Syphilis

1. Syphilis

2. Introduction • A chronic systemic infectious disease, transmitted during sexual intercourse or other intimate contact • From a pregnant woman to her fetus in utero or during birth • The causative agent:a spirochete, Treponema palidum, never successfully cultured on artificial media and never taking Gram-staining

4. Collection of specimen for dark-field or DFA-TP test • Penile ulcer after cleaning with gauze • Squeezing the ulcer to obtain exudate • Touching coverslip to ulcer to obtain fluid for dark-field examination

5. Transmission electron micrograph of Treponema pallidum subspecies pallidum in tissue

6. Etiology • In the United States • A high in 1947 • Sharp decline over the following 10 years • Gradually increase over each succeeding decade to the present • Earlier age pf first intercourse • Large numbers of lifetime partners • High prevalence in homosexual men • Decrease of federal dollars for control programs • Diversion of control efforts to other STD problems

7. Rates of primary and secondary syphilis in men and women compared with the rates of congenital syphilis. Numbers of cases of congenital syphilis are a highly sensitive indicator of the amount of infectious syphilis in a population and the need for control activities.

8. Clinical manifestations • Untreated syphilis; • a chronic disease, spread throughout the body hematogenously • In virtually every organ system • The infectious, clinically manifest stages of the disease – primary and secondary syphilis – only transient events • Latency; no clinical signs or symptoms, T. pallidum seen in some tissues

9. Years after infection 10 20 30 40 50 • The clinical course of untreated syphilis Late benign Cardiovascular Neurosyphilis Congenital

10. Serologic Onset Duration Months after Infection 1 2 3 4 5 6 7+ FTA-ABS MHA-TP Non-Treponemal • Correlation of serologi an cutaneous changes in untreated syphilis Cutaneous Onset Duration Months after Infection 1 2 3 4 5 6 7+ Chancre rash

11. Primary Syphilis • The chancre • the first clinical manifestation • Average of 3 weeks after infection (10 to 30 days) • At the site of treponemal invasion, usually on or near the genitals (of course on any skin or mucous membrane) • Single and painless, unless superinfected • Missing if on an inaccessible region (the cervix, pharynx, or rectum) • Nontender regional adenopathy • 2 to 6 weeks persisting, If untreated, and healing without scarring

12. Typical syphilic chancre of the coronal sulcus. This early asymptomatic chancre in the coronal sulcus shows characteristic induration and a “clean” base. Dark-field examination will almost always be positive, if no medication has been given or applied topically

13. Primary syphilis (continued) • Relapsing chancre at the same site, occasionally • The Typical chancre; indurated, clean base, and rolled edges • Secondary infection with bacteria or even herpesviruses • making ulcer somewhat atypical • Confusion with chancroid granuloma inguinale or with herpes • The labia and fourchette; the most typical areas for chancres to occur in women • Perianal, anal, or rectal chancres; in homosexual men and women with the history of analy intercourse

14. Large, indurated primary chancre of the penile shaft. This penile chancre has been present for several weeks, but it is still painless, and large. The induration produces a cartilaginous quality.

15. Atypical penile chancre. This chancre appears atypical, because it has come secondarily infected with bacteria. Dark-field examination may be difficult because of the presence of nonpathogenic treponemes.

16. Syphilic chancre of the labia majora. The lower labia and fourchette are the most common locations of primary chancres in women. This lesion was completely asymptomatic. • Perianal syphilis chancre. A well-developed anal chancre demonstrating the buttolike morphology typical of syphilis, however, this same appearance can be seen in granuloma injuinale and in chancroid. • Multiple chancres of primary syphilis. Multiple primary chancres are not uncommon in primary syphilis. They occur most frequently on the penis and vulva.

17. Primary syphilis (continued) • Healing lesions; problems in diagnosis • In their later stages; dark-field negative • Adenopathy; not prominent • Acquired syphilis in infants and children • extragenital sites, such as fingers or oral cavity

18. Healing chancre. Primary chancre heal spontaneously, as seen here in this almost revolved penile chancre. • Vulvar chancre in a child. A painless ulcer found on the genitals of a child should always raise the possibility of syphilis, but also may be acquired through nonsexual means.

