Case Report Localised Hyaline Vascular Type of Castleman’s Disease Mimicking Adult-Onset Still’s Disease Syh-Jae Lin 1 , Chuen Hsueh 2 and Hsun-Chin Chao 1 Departments of 1 Pediatrics and 2 Pathology, Chang Gung Children’s Hospital, Kweishan, Taoyuan, Taiwan Abstract: A previously healthy 18-year-old boy pre- sented with daily spiking fever, polyarthritis, and evanescent skin rashes, as well as hepatomegaly and Raynaud’s phenomena for 2 months. He was initially diagnosed with adult-onset Still’s disease (AOSD). During the period of follow-up, intermittent fever and migratory polyarthritis persisted and an insidiously growing mass over the right axillary region was noted 1 year after the diagnosis of AOSD. Excisional biopsy of the mass revealed a group of lymph nodes with histological features of the hyaline vascular type of Castleman’s disease. The patient’s symptoms disap- peared soon after excision of the lymph nodes. Keywords: Adult-onset Still’s disease; Castleman’s disease; Hyaline vascular variant Introduction Castleman’s disease was first described in a group of patients with localised mediastinal lymph node enlarge- ment, which has been referred to as giant lymph node hyperplasia [1]. Unlike the plasma cell variant of Castleman’s disease, which tends to be clinically aggressive with frequent systemic manifestation [2], the hyaline vascular variant of Castleman’s disease rarely presents with systemic symptoms [3,4]. Adult- onset Still’s disease (AOSD) is a clinical diagnosis characterised by quotidian spiking fevers, arthritis, evanescent rash, lymphadenopathy, hepatosplenomegaly and serositis [5]. A clinical picture compatible with the diagnosis of AOSD has not been described in the localised hyaline vascular type of Castleman’s disease. We report such a case in an 18-year-old male patient who presented prolonged fever and polyarthritis with an initial diagnosis of AOSD. The diagnosis of hyaline vascular type of Castleman’s disease was made 1 year later, when the patient developed an insidiously growing mass over the right axilla. Case Report A previously healthy 18-year-old boy was admitted to our institution with a history of fever, skin rash and multiple joint pain for about 2 months before admission. The admission examination disclosed an ill-looking boy with a high fever (body temperature 39 8C), slight hepatosplenomegaly, and swollen and tender bilateral wrists, elbows, knees and ankles. He had a generalised pruritic maculopapular rash, which was more evident when the fever attacked. Raynaud’s phenomenon was prominent, but lymphadenopathy was not. Laboratory studies showed the following values: haemoglobin 12.3 mg/dl, white blood cells 23100/mm 3 , with 92% neutrophils. The erythrocyte sedimentation rate was 85 mm/h, C-reactive protein (CRP) 121 mg/l, alanine aminotransferase 33 IU/l, serum ferritin 4920 ng/ml. Rheumatoid factor (RF) and antinuclear anti- bodies (ANA) were not detected, while cryoglobuli- naemia was present. Cultures of blood and urine were negative. There was no serological evidence of active streptococcal, salmonella, cytomegaloviral or Epstein- Barr viral infection. Other laboratory results, including Clin Rheumatol (1999) 18:485–487 ß 1999 Clinical Rheumatology Clinical Rheumatology Correspondence and offprint requests to: Dr Syh-Jae Lin, Department of Pediatrics, Chang Gung Children’s Hospital, 5 Fu-Hsin Street, Kweishan, Taoyuan, Taiwan. Tel: 886-3-3281200, ext 8218; Fax: 886-3-3288957.