J Korean Rheum Assoc.  2007 Jun;14(2):160-165. 10.4078/jkra.2007.14.2.160.

Two Cases of Adult Onset Still's Disease with Concomitant Hemophagocytic Syndrome

• Kim HS
• Park KW
• Kim SK
• Kim JY
• Park SH
• Shin JH
• Kim SG
• Choe JY

• KMID: 1526434
• DOI: http://doi.org/10.4078/jkra.2007.14.2.160

Abstract

Adult onset Still's disease is an rare inflammatory disease with the characteristic of fever, skin rash, arthralgia or arthritis, lymphadenopathy, leukocytosis and multiple systemic organ involvement. Its accurate pathogenesis has not been elucidated yet. Its clinical manifestation is also very diverse, from relatively mild symptoms to severe complications such as concomitant infection, liver failure, disseminated intravascular coagulation, myocarditis, adult respiratory distress syndrome, which may lead to death in some cases. Particularly, concomitant hemophagocytic syndrome is rare complication that could induce a fatal outcome. Thus it is important to diagnose early and start treatments. Until now, it has been reported in only one case of adult onset Still's disease in Korea. Here, we report two female cases of adult onset Still's disease with concomitant hemophagocytic syndrome.

Keyword

Adult onset Still's disease (AOSD); Hemophagocytic syndrome

MeSH Terms

Adult*
Arthralgia
Arthritis
Disseminated Intravascular Coagulation
Exanthema
Fatal Outcome
Female
Fever
Humans
Korea
Leukocytosis
Liver Failure
Lymphatic Diseases
Lymphohistiocytosis, Hemophagocytic*
Myocarditis
Respiratory Distress Syndrome, Adult
Still's Disease, Adult-Onset*

Figure

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  Fig. 1. Faint salmon-colored rashes on the neck, trunk (A), and face (B).

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  Fig. 2. Bone marrow aspiration reveals histiocytes phagocytizing red blood cells and neutrophils (χ 400).

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  Fig. 3. Atypical lymphocytes in peripheral blood smear (∗400) (A) and phagocytizing histiocyte (arrow) in bone marrow aspirates (x400) (B).

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