
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
Ulcerative 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
Sensory disturbances
Elevated ESR (60%)
Elevated ESR (59%)
Negative Hep B and Hep C
Negative Hep B and Hep C
Steroids symptomatically effective
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.