6533b82afe1ef96bd128b998

RESEARCH PRODUCT

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

Alexander EnkJürgen Knop

subject

medicine.medical_specialtyPrednisolonemedicine.medical_treatmentAzathioprineDermatologyWeber–Christian diseaseMycophenolic acidMalaiseRecurrencemedicineHumansGlucocorticoidsChemotherapybusiness.industryMiddle AgedMycophenolic Acidmedicine.diseaseDermatologySurgeryPanniculitis Nodular NonsuppurativeSubcutaneous nodulePrednisoloneFemalemedicine.symptombusinessPanniculitisImmunosuppressive Agentsmedicine.drug

description

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 methotrexate. Even with this treatment the disease tends to relapse and has an uncertain prognosis.

https://doi.org/10.1016/s0190-9622(98)70340-8