Korean J Dermatol.  2010 May;48(5):388-394.

Clinical Study on Adult Onset Still's Disease

• Yang YS
• Shin MK
• Hong SJ
• Lee YA
• Lee MH

Abstract

BACKGROUND
Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disease of an unknown etiology, and this is characterized by spiking fever, evanescent rash, arthritis and multiorgan involvement.
OBJECTIVE
This study was conducted to better understand the clinical characteristics of patients with AOSD, and especially the cutaneous features.
METHODS
We reviewed the medical records and cutaneous findings of 21 patients who were diagnosed with AOSD at our hospital from 2003 to 2009. The diagnosis of AOSD was based on the criteria proposed by Yamaguchi et al.
RESULTS
Eighteen (86%) out of the 21 patients were women, and the age of onset ranged from 18 to 55 years. Fever occurred in 21 (100%) patients, skin rash in 20 (95%), arthralgia in 18 (86%), sore throat in 10 (48%) and lymphadenopathy in 7 (33%). The cutaneous features were as follows: typical maculopapular eruption (70%), urticaria (10%), petechia and purpura (10%), persistent plaques and linear pigmentation (5%) and acne-like lesions (5%). The most common sites of skin lesion were the trunk (80%) and thighs (80%). The histopathologic findings of 9 patients showed non-specific chronic inflammation with a perivascular mononuclear preponderance in 6 patients, and the others were consistent with urticaria or leukocytoclastic vasculitis.
CONCLUSION
The various cutaneous features of this study suggest that dermatologists should consider AOSD in any patient with cutaneous eruption associated with fever and arthralgia.

Keyword

Adult onset Still's disease; Clinical features; Cutaneous lesions

MeSH Terms

Adult
Age of Onset
Arthralgia
Arthritis
Exanthema
Female
Fever
Humans
Inflammation
Lymphatic Diseases
Medical Records
Pharyngitis
Pigmentation
Purpura
Skin
Still's Disease, Adult-Onset
Thigh
Urticaria
Vasculitis
Vasculitis, Leukocytoclastic, Cutaneous
Vasculitis, Leukocytoclastic, Cutaneous