Neutrophilic urticarial dermatosis: a novel association with poststreptococcal rheumatic disease.

Neutrophilic urticarial dermatosis (NUD), a particular clinical and histological entity, can provide a strong pointer to underlying systemic disease, most frequently rheumatological diseases. We report the first case of NUD in association with a post-streptococcal rheumatic disease, with symptoms including recurrent sore throat, raised antistreptolysin O titre, persistent transient urticaria, polyarthralgia, rheumatic mitral valve disease and Jaccoud arthropathy. Histologically, NUD is characterized by an intense superficial and deep neutrophilic interstitial and perivascular infiltrate, without significant oedema or blood vessel damage. These neutrophils may have a tendency to concentrate along the basement membrane and extend into the epidermis, hair follicles, sebaceous glands and sweat glands (a feature termed 'neutrophilic epitheliotropism'). Clinicians should remain cognizant of NUD, and in particular its frequent association with an underlying inflammatory disorder.

Successful surgical repair of acute type A aortic dissection without the use of blood products.

We report successful surgical treatment of type A aortic dissection in a Jehovah's Witness without the use of any blood products. An interposition graft replacement of the ascending aorta was carried out. This was under right axillo-atrial cardiopulmonary bypass with antegrade cerebral perfusion via right a subclavian and left carotid cannula for 24 minutes at 28°C. Body temperature was kept at 32°C throughout. Autologous transfusion was deployed using cell salvage and a preoperative haemodilution technique. The patient was given tranexamic acid, desmopressin, recombinant factor VIIa, folic acid and epoetin alfa. Patients who object to transfusion represent a significant challenge, especially those who are at a high risk of coagulopathy associated with inherent aortic dissection leading to perturbed haemodynamics, cardiopulmonary bypass and hypothermic circulatory arrest. Type A aortic dissection repair is possible in patients refusing the use of blood products with blood salvage techniques and synthetic products that can limit the risk of bleeding. Minimal hypothermia is vital to preserve platelet function and avoid coagulopathy. Thus, a combination of normothermic/minimal hypothermia and antegrade cerebral protection with a blood conservation strategy can be deployed for a successful surgical outcome in aortic dissection without transfusion.