Emergence of panniculitis and haemophagocytic syndrome in a patient with chronic systemic lupus erythematosus.
Panniculitis rarely occurs in the course of systemic lupus erythematosus (SLE). When it occurs, it is thought to be mainly lupus erythematosus panniculitis (LEP). Here we describe a 32-year old Japanese woman with chronic SLE, who simultaneously presented facial lymphocytic lobular panniculitis and pancytopenia due to haemophagocytic syndrome. She showed several auto-antibodies against trilineage haematopoetic cells, an anti-cardiolipin antibody, and no evidence of viral infection, indicating that her haemophagocytic syndrome might be autoimmune-associated haemophagocytic syndrome. The panniculitis and haemophagocytic syndrome presented simultaneously, and these manifestations were dramatically improved with corticosteroid therapy; therefore, the lymphocytic lobular panniculitis could be linked to autoimmune-associated haemophagocytic syndrome in this case.
Deji
N
N
The Department of Medicine, Shiga University of Medical Science, Seta, Otsu, Japan.
Sugimoto
T
T
Fujimoto
T
T
Aoyama
M
M
Takeda
N
N
Sakaguchi
M
M
Uzu
T
T
Kashiwagi
A
A
D000328:Adult; D001706:Biopsy; D002908:Chronic Disease; D004630:Emergencies; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000473:pathology* / Q000601:surgery; D015435:Panniculitis, Lupus Erythematosus / Q000150:complications* / Q000473:pathology* / Q000601:surgery