CHAPTER 9 

The cutaneous manifestations of congenial syphilis tend to appear during the first few weeks or months of life. Syphilis is transmitted from the mother to the fetus via the placenta. The infection is therefore prenatal rather than congenital.

Skin manifestations of early congenital syphilis

1. Stigmata

   Congenital syphilis can be easily diagnosed in the presence of stigmata. Stigmata are found in young children due to destructive lesions of syphilis leading to scarring.

   These changes are known as " Hutchinson‘s triad" which include:

• Saddle nose

• Skin rhagades (radial scars)

• Corneal ulcers and opacities

• Hutchinson‘s teeth and mulberry moles.

2. Syphlitic PemphigusSymmetrical bullous eruption may be found on the palms and soles. Seropurulent fluid laden with treponemas comes out from the lesion. Later on crust covers the dull red eroded area.

3. Condyloma Lata

These lesions may affect muco- cutaneous junction.

Clinical features

Mucous membranes manifestations

Ulceration and destruction of the mucous membranes occur in the infected areas .

The mucous membrane of the nose mouth, throat and larynx are affected where muco-purulent discharge or blood stained discharge exudes from the nose. This is called "snuffles."

The infant has an aphonic cry due to involvement of the larynx.

Skin manifestations

The skin becomes red, glazed and radiating fissures may appear at the angles of the mouth, around the nares and anus. Healing of these lesions lead to formation of radial scars known as "rhagades"

Systemic Manifestations

• Old man look - due to constitutional symptoms as gastro-enteritis, pneumonia, weight loss and marasmus, which lead to wrinkled yellow brown skin.

• Syphilitic alopecia

• Scalp - the sides and back of the head show areas of hair loss, has the characteristic of "moth eaten" appearance.

• Eye lashes and eyebrows are absent.

• Nail dystrophy.

SKIN MANIFESTATIONS OF LATE CONGENITAL SYPHILIS

Late congenital syphilis causes a wide variety of signs and symptoms in untreated cases. A benign tertiary lesion is rare under the age of 5 years. A number of these are so characteristic:

1. Gumma :Gummatous ulceration of the mouth and nose where there may be perforation of the nasal septum with deformities of the nose.

   Destruction to the palate may cause regurgitation of food.

2. Stigmata : due to scarring from the early lesions.

Saddle nose

Rhagades around the mouth, nostril and anus.

Diagnosis of congenital syphilis

By the clinical picture.

Serological tests: VDRL ,TPI and FTA-ABS (IgM).

TREATMENT OF CONGENITAL SYPHILIS

It is very important to diagnose and treat the mother and contacts, as siblings. Contact -tracing should be considered.

Penicillin injection is the drug of choice.

Recommended dose for infants and children: Procaine penicillin 450,000 unit per kg body weight for 10 days.

Benzathine penicillin: may be administered for congenital syphilis as a single intramuscular injection of 50,000 units per kg. body weight .

Erythrocin can be given for 2-3 weeks when there is sensitivity to penicillin.

NON VENEREAL TREPONAEMAS
YAWS

Yaws is a non-venereal systemic infectious disease that begins frequently in childhood. The course of the disease in children is more rapid than in adults.

Treponema pertenue is the causative organism that is endemic in certain tropical areas.

Etiology

The causative organism Treponema pertenue is identical morphologically with T. palladium of syphilis. Flies of the genus Hippelates transmit infections to human beings. Mild trauma and even skin abrasion may facilitate seeding of the organisms into the skin.

Clinical features

1. The early stage

   Primary lesions - primary papule or groups of papules appear within one month or more after the organism enters the skin. This primary lesion is called "frambesiform" and localized to the skin only without involvement of internal organs. Infection is non-venereal and is not transmitted congenitally.

