ABSTRACT
OBJECTIVES: To report a case of multifocal fibrosclerosis with a nine-year follow up, and to discuss this disease's radiological appearance and management. The disease is a rare systemic disorder of unknown cause characterised by fibrous proliferation involving multiple anatomical sites. CASE REPORT: A 50-year-old woman presented with histological findings characterised by similar inflammatory processes involving the meninges, pituitary gland, peritoneum, retroperitoneum and orbits, prompting a search for a common pathophysiology. A diagnosis of multifocal fibrosclerosis was postulated. Symptom improvement was noted after treatment with prednisone and azathioprine. CONCLUSION: This is the first documented case of involvement of the cochleovestibular nerve in a patient with multifocal fibrosclerosis. The rare association between fibrotic diseases and masses showing various clinical patterns should be kept in mind by otolaryngologists, and imaging performed to investigate for multifocal fibrosclerosis. However, diagnosis can only be confirmed with tissue biopsy and histopathological examination.
Subject(s)
Meningitis/pathology , Retroperitoneal Fibrosis/congenital , Sensation Disorders/complications , Vestibulocochlear Nerve Diseases/pathology , Azathioprine/therapeutic use , Biopsy , Diagnosis, Differential , Disease Progression , Female , Glucocorticoids/therapeutic use , Humans , Immunosuppressive Agents/therapeutic use , Magnetic Resonance Imaging , Meningitis/complications , Meningitis/diagnosis , Middle Aged , Orbital Diseases/complications , Orbital Diseases/diagnostic imaging , Orbital Diseases/pathology , Prednisone/therapeutic use , Radiography , Recurrence , Retroperitoneal Fibrosis/complications , Retroperitoneal Fibrosis/diagnosis , Retroperitoneal Fibrosis/pathology , Retroperitoneal Fibrosis/therapy , Seizures/complications , Sensation Disorders/pathology , Sinusitis/complications , Vestibulocochlear Nerve Diseases/complicationsABSTRACT
Group B streptococcus (Streptococcus agalactiae) is a common etiology of bacteremia among adults. Pyomyoma is a rare infectious complication of uterine leiomyomas. We report the case of a diabetic postmenopausal woman with a giant pyomyoma simulating an ovarian cancer. It was associated with S. agalactiae endocarditis and deep venous thrombosis of the right external iliac and femoral veins. Treated initially with intravenous penicillin, amikacin, and anticoagulation, the patient later had abdominal hysterectomy with an uneventful recovery. We also review the cases of pyomyoma reported since 1945. Of 14 cases described (including ours), mortality was 21%. Endocarditis was never reported in association with pyomyoma. The presence of bacteremia and a leiomyoma should raise suspicion for this disease.
Subject(s)
Abscess/diagnosis , Endocarditis, Bacterial/diagnosis , Leiomyoma/diagnosis , Ovarian Neoplasms/diagnosis , Streptococcal Infections/diagnosis , Streptococcus agalactiae , Abscess/microbiology , Abscess/surgery , Diagnosis, Differential , Endocarditis, Bacterial/complications , Female , Humans , Leiomyoma/microbiology , Leiomyoma/surgery , Middle Aged , Risk Factors , Streptococcal Infections/complications , Tomography, X-Ray ComputedABSTRACT
Primary biliary cirrhosis (PBC) is a cholestatic liver disease, which is characterized by a chronic inflammatory destruction of intrahepatic bile ducts. It is a rare disorder whose precise etiology is still to be elucidated. Even though the liver is the principal target of PBC, other organ systems also might be affected. Muscular involvement has rarely been described in this disease, and in the majority of cases, muscular weakness has been interpreted as polymyositis. We report the case of a 48-year-old woman suffering from classic PBC, in association with a myopathy whose histological features are distinct from the cases reported before. We also performed a MEDLINE research for PBC and concomitant muscular diseases.
