This report describes clinical and immunologic features of five illust
rative cases of paracoccidioidomycosis in previously healthy children.
All had disseminated disease and two of them died despite treatment.
The major clinical presentation in four patients was fever and diffuse
superficial and intraabdominal adenopathy, with or without hepatosple
nomegaly. Other sites were also affected: three patients had multiple
osteoarticular lesions, occasionally with intense tissue destruction;
two had cutaneous eruptions; two had pericardial effusions; and two ha
d pulmonary involvement, once considered an organ spared in the young.
We detected variable lymphocyte responses to mitogens and to Candida
albicans antigen and nonresponsiveness to Paracoccidioides brasiliensi
s cell wall antigen. High concentrations of serum immunoglobulins and
anti-P. brasiliensis antibodies were present. These immune alterations
tended to resolve with treatment, suggesting a reversible nature of t
he immune defect. We conclude that this mycosis has a high morbidity a
nd mortality in children, which is probably related to an antigen-spec
ific immunodeficiency. Further studies are needed to increase knowledg
e of this mycosis in children.