Calciphylaxis: An important imitator of cutaneous vasculitis

Abstract

Case Report A 42-year-old man was transferred to our institution for management of severe ulcerations of the lower extremities thought to be secondary to vasculitis. Three months previously, the patient was hit on the left shin by a soccer ball. The next day, his left pretibial region became erythematous and swollen. He was given cephalexin for presumed cellulitis, and the erythema improved but the swelling did not. Within a few days, the erythema returned and the swelling extended to his calf. Small, tight bullae formed over the affected skin. His right leg also began to swell and blister. The patient developed generalized edema and decreased urine output. He was admitted to an outside hospital. The patient had hypertension and a baseline creatinine level of 2 mg/dl. Medications included amlodipine, furosemide, and acetaminophen/propoxyphene napsylate. There were no known drug allergies. Social and family histories were noncontributory. Upon admission, the patient was afebrile and vital signs were normal except for a blood pressure of 150/90 mm Hg. The examination was remarkable for erythema of the lower extremities with 3 pitting edema and mild flexion contractures of the knees. The skin below the knees was covered with scattered bullae, indurated violaceous patches, and ulcers. Initial laboratory results are shown in Table 1. A portable chest radiograph showed cardiomegaly with clear lung fields. Renal ultrasound revealed a right renal cyst, increased cortical echogenicity, and diminished renal cortices. Cephalexin was continued, and dialysis was initiated. Results of blood and urine cultures were negative. Cultures of the wounds grew mixed bowel and skin flora. Erythrocyte sedimentation rate was 85 mm/hour. Hepatitis B and C serologies, antinuclear antibody, antineutrophil cytoplasmic antibodies, and cryoglobulins were negative. Serum protein electrophoresis was normal. Intact parathyroid hormone (PTH) was increased at 196.5 pg/ml (normal range 10–55 pg/ml). There was no evidence of venous thromboembolism on duplex ultrasonography of the lower extremities or by a ventilation/perfusion scan. Transthoracic echocardiogram showed no evidence of endocarditis, atrial myxoma, or atheroembolic disease. Despite ciprofloxacin and ceftazidime, whirlpool treatments, and wound debridement, numerous ulcers with black eschar and indurated, erythematous margins developed below the knees. New violaceous and indurated lesions progressed proximally to involve the thighs followed by the lower abdomen. Punch biopsy samples of the left and right thigh were reported as necrotizing vasculitis. Treatment with methylprednisolone 60 mg intravenously every 6 hours was begun. The histopathologic findings, however, also included focal fat necrosis and a patent blood vessel with mural calcification, features suggesting calciphylaxis. A repeat punch biopsy sample of the thigh obtained 1 week later for immunofluorescent studies showed vascular and perivascular fibrin deposits that were suggestive of atrophie blanche from stasis dermatitis or livedo vasculitis. Mycophenolate mofetil was initiated and subsequently discontinued days later, when blood cultures grew Pseudomonas and Enterobacter. Computed tomography of the chest, abdomen, and pelvis revealed no masses or lymphadenopathy. Rheumatoid factor, anticardiolipin antibodies, lupus anticoagulant, and anti–double-stranded DNA antibody were repeatedly negative. Serum C3 and C4 were normal. Three months after the initial admission the patient was transferred to our institution for further treatment. He was stable and afebrile but in severe pain. The physical examination revealed extensive skin involvement (Figure 1). A necrotic lesion was present over the left lower abdomen Supported by the South Jersey Lupus Foundation. Dana Jacobs-Kosmin, MD: Albert Einstein Medical Center, Philadelphia, Pennsylvania; Raphael J. DeHoratius, MD: Thomas Jefferson University, Philadelphia, Pennsylvania. Address correspondence to Dana Jacobs-Kosmin, MD, Einstein Arthritis Center, 5501 Old York Road, Korman 103, Philadelphia, PA 19141. E-mail: jacobkd@einstein.edu. Submitted for publication May 10, 2006; accepted in revised form August 31, 2006. Arthritis & Rheumatism (Arthritis Care & Research) Vol. 57, No. 3, April 15, 2007, pp 533–537 DOI 10.1002/art.22616 © 2007, American College of Rheumatology