Objective To investigate the clinical characteristics of multisystem impairment of Sj(o)gren's syndrome (SS) to make quick and accurate diagnose and treatment to decrease the rate of misdiagnosis and mistreatment.Methods A case of thrombotic thrombocytopenic purpura with SS was reported and the related literatures were reviewed.The clinical manifestations,laboratory examinations,diagnostic criteria and therapy of thrombotic thrombocytopenic purpura with SS were analyzed.Results Purpura haemorrhagica,hemolytic anemia,central nervous systemic symptoms,kidney impairment and fever were the representative clinical manifestations of thrombotic thrombocytopenic purpura with SS.Plasmapheresis was the most effective therapy with the background treatment with glucocorticosteroid and immunosuppressive agents.Conclusion Thrombotic thrombocytopenic purpura with SS presents a complicated clinical manifestations,so misdiagnosis and mistreatment is very frequent.More attention should be paid to this specific situation because the disease is rare and has high mortality rate.

We report a case of congenital accessory scrotum and pseudodiphallia with perineal lipoma. A 7-month-old boy was admitted to Xiamen Children's Hospital for a postnatal perineal mass. Physical examination found a mass of 4 cm×4 cm between the scrotum and anterior edge of the anus, with pigmented and wrinkled skin resembling the scrotum without a testicle. There was also a penis-like swelling on the surface of the mass without a urethral orifice. The penis and scrotum with bilateral testis were normal, as were the other organs. The patient's chromosome was 46,XY. Perineal ultrasound, MRI, and voiding cystourethrogram indicated no association between the mass and the penis, urethra, rectum, and anus. A resection of the mass and penis-like swelling and perineoplasty was performed because the normal penis was found to rotate clockwise about 90 degrees during the operation. The pathology showed that the masses were accessory scrotum and lipoma and the penile-like swelling was a pseudodiphallia without corpus cavernosum. Congenital accessory scrotum and pseudodiphallia with a perineal lipoma are rare. It is essential to clarify the anatomical relationship between the mass and genitourinary tract or anorectum before operation.