LXIX Scleroma

Abstract

A native-born resident of Iowa was recently seen at the Univer sity Hospitals with florid scleroma. 40 From this patient we recovered an organism satisfying the descriptions of Klebsiella rhinoscleromatis (see Table I). In reviewing the literature regarding this disease, rare in this area, we were puzzled by the contradictory statements regard ing the etiological role of this organism in scleroma. KOCH'S POSTULATESDeveloping unequivocable proof of the etiological relationship between a specific micro-organism and a disease is very different from suspecting or having some evidence of a relationship. Koch first offered this kind of proof in a logical chain of experimental evidence, usually known as Koch's postulates. Briefly stated, these postulates are:

Aetiopathogenesis of Scleroma

“…K. rhinoscleromatis could be isolated in all the cases of the untreated group, however, the poor isolation rate of the organism in others, could be due to previous streptomycin treatment taken by the patients. Steffen and Smith (1961) confirmed the postulation, by reproducing the lesions of scleroma. They inoculated K. rhinoscleromatis in albino mice by intranasal and intra-cerebral routes.…”

A novel murine model of rhinoscleroma identifies Mikulicz cells, the disease signature, as IL‐10 dependent derivatives of inflammatory monocytes

“…Yet, the molecular and cellular mechanisms underlying rhinoscleroma are not yet understood. In this study, using several congenic and knock-out mice strains, we reinvigorated the initial work of Steffen and Smith (1961) and successfully developed and characterized a murine model reproducing the earliest signature of rhinoscleroma: the appearance of the characteristic Mikulicz cells in the respiratory tract. Mikulicz cells are often described morphologically as foamy macrophages.…”

“…To our knowledge, three other models of rhinoscleroma were previously developed in rabbit, rat and mouse (Gaafar et al, 2000; Steffen & Smith, 1961; Talaat et al, 1978). Despite different experimental conditions (infectious doses, inoculation route, time after infection), a common feature of all models is the occurrence of Mikulicz cells, confirming the pivotal function of these cells in the infectious process.…”

“…Mikulicz cells are only documented in rhinoscleroma, suggesting a central role in creating the immunopathological environment that sets the stage for chronic granulomatous inflammation (Canalis & Zamboni, 2001). There are few reports of infection of an animal model by K. rhinoscleromatis describing the formation of Mikulicz cells in either mouse (Steffen & Smith, 1961), rat (Gaafar et al, 2000) or rabbit (Talaat et al, 1978). Yet, the precise description of Mikulicz cells is not known, nor how they are recruited and what are the factors required for their maturation.…”