Search results for "Subcutaneous nodule"

An 18-month-old girl with hoarseness, stiff joints and subcutaneous nodules

1998

Treatment of relapsing idiopathic nodular panniculitis (Pfeifer-Weber-Christian disease) with mycophenolate mofetil

1998

Idiopathic nodular panniculitis (Pfeifer-WeberChristian disease) is characterized by fever and symmetric subcutaneous nodules often located on the lower extremities.1,2 Histologically, a lobular panniculitis with a pronounced neutrophilic infiltrate is observed. The course of the disease is characterized by acute onset with fever and malaise and the simultaneous appearance of painful subcutaneous nodules. Some patients experience involvement of the intra-abdominal or retroperitoneal fat. Relapses are frequent, and a fatal outcome has been described repeatedly. Therapy usually consists of steroids in medium to high dosages, as well as immunosuppressive drugs such as azathioprine or methotrex…

Hyaline fibromatosis syndrome (juvenile hyaline fibromatosis): whole-body MR findings in two siblings with different subcutaneous nodules distributio…

2017

Abstract: Hyaline fibromatosis syndrome (juvenile hyaline fibromatosis) is a rare, progressive, autosomal recessive disorder whose main hallmark is the deposition of amorphous hyaline material in soft tissues, with an evolutionary course and health impairment. It may present involvement of subcutaneous or periskeletal soft tissue, or may develop as a visceral infiltration entity with poor prognosis. Very few radiological data about this inherited condition have been reported, due to the extreme rarity of disease. We herein present a case of two siblings, affected by different severity of the disease, with different clinical features. They were examined by whole-body MR (WBMR) in order to as…