A 55-year-old Hispanic male presented to the hospital at the request of his primary care provider due to a two-month history of joint pain in the shoulders, elbows, wrists, and knees, decreased appetite and 20-pound weight loss in the setting of hypercalcemia, anemia, elevated serum creatinine, and positive urine Bence-Jones protein. He also endorsed a severely pruritic rash, chills, and fatigue that began three weeks prior to admission, but denied fever, headache, alopecia, chest pain, cough, diaphoresis, dyspnea, abdominal pain, nausea, vomiting, or diarrhea. Travel history was significant for a trip to Mexico 3 months prior to admission. His occupation is in landscaping.
Physical exam was significant for diffuse joint tenderness without swelling or warmth, and multiple raised, blanchable, erythematous papules and plaques distributed on the back, arms, legs, and face, as well as leonine facies (Figures 1 and 2). Labs were significant for a serum white blood cell count (WBC) of 3,700/microL (normal 4,500-1100/microL), absolute lymphocytes 400/microL (1,000-4,000/microL), hemoglobin of 8.6 g/dL (normal 14.0-17.5 g/dL), corrected calcium of 14.4 mg/dL (normal 8.6-10.3 mg/dL), 1,25-dihydroxyvitamin D of 154 pg/mL (normal 18-72 pg/mL), 25-hydroxyvitamin D of 20 ng/mL (normal 30-100 ng/mL), PTH of 1 pg/mL (normal 14-64 pg/mL), PTH-related protein of 24 pg/mL (normal 14-27 pg/mL), creatinine of 3.23 mg/dL (unknown baseline, normal 1.13 and 0.93 mg/dL), and 24-h urine protein of 700 mg (normal < 100 mg).
Due to positive Bence-Jones protein on urinalysis from an outside laboratory, the diagnosis of gammopathy was entertained. However, an in-house evaluation for monoclonal gammopathy, including serum and urine protein electrophoresis and immunofixation, skeletal survey, and bone marrow biopsy, was negative for gammopathy as well as infection and malignancy. Interestingly, skin punch biopsy revealed non-caseating granulomatous dermatitis, with negative stains for atypical mycobacteria, tuberculosis and fungal organisms (Figure 3). CT chest showed non-specific calcified granulomas at the hilum and left lung without hilar adenopathy or infiltrates. Renal biopsy demonstrated mild arterial nephrocalcinosis and segmental thickening of the glomerular basement membrane. Tissue cultures, serum Quantiferon TB gold, coccidioides antibodies, histoplasma antibodies, and blastomyces antibodies were all negative. Serum Angiotensin Converting Enzyme (ACE) level was normal. Screening electrocardiogram and transthoracic echocardiogram did not demonstrate any evidence of cardiac involvement. By exclusion of alternative diagnoses, the patient was treated for sarcoidosis with prednisone 20 mg daily, yielding substantial improvements in the rash, and arthralgia. The hypercalcemia resolved with steroids, fluid resuscitation, calcitonin, and pamidronate which also led to the improvement of renal function (Figure 4). Two weeks after discharge, he was started on azathioprine 50 mg daily with subsequent prednisone taper. As an outpatient, the patient was doing well with resolution of his rash, and azathioprine was up-titrated to 100 mg daily. The patient was subsequently lost to follow-up.