Table I.
Patient | Medical history | Presentation, Tx, and outcome |
Laboratory results | Urine toxicology | Skin biopsy specimen results |
---|---|---|---|---|---|
A 46 y Caucasian Female |
Substance abuse | 1-mo History of purpura and necrosis on bilateral ears, cheeks, and upper and lower extremities Tx: steroids Initial improvement, lost to f/u |
Neutropenia: no P-ANCA >2500 --Positive MPO and PR3 Anticardiolipin IgM positive ANA, hepatitis, HIV negative Cryoglobulins negative |
Not performed on admission, but previously positive for cocaine and THC |
Small vessel vasculitis with intravascular thrombi |
B 57 y African American Female |
Substance abuse, depression, anxiety, hypertension, and recurrent Staphylococcus aureus skin infections |
1-wk History of purpura and necrosis of bilateral ears and cheeks Tx: IV antibiotics, gradual improvement; neutropenia treated with filgrastim Relapse 6 mo later with same presentation, laboratory results, and positive cocaine urine test result |
Neutropenia: yes P-ANCA >2500 --Positive MPO and PR3 Anticardiolipin IgM positive ANA, SSA, SSB, dsDNA, C3, C4 negative Hepatitis, HIV negative Cryoglobulins negative |
Positive for cocaine | Intravascular thrombi |
C 46 y Hispanic Female |
Substance abuse, hepatitis C, hypothyroidism |
Bilateral ear necrosis, diffuse retiform purpura with necrosis on neck, trunk, and extremities Tx: IV methylprednisolone Gradual improvement initially, lost to f/u |
Neutropenia: yes P-ANCA 640, C-ANCA 1280 Anticardiolipin IgM positive ANA, SSA, SSB, dsDNA, C3, C4 negative Cryoglobulins negative |
Positive for cocaine and opiates |
Multiple intravascular thrombi |
D 22 y Hispanic Female |
Substance abuse | 3-wk History of purpura and necrosis of bilateral ears, cheeks, nasal tip, buttocks, and thighs Tx: IV methylprednisolone Skin and neutropenia improved rapidly |
Neutropenia: yes P-ANCA 2560 --Negative MPO and PR3 Anticardiolipin IgM positive ANA, SSA, SSB, dsDNA negative HIV, hepatitis negative Cryoglobulins negative |
Positive for cocaine and opiates |
Small vessel vasculitis with multiple intravascular thrombi |
E 37 y Hispanic Male |
Substance abuse | Tender bilateral ear purpura and necrosis, diffuse retiform purpura on trunk and extremities Tx: supportive Rapid improvement of skin |
Neutropenia: no P-ANCA 640 CRP 16.2 mg/L, ESR 41 mm/h, D-dimer elevated at 3260, LDH 390 IU/L, PTT 37.7 sec, INR 1.3 ANA 1:40 HIV negative |
Positive for cocaine | Leukocytoclastic vasculitis IF: vascular deposits of IgM, with weak staining for C3, IgG, and IgA;vascular and perivascular staining for fibrin |
F 50 y African American Male |
Substance abuse | Altered mental status;3-wk history of tender purpura and necrosis of bilateral ears; purpura and bullae over trunk and extremities Tx: antibiotics Mental status and skin improved rapidly |
Neutropenia: no P-ANCA 640 CRP 5.67 mg/L, ESR 32 mm/hr, D-dimer elevated at 548, PTT 39.6 sec ANA 1:40 HIV negative |
Positive for cocaine | Leukocytoclastic vasculitis and focal panniculitis IF: vascular deposits of IgM, IgA, C3, and weakly IgG; interstitial and vascular positive staining for fibrin |
ANA, Antinuclear antibody; C-ANCA, cytoplasmic antineutrophil cytoplasmic antibody; CRP, c-reactive protein; dsDNA, double-stranded DNA; ESR, erythrocyte sedimentation rate; f/u, follow-up; IF, immunofluorescence; INR, international normalized ratio; IV, intravenous; LDH, lactate dehydrogenase; MPO, antimyeloperoxidase antibodies; P-ANCA, perinuclear antineutrophil cytoplasmic antibody; PR3, anti-proteinase-3 antibodies; PTT, partial thromboplastin time; SSA, Sjogren Syndrome A (anti-Ro); SSB, Sjogren Syndrome B (anti-La); THC, tetrahydrocannabinol (marijuana component); Tx, treatment.