[Infliximab for a girl with refractory pyoderma gangrenosum].

Pyoderma gangrenosum is a rare chronic ulcerative noninfectious disease of the skin. Half of patients are complicated with other autoimmune diseases, most commonly inflammatory bowel disease, Takayasu disease, and rheumatoid arthritis. It has been reported that approximately 4% of them were childhood-onset. The conventional treatments of pyoderma gangrenosum were described as systemic corticosteroids and cyclosporine. The combination of corticosteroids with immunosuppressants such as tacrolimus, mycophenolate mofetil has been reported as steroid-sparing modalities. We herein reported a girl, 12 years of age, having pyoderma gangrenosum refractory to the conventional combination of systemic prednisolone with cyclosporine, but successfully treated with infliximab, the anti-TNFalpha monoclonal antibody. Rapid improvement of pyoderma gangrenosum was seen within three doses of infliximab infusion. All skin lesions eventually healed completely and new skin ulcers were never coming out again. The dramatical improvement suggested that infliximab should be considered for patients with refractory pyoderma gangrenosum though further experiences and investigations are required to determine the mechanism of infliximab.

[A case of aortitis syndrome diagnosed with 18F-fluorodeoxyglucose-positron tomography (18F-FDG-PET) in the early pre-pulseless phase].

A 14-year-old girl with aortitis syndrome in the early pre-pulseless phase was admitted to our hospital because of slight fever, neck bruit, asymmetrical blood pressure, stenosis or dilatation of the main branch arteries in aorta on chest computed tomography. Laboratory examination revealed a high level of C-reactive protein and an elevated erythrocyte sedimentation rate, as well as hypergammaglobulinemia, and 18F-FDG-PET revealed an accumulation of 18 fluorodeoxyglucose in the great vessels. She was first given pulse therapy with a combination of methylprednisolone and intravenous cyclophosphamide, and then maintenance therapy with oral prednisolone and azathioprine. All the abnormal laboratory parameters improved to normal levels within a month. We suggest that early diagnosis of aortitis syndrome may permit early treatment in the early pre-pulseless phase and could possibly prevent progression to the occlusive phase.