Frostbite and Cold Agglutinin Disease: Coexistence of Two Entities Leading to Poor Clinical Outcomes.

An 83-year-old woman was admitted to the emergency department for a 7-day history of fatigue and progressive cyanosis in the feet and hands after cold exposure despite physical protective measures. Upon arrival, the patient presented with necrotic cutaneous lesions in both hands and distal lower extremities. Upon admission, hemoglobin was 7.6 g/dL and laboratory tests were consistent with cold agglutinin disease (CAD), the presence of monoclonal IgM, and flow cytometry consistent with lymphoplasmacytic lymphoma, but MYD88 L265P mutation was negative. The patient required blood transfusion, resulting in stabilized hemoglobin and a decrease in markers of hemolysis. Treatment with aspirin 250 mg daily and intravenous iloprost 0.5 mL/h was initiated with a poor clinical response at day 4. Amputation was required. Plasma exchange was performed and chemotherapy with rituximab and bendamustine was initiated. The clinical course was marked by further necrosis, prompting discussions regarding an additional amputation that was not performed considering the high surgical risk and refusal by the patient. Supportive treatment was initiated, and the patient expired one month after hospital admission.

Polymyalgia Rheumatica During the Course of Limited Cutaneous Scleroderma: An Unusual and Challenging Coexistence.

A 50-year-old patient with a history of limited cutaneous scleroderma began with polyarthralgia (left shoulder, elbows and hips) without stiffness or associated inflammatory syndrome. Treatment with oral anti-inflammatory drugs was started on suspicion of peripheral spondyloarthritis with partial response. This progressed with the appearance of stiffness and functional limitation of the hips as well as an increase in the inflammatory syndrome two weeks after onset. It was decided to perform an 18F-FDG-PET scan compatible with polymyalgia rheumatica. The patient was treated with oral corticosteroids with an excellent response after one week of treatment. LEARNING POINTS: Polymyalgia rheumatica should be considered, even in young adults, with atypical clinical presentation.Post-infectious and paraneoplastic inflammatory rheumatism should be ruled out before considering the diagnosis of polymyalgia rheumatica.18F-FDG-PET plays an important role in the positive diagnosis of PMR and in the differential diagnosis.