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Late Hereditary Syphilis

bone, frequently, manifestations, occur, tibia, periostitis, lesions and bones

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LATE HEREDITARY SYPHILIS By late hereditary syphilis we mean all organic affections of later childhood and of adult life, that are caused by hereditary syphilis and that are analogous to the teritary manifestations of the acquired form. Age is of less significance here than the peculiar manifestations of syph ilis; the gamma being the foundation of late hereditary syphilis. As a rule gummatous processes in hereditary syphilis do not arise before the fifteenth year, but when they occur earlier, as they do exceptionally, even in the third and fourth year, they must be classed nevertheless as manifestations of late hereditary syphilis. (See Recurrences in Early Childhood.) There are authors who still believe in the possibility of a latent period in hereditary syphilis to the time of puberty, and who consider only such cases as late hereditary syphilis in which tertiary symptoms have not been preceded by early manifestations of syphilis during the first few years of life. There are no plausible grounds for assuming such an outbreak of tertiary symptoms alone. In children who apparently have late hereditary syphilis alone, early manifestations may have been overlooked because of the possible absence of eruptions, or there may have been a contact syphilis that ran its course early in childhood and escaped observation.

As the extreme age at which condylomatous recurrences may take place in hereditary syphilis I would designate the sixth year, during which year I have still found condylomat a of the mucous membrane. As a rule these do not occur even after the fourth year.

In late hereditary syphilis, genuine syphilitic manifestations are much more sharply distinguished from parasyphilitic affections than in the early period of the disease, these parasyphilitic manifestations in volving especially the central nervous system in the form of tabes and paralytic dementia. Late hereditary syphilis frequently appears first during the period of second dentition or during puberty.

We will now discuss briefly in the order of their frequency the lesions of the different organs involved in late hereditary syphilis.

First are affections of the eye which will only be mentioned, leav ing their detailed discussion to the oculist. Here belong parenchymatous keratitis, gummatous involvement of the iris and the so-called deep inflammations of the eye, choreoretinitis and optic neuritis.

Very frequently ocular and aural lesions appear at the same time, but the latter usually occur later. When the ears are affected, there is rapid, usually bilateral, deafness. It occurs much more frequently after puberty than before and is accompanied by symptoms of dizziness and subjective noises. Deafness is due to neuritis acustica (Meniere's

disease).

Late syphilitic changes in the osseous system take place, either as a diffuse hyperplastic ostitis and periostitis, or as a gummatous process; both lesions, however, may occur not only simultaneously in the same individual but also in the same bone.

The hyperplastic ostitis and periostitis may involve the whole skeleton (Lannelongue) and may cause visible swellings of the cranial bones as well as of the long hollow bones (see Plate 30). And yet hyper plastic processes in the cranial bones occur incomparably less frequently in late than in early hereditary syphilis. The long hollow bones are most frequently affected, especially the shinbones, their involvement in late hereditary syphilis forming an extremely characteristic clinical picture. In general, all of the bone lesions of tertiary acquired syphilis occur also in late hereditary syphilis. Lannelongue considers the so called Paget's bone disease, which is a diffuse, progressive periostitis leading to hyperostosis and finally affecting the whole skeleton, as noth ing more than hereditary bone syphilis.

As mentioned above, the tibia is the bone most frequently involved. There occur at first on the crest of the tibia rather soft and very tender swellings. The skin over these areas is frequently slightly reddened and sensitive. At the same time there is frequently spontaneous pain in the lower extremities increased decidedly on attempting to move them. The first stage of the affection during which new tophi appear one after the other on the edge of the tibia, may last for months or may progress so rapidly that they resemble erythema nodosum. The second stage is the real stage of hyperostosis. The entire shin becomes swollen, thickened, and the sharp edge of the bone disappears. The anterior border of the bone becomes rounded and is covered with palpable hard nodules and rough areas. The whole bone is diseased. Anatomically this deformity is due to the continuous formation of new periosteal bone layers about. the primary bone. Even the hollow portion of the bone is, in many cases, obliterated for considerable distances and is filled by compact bone substance. The tibia is frequently bent, with marked anterior convexity, the bone assuming the shape of a Turkish sabre; or, if the ossifying periostitis of the anterior margin of the tibia far exceeds that of the other surfaces, we have the sabre sheath defor mity of Hutchinson and A. Fournier (see Plate 30).

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