J Rheum Dis.  2013 Aug;20(4):243-246. 10.4078/jrd.2013.20.4.243.

A Case of Adult-Onset Still's Disease Presenting with Periorbital Edema

• Yoon DL
• Lee S
• Park SY
• Kim YJ
• Koo BS
• So MW
• Kim YG
• Lee CK
• Yoo B

• KMID: 2297539
• DOI: http://doi.org/10.4078/jrd.2013.20.4.243

Abstract

Adult-onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown etiology that is characterized by high-spiking fever, arthralgia, sore throat, and skin rash. The typical rash of AOSD is an evanescent, salmon-colored erythema, which is considered to be the major diagnostic criterion. Recently, other cutaneous manifestations of AOSD, such as persistent plaque and urticaria, have been reported. Here, we report a rare case of AOSD presenting with periorbital swelling and erythema. A 47-year-old woman was presented with periorbital swelling, erythema, high fever, arthritis, and a sore throat. One year prior to admission, she was diagnosed with AOSD based on the diagnostic criteria of Yamaguchi. The patient's periorbital swelling and erythema may not have been associated with periorbital cellulitis because they did not respond to antibiotics but did improve after treatment with steroids. Considering all of her signs and symptoms with a history of AOSD, periorbital lesion was suspected as atypical cutaneous manifestation of AOSD.

Keyword

Adult-onset Still's disease; Cellulitis; cutaneous manifestation

MeSH Terms

Arthralgia
Arthritis
Cellulitis
Edema
Erythema
Female
Fever
Humans
Middle Aged
Pharyngitis
Steroids
Still's Disease, Adult-Onset
Urticaria
Steroids

Figure

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  Figure 1. (A) Cellulitis-like periorbital swelling of both eyelids. (B) Improvement was noted after 5 days of steroid treatment.

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  Figure 2. (A) Orbit CT scan showing left-side dominant periorbital soft tissue swelling and heteroge-neous enhancement (arrow). (B) Neck CT showing diffuse swelling of the left parotid gland with periglandular infiltration (arrow).

Reference

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