The Great Imitator, Syphilis-Medical Staff Conference, University of California, San Francisco

Refer to: Fitzgerald F: The great imitator, syphilis-Medical Staff Conference, University of California, San Francisco. Medical Staf Conference West J Med 134:424-432, May 1981 The Great Imitator, Syphilis These discussions are selected from the weekly staff conferences in the Department of Medicine, University of California, San Francisco. Taken from transcriptions, they are prepared by Drs. David W. Martin, Jr, Pro- fessor of Medicine, and James L. Naughton, Assistant Professor of Medi- cine, under the direction of Dr. Lloyd H. Smith, Jr, Professor of Medicine and Chairman of the Department of Medicine. Requests for reprints should be sent to the Department of Medicine, University of California, San Fran- cisco, School of Medicine, San Francisco, CA 94143. DR. TIERNEY: * The topic of this nmedical staff lology. By the mid-1950's hospitals were no coniference is the "great imitator," syphilis. Dr. longer required to do routine admission serological Faithl Fitzgerald, a University of Californiia, San tests for syphilis to become accredited. A genera- Francisco, alumna who has recently returned to tion of physicians were trained without seeing Califortnia after a two-year absenice, will discuss syphilis in all its protean manifestations. It was this disease of increasing importance. no longer the case, as it had been in Osler's time, that to know in its manifestations the 15th and 17th syphilis many DR. FITZGERALD:t Between and variations was to know clinical medicine.1 centuries, waves of pestilence swept Europe, deci- mating populations. The Black Death, bubonic But syphilis flourished in neglect. Its resurrec- plague, persists today but only in pockets. Typhus tion was abetted by increasing promiscuity, a and typhoid have been largely eliminated by relatively simple therapy and a loss of detective modern hygiene. Smallpox is, at last, a conquered diagnostic skill. Because syphilis had been "con- disease. But greatpox, syphilis (so called because quered," the federal government withdrew fund- it was so much more dreadful than smallpox), is ing for US Public Health Service (usPHs) epide- still epidemic among us. miologic studies and case finding.2 Apocrypha has it that the sailors of Columbus In the early 1970's there were 23,000 cases of brought gonorrhea to the New World and returned infectious syphilis per year reported to the USPHS. with syphilis to the Old. As in much early colonial A conservative estimate has it that for every case trade, Europe got more than it gave. reported, nine are not. This implies almost a The history of syphilis is a complex drama in- quarter of a million new cases of syphilis per volving the rise and fall of kings, changing govern- year in the United States.3 There is still a great ments and devastating wars. The final act was to deal of syphilis for primary physicians to recog- have been played in 1943, with the general intro- nize, understand and treat. duction of penicillin. It was a "magic bullet"; cases of reported syphilis plummeted. The words Pathophysiology "and syphilology" were dropped from the title Syphilis may have lessons to teach us in this of the AMA A tchives of Dermatology and Syphi- modern immunologic era of medicine. Consider *Lawrence M. Tierney, Jr, MD, Associate Professor of Medi- this most unusual infectious disease: An organ- cine; Assistant Chief. Medical Service, Veterans Administration ism, Medical C(enter, San Francisco. the delicate spirochete Treponenma pallidum, tFaith Fitzgerald, MD, Associate Professor of Medicine; Asso- is no longer than a red blood cell. It is exquisitely ciatc Dean for Student and Curricuilar Affairs, University of Cali- fornia, Davis, School of Medicine. scnsitive to drying, heat and air. Yet it can infect 424 MAY 1981 v 134 * 5 SYPHILIS lation have continued to 19558 (Figure 1). The ABBREVIATIONS USED IN TEXT various stages of syphilis are discussed below (see FTA-ABS= fluorescent treponemal antibody Table 1). absorption (test) TPI = Treponema pallidum immobilization (test) A cquired Syphilis Primary. The cardinal feature of the primary stage is the chancre, occurring at the site of inocu- a human being anytime from the fourth month lation within three months of the exposure. Most of intrauterine life to old age. It may affect any chancres begin as papules, then erode and become system and can remain destructive for the victim's ulcerative. They are painless, indolent, punched- entire lifetime. Or it may lie dormant for a quar- out lesions which have a scanty yellow discharge. ter century or more, living in harmless parasitism They teem with spirochetes and are highly infec- with its host. tious. They may appear anywhere, although 95 At its worst, it is a prolonged infectious poly- percent are genital. They have been noted to vasculitis with immunopathologic overlay; at best, occur in or around the mouth, anus, breast, rec- it is an inconvenient or embarrassing blood test tum, cervix and colostomy stoma. Untreated, they result. heal spontaneously. In the overwhelming majority of cases, the Secondary. Generally this stage is