of adult Still's disease with atypical rash
Saito A Sato
Y Miyata M Nishimaki T
In our department and related institutions, we have encountered 10 patients with Adult Still's disease who fulfilled the preliminary criteria for classification as Adult Still's disease, proposed by the Adult Still's Research Committee in Japan. The three major criteria are fever, arthralgia and typical rash, and 8 of 10 patients had an atypical rash; one satisfied two major criteria, and had an atypical rash and the other satisfied three major criteria and had an atypical rash on her eyelids.
Here, we present the two cases of Adult Still's disease with atypical rash. The first patient was a 36-year-old male with an itchy annular erythema chronicum migrans, frequently seen inpatients with Lyme's disease, on his back. His clinical symptoms improved and the erythema disappeared after treatment with corticosteroids.
The second patient, a 17-year-old female, had three major findings. In addition to the typical rash on her face, she had a heliotrope rash, usually seen in patients with dermatomyositis, on her eyelids. The typical rash on her face was related to her other clinical manifestations, and improved after treatment with corticosteroids. However, the rash on her eyelids showed no improvement after steroid therapy, suggesting that the erythema was probably not related to Adult Still's disease.
Typical rash in
Adult Still's disease, defined as a macular or maculopapular nonpruritic
salmon pink eruption, was demonstrated to have the highest relative value
associated with relatively high sensitivity and specificity for the
diagnosis of Adult Still's disease. However, we have to be aware that some
patients with Adult Still's disease could also have an atypical rash.
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