[Sepsis with acral necrosis in a patient with Sharp syndrome - case 8/2010]

We report on a patient with Sharp-Syndrome who was referred to our emergency department with sepsis. In addition, the patient showed acral necrosis, particularly of the distal phalanges of the hands and of the tip of the nose.

Laboratory analyses showed an elevation of the inflammatory parameters (C-reactive protein elevation, leukocytosis). Furthermore, procalcitonin and the D-dimers were increased, antithrombin III, however, was decreased. The thoracic/abdominal computed tomography (CT) showed bilateral inferior lobe pneumonia with concomitant pleural effusions. As a secondary diagnostic finding the thoracic/abdominal CT and the abdominal ultrasound showed a markedly reduced size of the spleen. Finally, bacteria (Streptococcus pneumoniae) were found in the blood of the patient.

We diagnosed pneumococcal sepsis with disseminated intravasal coagulation and acral necrosis caused by pneumonia. The immune status was impaired due to immunosuppressive therapy (prednisolon and azathioprin) and functional asplenism. The patient was stabilized with antibiotic treatment, hydration, and drotrecogin (protein C). Transiently catecholamin treatment and oxygen substitution were necessary. Alprostadil was used to treat acral circulatory disorder.

This case report shows the importance of consequent screening for organ manifestations in autoimmune diseases. In particular, this case report highlights the relevance of prophylactic vaccination in patients with autoimmune diseases, primarily those with autosplenectomy. Furthermore, this article gives a short overview about the pathogenesis, the diagnostic criteria of the Sharp-syndrome. The frequencies of organ involvement and the treatment options are also discussed.