Sarcoidosis(redirected from Besnier-Boeck-Schaumann syndrome)
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in man, a systemic granulomatous disease that affects various organs and tissues and produces a variety of symptoms. It was initially described as a skin disease in 1889 by the French dermatologist E. Besnier and the Norwegian dermatologist C. Boeck. Sarcoidosis was first studied as a systemic disease by the Swedish dermatologist J. Schaumann in the first quarter of the 20th century.
The etiology of sarcoidosis is unknown. The disease was once believed to be tuberculous in origin, but this theory has been abandoned by most specialists. Sarcoidosis seems to be similar to reticulosis. It affects the skin (sarcoids of Boeck and other manifestations), the peripheral lymph nodes, and, most commonly, the thoracic lymph nodes and the lungs. Sarcoidosis of the eyes is often in the form of iridocyclitis, and sarcoidosis of the bones is often in the form of osteitis fibrosa cystica. The nervous system and other organs are less commonly affected. There is no cardinal symptom.
Sarcoidosis is diagnosed on the basis of all available data. The disease process, which is usually chronic, is studied, as are the roentgenologic symptoms of pulmonary involvement. The morphological structure is examined: sarcoid granuloma, which consists of epithelioid, lymphoid, and giant noncaseating cells, is characteristic.
The prognosis is usually favorable, and spontaneous involution, especially of the thoracic lesions, is possible. Relapses are common. The prognosis is worse if pneumosclerosis has developed and if the eyes and central nervous system are involved. When the disease is not treated, the mortality rate is approximately 5 percent. Sarcoidosis is treated by the prolonged administration of corticosteroids.
REFERENCESRaben, A. S. Sarkoidoz. Moscow, 1964. (Bibliography.)
Lebacq, E. La sarcoïdose de Besnier-Boeck-Schaumann. Brussels, 1964.
5th International Conference on Sarcoidosis. Prague, 1971.
A. S. RABEN