Extrapulmonary pneumocystosis is an exceedingly rare complication of P
neumocystis carinii pneumonia (PCP). Prior to the advent of the human
immunodeficiency virus type 1 (HIV-1) epidemic, only 16 cases of extra
pulmonary pneumocystosis in individuals who were immunocompromised by
a variety of underlying diseases had been reported. Since the beginnin
g of the HIV-1 and related PCP epidemic, at least 90 cases of extrapul
monary pneumocystosis have been reported. This review briefly presents
a history of the discovery of P. carinii and its recognition as a hum
an pathogen, the controversy regarding its taxonomy, and the epidemiol
ogy of this organism. A more detailed analysis of the incidence of ext
rapulmonary pneumocystosis in HIV-1-infected individuals and its occur
rence despite widespread prophylaxis for PCP with either aerosolized p
entamidine or systemic dapsone-trimethoprim is presented. The clinical
features of published cases of extrapulmonary pneumocystosis in non-H
IV-1-infected individuals are summarized and contrasted with those in
HIV-1 infected individuals. The diagnosis of extrapulmonary pneumocyst
osis is discussed, and because clinical microbiologists and pathologis
ts are the key individuals in establishing the diagnosis, the characte
ristic microscopic morphology of P. carinii as its appears when staine
d with a variety of stains is presented and reviewed. The review concl
udes with a brief discussion of treatments for extrapulmonary pneumocy
stosis.