A dermatomyositislike syndrome associated with viral infection has been reported as a rare complication of congenital X-linked (Bruton's) agammaglobulinemia.1-5 The syndrome is characterized by muscle weakness, edema, and woody induration of the skin with a violaceous rash over the extensor surfaces of joints. Death results from an accompanying progressive meningoencephalitis caused by echovirus infection despite appropriate γ-globulin replacement therapy.2-5 We describe such a patient with Bruton's agammaglobulinemia who died of meningoencephalitis caused by coxsackievirus B3.
Methods.—Viral cultures of premortem and postmortem specimens were performed at the Virus Diagnostic Laboratory, Methodist Hospital, Houston, and at the Centers for Disease Control, Atlanta.
Report of a Case.—A 28-year-old man had congenital X-linked agammaglobulinemia that was diagnosed at 4 years of age and reported by Sorensen and Partington in 1956.6 At the time of diagnosis, therapy was started with intramuscular (IM) γ-globulin injections, with a subsequent decrease in his
COOPER JB, PRATT WR, ENGLISH BK, SHEARER WT. Coxsackievirus B3 Producing Fatal Meningoencephalitis in a Patient With X-Linked Agammaglobulinemia. Am J Dis Child. 1983;137(1):82-83. doi:10.1001/archpedi.1983.02140270072022