19. Digital syphilic chancre. Occupational exposure of health care workers may be the cause of chancres on the hands. • Chancre of the tongue. Dark-field examination of mouth lesions may not be reliable due to the presence of saprophytic spirochetes. However, the direct FA test is useful in this situation.

20. Secondary syphilis • Onset of the secondary syphilis; from 6 weeks to 6 months after infection, if not treated • Primary chancre may still be present • Spirochetes in the bloodstream and to most tissues and organs • Multiplication • Fever, malaise, headache, sore throat, arthralgias and anorexia • Generalized adenopathy in more than half of patients; Hepatomegaly, and occasionally splenomegaly • Leukocytosis, anemia, and elevation of the erythrocyte sedimentation rate

21. Oval hyperpigmented macules of the trunk and extremities in early secondary syphilis. The eruption was generalized, but not readily visible, and therefore unnoticed by the patient.

22. Secondary syphilis (continued) • Syphilic hepatitis; mild derangement of liver enzymes and a markedly elevated alkaline phosphatase • A rash (syphilid) in about 75 % of patients, extremely variable in appearance; • symmetrical discrete erythematous, brown or hyperpigmented macules → the earliest generalized syphilid • Commonly from the trunk; becoming enlarged and annular • scaling, and pruritus; absent

23. Secondary syphilis (continued) • Some of the macules; thickened and papular • Macular syphilids may coexist with the papular forms • Papular syphilids more common than macular eruptions; easier to see(?) • If untreated for several weeks; the papules may develop a dry, thin collarette of scale, which peels off easily.

24. Early papular syphilis. The lack of scale suggests that this is an early form of papular syphilis. Erythema and firmness of the papules on palpation are characteristic. • Macular and papulosquamous forms of syphilis coexsisting in syphilis of 1 month’s duration. This eruption was completely asymptomatic. • Papular secondary syphilis. The generalized wrythematous papules are quite obvious to both patient and physician.

25. Populosquamous secondary syphilis • The annular scaling seen here had been present for several weeks. It is quite common. • A close-up view of the characteristic colarette of scale and hyperpigmentation seen in untreated secondary syphilis

26. Secondary syphilis (continued) • Macular and papular syphilids in the palms and soles; distinguishable from other dermatoses • Many varieties of papular syphilids • Papulosquamous • Annular • Lenticular • Syphilis cornee • Psoriasiform • framboesiform

27. Varieties of papular syphilids • Palmar and plantar papulosquamous secondary syphilis • Papulosquamous syphilids are typically flat papules, which are red, indurated, and slightly scaly. Lesions may be limited to the genital region. • Annular syphilids are florid annular scaly plaques, some with a targetoid hyperpigmented center. • Smooth firm pea-sized brown papules characterize the lenticular form of secondary syphilis. • Large and small psoriasiform plaques with thick scale and an irregular shape in late secondary syphilis • Verrucous and eroded (framboesiform) lesions of late secondary syphilis in the coronal sulcus

28. Condylomata lata • Typical condylomata lata on the labia and perineum are most gray plaques and papules • Flat, broad-based dark-field positive plaques are seen in the folds of the foreskin

29. Condylomata lata. Perianaly ondylomata detected in a patient, who sought help because of a palmar rash

30. Condylomata lata. Unusually verrucous condylomata lata resembling condylomata acuminata in a patient who presented with generalized macular eruption

31. Broad-based moist, dark-field positive condylomata on the thigh. Note the other erosive lesions of secondary syphilis on the penile shaft.

32. Split papules, seen here on the posterior auricular fold, may also be present at the angles of the mouth.

33. Mucous patches in secondary syphilis • Serpiginous mucous patches on the labial mucosa and tongue were the presenting sign of syphilis in this patient • Mucous patches are seen on the tongue. • As described in B.

