HISTOLOGIC, ULTRASTRUCTURAL, AND IMMUNOCYTOCHEMICAL FEATURES OF THE GRANULOMAS SEEN IN A CHILD WITH THE SYNDROME OF FAMILIAL GRANULOMATOUS ARTHRITIS, UVEITIS, AND RASH

A family with a syndrome consisting of granulomatous arthritis, uveiti s, and rash was first described in 1985. Another family with the same syndrome was described subsequently in 1990. The condition, in both fa milies, is clinically different from sarcoidosis. To verify whether th e granulomas in such patients also could be distinctive, we performed histologic, ultrastructural, and immunocytochemical studies on skin an d synovial biopsy specimens from a child under our care who suffered f rom the same syndrome. The results indicated that the granulomas could not be distinguished from those seen in sarcoidosis either by light m icroscopy or by immunocytochemical studies. However, by electron micro scopy, ''wormlike'' or ''comma-shaped'' bodies were noted within the c ytoplasm of epithelioid cells. The significance of this observation is discussed.