Presenter: Betsy Leveritt, D.O.
Dermatology Program: Wellington Regional Medical Center
Program Director: Brad Glick, D.O.
Submitted on: Aug 14, 2010
CHIEF COMPLAINT: A 52 year old Caucasian male with a past medical history of liver cirrhosis, subsequent hepatorenal syndrome, and end stage renal disease (ESRD) on hemodialysis (HD), presented with a several month history of numerous painful, erythematous-violaceous plaques across the abdomen and bilateral thighs and upper extremities, accompanied by necrotic erosions of the lower extremities.
Signs and symptoms: A review of systems revealed low grade fevers with occasional chills, night sweats, and a significant weight loss of at least 13.6 kg over the preceding 3-4 months.
Previous Treatment: During a prior hospital admission, the patient manifested these same skin lesions, and was given a 3 month course of intravenous antibiotics for a biopsy proven Actinomyces infection. He also recalled being given Heparin during that same hospital stay.
Physical examination revealed a cachetic appearing male. Erythematous-violaceous, annular and reticulated, infiltrative appearing plaques were noted across the abdomen, inner thighs, and upper extremities (Figure 1). 12-16 cm erosions with necrotic centers, jagged edges, and pink, slightly rolled borders with a lack of undermining were also seen on both lower extremities (Figure 2). The pedal and popliteal pulses were 1-2+/4 bilaterally.
Pertinent laboratory studies revealed an elevated blood urea nitrogen (BUN) 45 mg/dL and creatinine 4.3 mg/dL, with a normal calcium 8.8 mg/dL, ionized calcium 1.3 mmol/L, and parathyroid hormone intact (PTHI) 15.6 pg/mL. Phosphorous was slightly elevated at 5.3 mg/dL. A calcium-phosphorous product was normal at 46.64 mg/dL. Albumin was decreased at 1.1 mg/dL, and alkaline phosphatase was elevated at 286 units/L. A heparin pf4 antibody was critically high. Furthermore, the lupus antiphospholipid antibody was positive at greater than 8 seconds, accompanied by an elevated APTT 1.5 hour at 46 seconds and an APTT 1:1 saline at 57 seconds. LA 1 (DRVVT) and LA2 (DRVVT) were normal. The remainder of the lupus anticoagulant panel was also negative. IgG was elevated at 1730 mg/dL, with a normal IgM level. A decrease in protein C antigen at 43% was also noted. However, protein S, both free and total, were normal at 76% and 69%, respectively. ANA, rheumatoid factor, as well as cryoglobulins were all negative. The patient was also anemic, with a hemoglobin (Hgb) 10.5 g/dL and hematocrit (HCT) 32.3%, as well as thrombocytopenic with a platelet (PLT) count of 107 K/mcl. HIV, Hepatitis B, and Hepatitis C serologies were negative. Tissue culture and smears for aerobic and anaerobic bacteria, fungi, and acid fast bacilli (AFB) were also negative. Paracenteses were negative for infection and malignancy.
Histopathology revealed intravascular calcification of small and medium sized blood vessels associated with focal thrombosis, with adjacent secondary fibrosis and reactive neutrophilic infiltrate (Figure 3). A Von Kossa stain also showed intravascular calcium deposition. No primary vasculitis was evident. PAS and Fite stains were negative for micro-organisms. Direct immunofluorescence was also negative for vascular deposition of immunoreactants.
1. Medium vessel vasculitis ( i.e. polyarteritis nodosa)
2. Inflammatory/ connective tissue disorder (nephrogenic fibrosing dermopathy , lupus panniculitis, pyoderma gangrenosum, and erythema nodosum)
3. Infectious etiology (cellulitis, septic emboli, ecthyma gangrenosum, necrotizing fasciitis, mucormycosis, aspergillosis)
4. Hypercoaguable-occlusive states (i.e. underlying malignancy, antiphospholipid antibody syndrome, protein C or S deficiency, factor V Leiden deficiency hyperhomocysteinemia)
5. Heparin or warfarin-induced skin necrosis