| Vasculitis Mimicry in Patient with Recurrent Cellulitis, Nephrotic Syndrome and Elevated ESR | ||
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CLINICAL VIGNETTE Vasculitis Mimicry in Patient with Recurrent Cellulitis, Nephrotic Syndrome and Elevated ESRRania Shammas, M.D. and Mihaela Taylor, M.D. Case ReportThe patient is a 77-year-old female with past medical history of heavy tobacco use, hypertension, hyperlipidemia, cerebrovascular accident without residual, chronic renal insufficiency with nephrotic range proteinuria and resistant lower extremity cellulitis who was readmitted to the hospital for worsening pain and erythema of the lower extremities. This was the patient's sixth hospitalization for cellulitis. The patient has been dealing with chronic swelling, redness, and pain in her lower extremities for about a year and a half. She had a stroke in November 2006 followed by seizure with a fall and fracture of her left femur in 3 places requiring repair with titanium rod. Following these events, the patient reported the occurrence of the swelling in the lower extremities in January 2007. The patient remembers that initially the legs were extremely swollen with blistering and heavy scaling. The patient was placed on diuretics and repeated courses of antibiotics with minimal benefit. Two weeks prior to this hospitalization, the patient was treated for cellulitis with intravenous antibiotics, again without significant improvement. Because of resistant hypertension and chronic renal insufficiency, a magnetic resonance angiogram of the renal arteries was performed which showed significant vascular disease. There was high-grade focal stenosis of the right common iliac artery, moderate stenosis of the left common iliac artery with focal dissections of bilateral common iliac arteries and focal ulceration or ectasia of the mid left external iliac artery with mild proximal stenosis. This study also showed significant stenosis in the right hepatic artery, the celiac trunk and inferior mesenteric artery at its origin. She also had high-grade stenosis of the proximal left renal artery. The patient had an erythrocyte sedimentation rate (ESR) of 30. Work-up for osteomyelitis was negative with a normal x-ray and bone scan. The question of an underlying vasculitis causing an elevated sedimentation rate and recurrent cellulitis was then raised and a rheumatology consult was obtained. The patient reported general fatigue but denied weight loss, fevers or night sweats. The patient denied a history of chronic sinusitis, ear infection, or mastoiditis that would be suggestive for Wegener's, microscopic polyarteritis or Churg-Strauss vasculitis. She has a nasal septal defect, which may be related to heavy use of cocaine in her 20s. She also admitted to smoking 4 packs a day since age 14 years up to age 72 years. She denied headaches or skull tenderness indicative of giant-cell arteritis. She reported vision problems related to multiple cataract surgeries and dry eyes. The patient has chronic lower back pain from previous trauma but no peripheral joint swelling, redness, or history of arthritis in the small joints of the hands and feet. She has not experienced photosensitive skin rashes or mouth sores. She has had no miscarriages or episodes of thrombosis. The patient's family history is unremarkable for connective tissue disorder. Physical examination was remarkable for diffuse erythema below the knees, scaling and atrophy of the skin without hair follicles, and edema left side greater than right. Livedo reticularis was noted on her hands and feet. She had 1+ peripheral pulses. Peripheral joints had no tenderness or inflammation. Her laboratory data was significant for an ESR between 90 and 130, C-reactive protein level of 7.7, normal serum protein electrophoresis and urine protein electrophoresis, normal complements, negative antinuclear antibody, dsDNA, rheumatoid factor, Smith, ribonucleic protein and anti-neutrophil cytoplasmic antibody. She had a mild normocytic anemia with a normal white count and an eosinophil count elevated at 16%. She had a low albumin with nephrotic range proteinuria and a baseline creatinine of 1.6. A lower extremity duplex was negative for thrombosis. DiscussionGiven the patient's history of diffuse atherosclerosis related vasculopathy, livedo reticularis, and peripheral eosinophilia, it was determined that cholesterol emboli was a likely etiology of the patient's chronic lower extremity dermatitis and the chonic renal insufficiency with nephrotic range proteinuria. The chronology of events in the history (i.e. the rash and nephrotic syndrome starting after the embolic stroke and orthopedic surgery) supports the diagnosis. Cholesterol crystal embolization occurs when portions of plaque break off and embolize distally, resulting in partial or total occlusion of small arteries. This leads to tissue or organ ischemia. The embolization may be precipitated spontaneously or iatrogenically by vascular procedures. The clinical manifestations of atheroembolic disease may mimic systemic disorders involving organs such as the skin, kidneys, heart, brain, eye and gastrointestinal system as portions of plaques can break off and embolize to any or all of these organ systems. The symptoms, therefore, can be variable. The elevated eosinophil levels may point to a vasculitis such as Churg-Strauss, but she does not have any history of asthma or peripheral nervous involvement that would be common with this vasculitis. It is known that atheroembolic disease is associated with acute or chronic renal failure, especially after an inciting event. Nephrotic syndrome is a less common finding; however, there have been case reports linking atheroembolic disease with nephrotic range proteinuria. It has even been suggested that atheroembolic disease be considered in the differential diagnosis of nephrotic syndrome in elderly patients with serious vascular disease.1 The ESR is also elevated in almost all patients with the nephrotic syndrome or end-stage renal disease. Twenty percent may have elevations as high as 100, a level usually associated with disorders such as infection, cancer, or collagen vascular diseases.2 A direct relation between the degree of proteinuria and the ESR has been noted in which the ESR was approximately 10 times the daily rate of protein excretion.3 Thus, this patient's persistently high elevated ESR may have been a combination of her atheroembolic disease and likely associated nephrotic syndrome. The patient was treated with antibiotics, her blood pressure regimen was optimized, her statin maximized and she was started on aspirin 325 mg daily. In summary, atheroembolic disease can mimic systemic disorders such as vasculitis leading to recurrent skin manifestations masquerading as cellulitis, renal insufficiency including nephrotic syndrome and an elevated sedimentation rate. REFERENCES
Submitted on September 29, 2008 |
© copyright 2009 Anonymous & UCLA Department of Medicine