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Two cases of adult Still's disease with atypical rash

Saito A  Sato Y  Miyata M  Nishimaki T  
 Kasukawa R  

In: Ryumachi (1998 Jun) 38(3):516-22

ISSN: 0300-9157  (Published in Japanese)

Adult Still's disease is a febrile disorder of unknown etiology,  characterized by spiking fever, arthralgia, leukocytosis and a  typical rash. However, most of these clinical findings, except for  the typical rash, are not specific to this disease: therefore, the  typical rash is the most important clinical finding for diagnosing
 this disease and physicians often have difficulty in making a  definite diagnosis without the typical rash. 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.

Institutional address:
       Department of Internal Medicine II
       Fukushima Medical College.


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