2. Secondary lesions - these lesions appear as amber-yellow, soft, crusted frambesiform granulomatous nodules on the face and extremities. The lesions usually heal without destruction or scarring. The lesions heal with mild atrophy and some depigmentation where later these lesions become hyper pigmented. Pigmentation is severe in palmer and plantar lesions. They may not be easily diagnosed clinically and may resemble some common skin diseases.

3. Tertiary stage: this stage may appear after a long chronic course extending years after the primary stage where there is numerous relapses and spontaneous cure may occur.

4. Late tertiary stage of yaws - may end with horrible destruction of the skin and bones leading to disfiguration.

Manifestations of late tertiary stage:

• Systemic manifestations

Systemic manifestations are more severe in children. These include low-grade fever, malaise, and generalized lymphadenopathy.

Some disability may result from involvement of palms and soles. Periosteitis and rarefaction are more extreme in children.

• Skin manifestations

Skin lesions are large nodules that may reach up to 15 cm. in diameter with ulceration where healing occurs in certain areas, and new lesion extending to a new area causing more destruction. The scarring leads to atrophic and hyperpigmented areas.

Lesions of the palms and soles show extensive thickening and superficial ulcerations with vegetative annular lesions.

Absence of mucous membrane lesion in the secondary stage differentiates yaws from syphilis.

Absence of congenital transmission in yaws.

Involvements of internal organs are very rare in late tertiary stage of yaws.

Diagnosis

• Typical clinical picture.

• The presence of characteristic lesions of the palm and soles in an endemic area is diagnostic.

• Demonstration of the spirochetes by dark field.

• Positive serological test for syphilis.

Treatment

Penicillin is the drug of choice.

WHO recommended dosage for treatment of Yaws is as follows:

• Procaine penicillin in oil with aluminium monostearate (PMA ) as a single dose for children under 15 years old is 600,000 units .

• Patients above 15 years old needs 1,200,000 unit for active cases of yaws

• Latent cases and contacts need half the dose of children under 15 years old. (300,000 units)

PINTA

Pinta is a non-venereal treponemal infection called "blue stain disease."

Peculiar pigmented patches of the skin, affecting mainly colored races characterize the disease. It is caused by Treponema carateum that is indistinguishable morphologically from the organisms causing syphilis and yaws.

The disease is endemic in tropical and subtropical regions.

Modes of Infection

• Flies of the genus Hippelates transmit the infection.

• Direct contact with the patients.

• During the first stage, infected babies may infect their mothers during breast-feeding by direct contact where a breast chancre may develop.

Clinical Picture

The incubation period is similar to other treponemal infections

1. Primary stage

   This stage begins after one to several weeks from infection .

   The lesions begin as a tiny red papule on the legs and on the exposed areas that becomes elevated ill-defined erythematous plaque.

   Satellite papules may surround the primary lesion after few months and fuse together forming configuring patterns.

   No ulceration of the lesion and that characterizes Pinta from syphilis.

   STS are non-reactive in the primary stage and dark field examination may be positive.
    

2. Secondary stage

   This stage will take from 5-12 months to appear following the primary stage. The lesions may be inconspicuous and may pass without notice.

3. Late stage

   This stage appears after a very long course taking years in adults and has a very chronic course. The lesions appear on the face, head and neck as stippled discrete or localized slate blue pigmentation and replaced after a long time by depigmented spots resembling vitilligo. Hence this stage is sometimes called "dyschromic stage". The skin shows blue patches (blue pinta) and white areas (white pinta ) . The lesions may become hyperkeratotic.

   Skin gummata may end in deep ulceration and destruction of the sites involved in the skin, bones and nose, and rarely the pharynx leading to extensive deformities.

   Involvement of the internal organs and neurological manifestations are rare .

Treatment

Penicillin is the drug of choice .

The recommend dose for adults is four injections of Benzathine penicillin 1,200,000 units every four days. The blue color, the depigmented areas and the hyperkeratotic lesions begin to disappear after treatment.