34. Alopecia in secondary syphilis • The patchy or “moth-eaten” alopecia may not be noticed by the patient, but can be found by the alert examiner. • Occasionally a more diffuse alopecia accompanies secondary syphilis

35. Differential Diagnosis • The eruption of secondary syphilis • Almost infinitely varied • Mimicking many common dermatoses • The brown-red hyperpigmentation and line scale vs. characteristically oval, slightly scaly, brown-red eruptions of pityriasis; generalized adenopathy is absent and serologic tests for syphilis are negative in pityriasis rosea • Hyperpigmented oval plaques of secondary syphilis on the upper back vs. a common form of hyperpigmented tinea versicolor; adherent KOH-positive scales in tinea versicolor

36. Differential diagnosis in secondary syphilis • Early papulosquamous form of syphilis • Pityriasis rosea

37. The generalized macular and papular eruptions of syphilis vs. generalized scabies; pruritus in scabies, pronounced, and the lesions frequently excoriated • The scattered papulosquamous eruptions of secondary syphilis vs. the guttae variety of psoriasis; Psoriatic scaling frequently quite thick and adherent • Fairly large erythematous plaques on the penis vs. fixed drug eruption; later scaly and hyperpigmented in fixed drug eruption • Annular palmar or plantar macules or plaques in some cases of syphilis vs. the characteristic “target” or “iris” lesions of erythema multiforme; erythema multiforme usually not scaly and bullous

38. Differential diagnosis in secondary syphilis • Generalized papular form of secondary syphilis • Generalized scabies

39. Differential diagnosis in secondary syphilis • Papulosquamous secondary syphilis of the penis • Psoriasis involving the genitals

40. Differential diagnosis in secondary syphilis • Erythematous penile plaques of secondary syphilis • Fixed drug eruption

41. Differential diagnosis in secondary syphilis • Targetoid annular papulosquamous secondary syphilis of the palms • Erythema multiforme on the palm

42. Differential diagnosis in secondary syphilis • Papular secondary syphilis of the plantar surface of the foot • Pityriasis lichenoides chronica involving the leg and plantar aspect of the foot

43. Differential diagnosis in secondary syphilis • Discoid secondary syphilis of the face • Discoid lupus erythematosus

44. Latent syphilis • The period of quiescence after completion of the secondary stage of disease • No clinical manifestations • An exposure history and a positive serological test; the only way of diagnosis • Divided into early and late phases • During this period; • Relapses of secondary disease • Infection of a partner (during early latency) • Risk of transmission of the pregnant woman to her fetus • Risk decrease as latency progresses

45. Late Syphilis • Three main manifestations • Cardiovascular, gummatous, and meningovasculoar (neural) • Occurring decades after infection • The meningeal and cerebrovascular forms; as early as within a year after initial infection

46. The primary pathologic lesions of neurosyphilis. Schematic diagram showing the endarteritis of cerebral blood vessels with lymphocytic infiltration and obliteration of the vessel lumen.

47. Cardiovascular syphilis • Uncommon, today • Estimated to occur in approximately 10 % of cases of untreated syphilis • Affecting black patients more commonly than white ones • Dilation of the aortic ring with incompetence of the valve, left ventricular hypertrophy, aortic root dilation with aneurysm formation and stenosis of the coronary artery ostia

48. Cadiovascular syphilis. Syphilitic aortic aneurysm with erosion through the chest wall. • Cardiovascular syphilis. Narrowing of the coronary ostia in syphilitic aortitis

49. Late Benign Syphilis • The gummatous lesion; a severe inflammatory response to treponemal antigens (exact mechanism of pathogenesis, not known) • The active lesions; granumomas • Older lesions; extensive fibrosis • The lesions heal with deep scarring and fibrosis • Difficult to detect treponemes in gummata • Virtually any organ system; but the skin and bones most commonly • Skin lesions; nodular, noduloulcerative, or gummatous

50. Benign tertiary or gummatous syphilis • Ulcerating facial gummata such as these are now unusual in the United States although they are still common in other parts of the world • Serpiginous gummata of the forearm. Note the active border and areas of partial spontaneous resolution, with scarring