[A patient with acute sarcoidosis associated with fever, polyarthritis, and erythema nodosum: a typical of Löfgren's syndrome].
A 26-year-old female was admitted to Aoyama Hospital in February 1996, for evaluation of abnormal chest shadows and polyarthritis. She visited our clinic in December 1995 with complaints of fever, fatigue, and polyarthralgia which lasted for 3 weeks. Two weeks later she developed erythema nodosum on her lower legs with the exacerbation of polyarthritis. Erythrocyte sedimentation rate was 108 mm/hr and CRP 4.9 mg/dl. A chest radiograph showed hilar lymphadenopathy and multiple nodular shadows in both lung fields. On admission, she had arthritis of the shoulders and knees, erythema nodosum on her lower extremities, and keloid-like skin lesion on her left knee. Thoracoscopic lung biopsy of nodular lesion and skin biopsy of keloid-like lesion revealed noncaseating granuloma of epithelioid cells with lymphocytes, macrophages, and giant cells, confirming the diagnosis of sarcoidosis. She was cured in three months, and was well and free of the symptoms thereafter. Löfgren's syndrome is acute sarcoidosis, characterized by arthritis, erythema nodosum, and bilateral hilar lymphadenopathy. This syndrome is common in Europe and is closely related to HLA-B8 and DR-3. The frequency of HLA-B8 and DR-3 in Japanese is almost 0%, explaining the rare onset of this syndrome in Japan. Our case is the second report of typical Löfgren's syndrome in Japan, although the patient did not have these HLA loci.
Koseki
Y
Y
Institute of Rheumatology, Tokyo Women's Medical College.
Terai
C
C
Nakajima
H
H
Tokuda
H
H
Ichikawa
N
N
Tsutsumino
M
M
Nakajima
A
A
Akama
H
H
Taniguchi
A
A
Hara
M
M
Kashiwazaki
S
S
D000208:Acute Disease; D000328:Adult; D001168:Arthritis / Q000209:etiology*; D004893:Erythema Nodosum / Q000209:etiology*; D005260:Female; D005334:Fever / Q000209:etiology*; D006801:Humans; D012507:Sarcoidosis / Q000175:diagnosis*; D013577:Syndrome