clinically disease is transmitted by sexual activity. Trans- manifest within six months of exposure. The mission of the treponeme can occur through chancre or chancres may still be present but are mucous membranes, minutely abraded skin or, usually healing. This stage has protean manifes- some believe, by way of normal hair follicles. En- tations, but four major syndromes have been tering through the dermis, the spirochetes seed seen: (1) a constitutional, flu-like illness; (2) rapidly into the lymphatics and from there enter the bloodstream to disseminate throughout the CA body. Invading the perivascular lymphatics of a i primory & secondary multiple organs, the treponemes stimulate L.L _J cellular inflammatory response to granulomata |24% Late relapse formation and proliferation in the intima of the 15% Benign 10% Cardiovac. 8% Neurosyphilis vasa vasorum. The resulting decrease or loss of .deUt(dinic= = (clnical blood supply by tissues served by the injured ves- laz{teantat the acute sels may lead to necrosis. Eventually, 0 5 10 15 20 25 30 inflammation is replaced by fibrous scar tissue. YEARS AFTER INFECTION T pallidum organisms may episodically, in the Figure 1.-Results of studies of the course of untreated early stages of disease, break out again as spiro- syphilis in 2,000 patients. chetemia. Or they may assume an intracellular sequestration in multiple tissues, protected by the cell, some think, from antibodies and antibacterial TABLE 1.-The Stages of Syphilis agents.4 ACQUIRED SYPHILIS The antibody response to the treponemes may Primary Immune-deposit dis- Secondary be itself immunopathologic. Latent ease in syphilis so far has been documented in Early latent the kidneys.5-7 Late latent Tertiary In later stages, tissue hypersensitivity to T pal- Late benign syphilis lidum may become prominent, including prolifera- Cardiovascular syphilis tive fibrosis with hypertrophic masses, reactive Neurosyphilis Meningovascular syphilis tissue and necrosis-that is, formation of gum- Aseptic meningitis mata. Tabes dorsalis Preataxic Stages of Syphilis Ataxic Paralytic Beginning in 1891, Boeck in Oslo studied 2,000 General paresis of the insane patients with untreated syphilis to determine the Latent neurosyphilis natural course of the disease. Studies of this popu- CONGENITAL SYPHILIS THE WESTERN JOURNAL OF MEDICINE 425 SYPHILIS generalized lymphadenopathy with or without late-stage involvement are tabes dorsalis and the splenomegaly; (3) rash, involving skin and mu- general paresis of the insane. Active meningovas- cous membranes, and (4) visceral involvement, cular syphilis is more commonly associated with such as hepatitis, nephritis, osteitis or gastritis. secondary than with tertiary involvement. The secondary stage is also infectious and, like the primary, will resolve without therapy. Conigeniital Syphilis Lcatetnt. Latent syphilis is hidden. By definition, A child born to a syphilitic mother may acquire there are no clinical manifestations. the disease by transplacental transmission of T Early latent is that period, generally two to pallidlum. Because the organism does not cross four years from infection, during which spon- the placental barrier until the 16th to 19th week, taneous clinical relapse may occur, with resump- early detection and therapy of the pregnant tion of spirochetenmia and, usually, a secondary woman is effective. 12, l3 lues-like condition. During the four years follow- In untreated women, 25 percent of infected ing inoculation, the victim remains an epidemio- infants die in utero. Another 25 percent will die logic threat because secondary lues is infectious. shortly after birth. In about 40 percent of the In almost a quarter of untreated patients this infants that survive-if untreated-symptomatic clinical recrudescence will occur. syphilis will occur, with developmental retarda- Laite ltatent is that stage of the disease after the tion, ocular, dental, neurological, bony, articular fourth year from infection when the victim is no or visceral lesions all possible.14 longer contagious but remains host to living treponemes. Review of Systems In two thirds of untreated people, spirochetes It has been said that syphilis, the great imitator, and host will live amicably togetlher until the can look like anything. But if that is true, a patient dies of other causes. In about a third, clinician can have no discrete reason to suspect however, the organism will continue to act upon syphilis in any single circumstance. It must, then, the host to cause a variety of mischief-or, ter- be kept as a differential diagnosis to be considered tiary syphilis.8-10 in almost all cases. To illustrate this, it is useful Tertiary. Late benzigni syphilis occurs in about to approach the disease from the point of view of 15 percent of untreated victims. Gummata are its systems, as one would do in ordinary history and cardinal feature. A gumma seems to represent a physical examination, rather than reviewing the proliferative reaction in which, though the pa- constellation of symptoms and signs in each stage; tient appears to be in a latent state, there is the latter all tend to merge and cross over anyway.

Load more