DOI: 10.36347/SJMCR.2019.v07i12.016

Pages: 776-780

Cutaneous involvement in hyper-IgG4 syndrome is rarely reported and rarely is in the foreground. We report an observation of a 56-year-old woman had an infiltrated erythematous placard of the face, surmounted by subcutaneous nodules in front of the cheeks, evolving during 10 years. Cutaneous biopsy showed dense lymphoplasmocytic septal panniculitis with fibrosis. The synthetic antimalarials were prescribed in the hypothesis of a lupus panniculitis, associated with the general steroids, without improvement. Paraclinical investigations revealed pachymeningitis, mediastinal lymphadenopathy, and a high IgG4 serum assay. The diagnosis of hyper-IgG4 syndrome was retained. Given the corticoresistance, treatment with rituximab has been proposed. The patient experienced acute respiratory distress with fatal evolution. This observation underlines the interest of looking for the IgG4 syndrome in the case of an infiltrated placard with histology fibrosis and a dense lymphoplasmocytic infiltrate, after eliminating the other differential diagnoses.