Syphilis is a chronic infectious disease caused by Treponema pallidum, classified as a sexually transmitted disease. It is characterized by a prolonged and insidious course with diverse symptoms, which may remain latent for years or even a lifetime. In the early stages, the condition predominantly affects the skin and mucous membranes, while the later stages may involve the heart, central nervous system, bones, liver, spleen, and other organs. Congenital syphilis is transmitted in utero, while acquired syphilis is predominantly transmitted through direct sexual contact. It can also spread through kissing, sharing eating utensils, damaged skin or mucosa, blood transfusion, or breastfeeding.
The pathogen enters through the skin or mucosa, progressing through three distinct stages: the primary stage (chancre), the secondary stage (syphilitic rash), and the tertiary stage (gummas).
Clinical Features
Treponema pallidum can invade any organ, resulting in a wide variety of clinical manifestations.
Nasal Syphilis
Congenital nasal syphilis generally presents between the ages of 3 and puberty. In addition to nasal deformities like saddle nose caused by gummas damaging the nasal septal framework, associated features may include Hutchinson’s triad (interstitial keratitis, notched teeth, and sensorineural hearing loss or labyrinthitis) and ichthyosis-like skin changes. Acquired nasal syphilis is rare in the primary and secondary stages but more commonly seen in the tertiary stage. Gummas may lead to nasal septum and hard palate perforation, saddle nose deformity, syphilitic osteitis, and mucosal atrophy following gumma resolution. Symptoms may include localized swelling, pain, and fetid nasal discharge with crusting, as well as osteitic symptoms. Differential diagnosis should take into account nasal tuberculosis, leprosy, rhinoscleroma, and malignancies.
Systemic anti-syphilis treatment forms the cornerstone of management, with local therapy involving agents like 0.5% streptomycin solution for nasal irrigation or 30% trichloroacetic acid for ulcer cauterization.
Pharyngeal Syphilis
Primary pharyngeal syphilis is rare and typically presents as a unilateral tonsillar chancre with ipsilateral cervical lymphadenopathy, which is firm and non-tender. Secondary pharyngeal syphilis may manifest approximately 2 months after the chancre as a scarlet fever-like pharyngitis with mucosal erythema and tonsillar swelling. Round or oval mucosal patches of varying sizes often appear in the oral and pharyngeal mucosa. These lesions are infiltrative, grayish-white in color, and may be accompanied by generalized lymphadenopathy and diffuse skin rash. Tertiary syphilis develops years after the initial infection and may be characterized by gummas, which resolve to form ulcers and eventually scars. This may result in hard palate perforation, pharyngeal adhesions, strictures, and occlusive deformities.
Laryngeal Syphilis
Laryngeal syphilis is uncommon. Congenital laryngeal syphilis generally develops between infancy and adolescence, while acquired cases are more frequently observed in middle-aged adults. Primary laryngeal syphilis is exceedingly rare but may appear as chancres in the epiglottis. Secondary laryngeal syphilis resembles catarrhal laryngitis. Tertiary laryngeal syphilis is classified into four types: gumma type, ulcerative type, perichondritis and chondritis type, and scar and adhesion type.
Otic Syphilis
Early congenital syphilis typically manifests within the first 1–2 years of life and may result in deafness or deaf-muteness due to meningitis, neuritis, otitis media, or labyrinthitis. Late congenital syphilis often presents between 6 and 10 years of age, commonly as multi-gummatous lesions of the temporal bone causing labyrinthitis and total deafness. Labyrinthine destruction may lead to labyrinthine fistula or stapes footplate loosening due to softening of the annular ligament, producing a positive Hennebert’s sign (a positive fistula test with a structurally normal middle ear). The symptoms of acquired inner ear syphilis resemble those of late congenital syphilis and may include facial nerve paralysis and labyrinthitis.
Diagnosis
The diagnosis is typically based on a history of syphilis exposure, family and personal medical history, combined with clinical symptoms, signs, and serological testing.
Treatment
Antisyphilitic Therapy
Penicillin remains the first-line treatment, while erythromycin is an alternative for penicillin-allergic individuals.
Symptomatic Treatment
Local care involves cleansing the lesions with solutions such as normal saline, boric acid, furacilin, or hydrogen peroxide, and maintaining cleanliness. Scar deformities may be addressed through reconstructive surgery.