BEJEL

Bejel is an endemic non venereal disease. Large percentage of the population in the endemic areas is infected before puberty. Their children may infect adults who escape infection in childhood . There is no evidence of transmission in utero.

Secondary stage: manifests with generalized papular , annular papular and papulo-squamous eruption . Mucous membrane involvement is in the form of condylomas and mucous patches which simulate other manifestations of endemic Treponema . Hoarseness of the voice and nocturnal bone pains are common and characteristic symptoms .

Latent stage : this stage manifests, with destruction of bone, tissues, systemic involvement of cardiovascular and nervous system .

Treatment

The same as other types of non-venereal treponemas.

REFERENCES

1. Boot JM, Oranje AP, Menke HE et al. congenital syphilis in the Netherlands: diagnosis & clinical features. Genitourin Med 1989; 65: 300-3.

2. Bradlaw RV. The dental stigmata of prenatal syphilis. Oral Surg Oral Med Oral Pathol 1953; 6: 147-58.

3. Browne SG. Yaws. Int J Dermatol 1982; 21: 220-3.

4. Bengali FS. Involvement of aortic valve and ascending aorta in congenital syphilis. Br J Vener Dis 1961; 37: 257-67.

5. Dunlop EMC, Wink RB. Incidence of corneal changes in congenital syphilis. Br J Vener Dis 1954; 30: 201-9.

6. Feign D, Glenn M, MacBride-Stewart G et al. Yaws in the Solomon Islands. J Trop Med Hyg 1990; 93: 52-7.

7. Harter CA, Benirschke K. Fetal syphilis in the first trimester. Am J Obstet Gynecol 1976; 124: 705-11.

8. Hackett CJ, Loewenthal LJA. Differential Diagnosis of Yaws. Monograph Series, No. 45. Geneva: WHO, 1960.

9. Mascola L, Pelosi R, Blount JH et al. Congenital syphilis revisited. Am J Dis Child 1985; 139: 57-80.

10. Medina R. Pinta. An Endemic Treponematosis in the Americas. WHO INT/VDT/204.65.

11. Noordhoek GT, Cockayne A, Schouls LM et al. A new attempt to distinguish serologically the subspecies of Treponema pallidum causing syphilis and Yaws.J Clin Microbiol 1990; 28: 1600-7.

12. Robinson RCV. Congenital syphilis. Arch Dermatol 1969; 99: 599-610.

13. Willcox RR. Njovera: an endemic syphilis of Southern Rhodesia. Lancet 1951; i: 558-60.

14. Whittet HB, Quiney RE. Nasal manifestation of yaws. J Laryngol Otol 1988; 102: 1147-9.

15. Hackett CJ, Loewenthal LJA. Differential Diagnosis of Yaws. MonographSeries, No. 45. Geneva: WHO, 1960.

16. Mascola L, Pelosi R, Blount JH et al. congenital syphilis revisited. Am J Dis Child 1985; 139: 57-80.

17. Noordhoek GT, Cockayne A, Schouls LM et al. A new attempt to distinguish serologically the subspecies of Treponema pallidum causing syphilis and Yaws.

18. WHO. Treponemal infections. Technical Report Series No. 674. Geneva: WHO,1982.

19. WHO. Expert Committee on Venereal Diseases and Treponematoses. Sixth Report. Technical Reports Series No. 736. Geneva: WHO, 1986.

TROPICAL ULCER

Tropical ulcers occur in the tropical areas during the rainy seasons. The disease is more common in school children .

Different organisms such as pyogenic organisms, syphilis , yaws and Vincent‘s angina may cause tropical ulcer.

CLINICAL FEATURES

The lesion begins as an inflammatory papule, frequently on a pre-existing abrasion or mild trauma on the legs and arms. The lesion is usually single and unilateral, meanwhile it is not uncommon to find multiple ulcers upon both legs. The papule is transformed into a vesicle, which ruptures leading to an ulcer. The ulcer may be small or large with elevated or depressed smooth, ragged and undermined edges.

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