04 January 2021>: Articles
Intravenous Immune Globulin (IVIG) Therapy After Unsuccessful Treatment with Corticosteroid and Cyclosporine A in Pfeifer-Weber-Christian Disease: A Case Report
Unusual or unexpected effect of treatment, Rare disease
Geunwoong Noh A* , Chi Wha Han ADOI: 10.12659/AJCR.929519
Am J Case Rep 2021; 22:e929519
Figure 1. Effects of intravenous immunoglobulin (IVIG) therapy in Pfeifer-Weber-Christian disease (PWCD). (A) Change in skin nodules by IVIG therapy. Multiple crops of palpable masses on the left anterior chest decreased in size after IVIG therapy (before, immediately after, 3 days after, and 8 weeks after IVIG therapy). Chest deformities remained. The red arrows indicate masses. (B, C) Changes in computed tomography (CT) scan findings. The initial computed tomography (CT) scan findings were diffuse extensive infiltrative lesions involving the left anterior chest and abdominal wall, mainly involving the subcutaneous layer, with focal skin thickening, and probable lymphoma infiltrating subcutaneous fat was suggested with a probable diagnosis of cutaneous lymphoma as in the previous diagnoses in the other hospitals. On CT scan, the improvement in infiltrative multiple lesions on the left anterior chest began after 3 days. The lesions on the CT scan were much improved 8 weeks after IVIG therapy. Red arrows indicate masses. Figure B is the middle mass, and Figure C is the lower mass in Figure A.