非定型疹として膿疱を伴った成人Still病

・丘疹や膿疱などの非定型疹を伴う成人Still病を経験．Stevens-Johnson症候群と血球貪食症候群を合併した．

・膿疱部では角層内と表皮上層に異常角化細胞あり．

・治療抵抗性の痒みの強い丘疹にコルヒチンが有効であった．

（「症例のポイント」より）

　

A case of adult onset Still disease with pustular eruptions as atypical cutaneous manifestations

　

Kato, Kohei1）Kawai, Rana2）Furukawa, Yuki2）Ichimasu, Nao2）Ugajin, Tsukasa3）Katagiri, Kazumoto2） 1）Department of Dermatology, Tokyo Metropolitan Bokutoh Hospital 2）Department of Dermatology, Dokkyo Medical University Saitama Medical Center 3）Department of Dermatology, Tokyo Medical And Dental University Hospital Faculty of Medicine

　

A 77-year-old female presented to our hospital with a 18-day history of fever, skin rash, and liver dysfunction following sore throat. Clinical examination revealed pustules and erythema on her upper arms, scattered erythematous papules and erythematous plaques on the limbs and trunk. Histological examination revealed the subcorneal pustules and individual necrotic keratinocytes in the horny layer and the upper epidermis, which were characteristic features of atypical skin rash of adult onset Still disease. Persistent pruritic erythematous papules that remained after administration of systemic glucocorticoids disappeared by administration of colchicine. Then she developed Stevens-Johnson syndrome due to drugs, cytomegalovirus antigenemia, and hemophagocytic syndrome following bacterial infection, which were successfully treated by systemic glucocorticoids and cyclosporine in addition to antibiotics and antiviral medicine. Atypical rash including pustules, as shown in this case, is important findings for precise diagnosis of adult onset Still disease as well as an indicator of prognosis.