A case of Weber-Christian disease with later development of rheumatoid arthritis

Abstract

summaryWeber Christian disease (WCD) is a syndrome characterized by recurrent subcutaneous nodules, fever, occasional lipoatrophy, fatigue, arthralgia, and myalgia. We report a case of WCD associated with rheumatoid arthritis. A 65 year old woman consulted our outpatient clinic because of bilateral hand swelling. The patient had presented with fever and subcutaneous nodules in her trunk and upper and lower extremities in 1983. At that time, the dermatology department diagnosed this patient as having WCD after biopsy of the nodules demonstrated lobular panniculitis. She has been treated with corticosteroid (5 15 mg/day) since then. The patient continued to have recurrent episodes of transient in‰ammatory arthritis in the small joints of theˆngers and fever, and was initially assessed at our institution in October 2007. Finally, in November 2007, she was diagnosed as having both WCD and rheumatoid arthritis (RA) and treated with corticosteroid (5 mg/day) and methotrexate (MTX) (7.5 mg/week). Thereafter, her clinical symptoms gradually improved. This is the second case of WCD showing the subsequent development of RA, successfully treated with MTX, in the English literature. This case may provide clinical insight into WCD and RA.

Long-term follow-up of a female patient with Weber–Christian panniculitis

“…В литературе представлено очень мало данных о прогнозе и выживаемости у пациентов с болезнью Вебера-Крисчена, поскольку известны лишь единичные случаи длительного (более 5 лет) наблюдения [4][5][6][7]. В связи с этим в настоящее время не существует прогностических критериев заболевания и предикторов, определяющих характер его течения и степень активности, тяжесть состояния пациентов и риск развития висцеральных поражений.…”