Observance of Kassowitz law-late congenital syphilis: Palatal perforation and saddle nose deformity as presenting features
A 9 year-old girl, born as the fourth child to non-consanguineous parents, was reported to our department with perforation of palate for routine Venereal Disease Research Laboratory (VDRL) screening from the ENT department. The patient had history of regurgitation of food and change of voice for the past 6 months. There was no history of trauma, loss of weight, skin lesions or fever. Also, there was no history suggestive of sexual abuse. Physical examination revealed saddle nose deformity [] and perforation of hard palate []. The size of perforation was 5-mm in diameter and the edges were regular. Ophthalmic and central nervous system examination were normal. VDRL was reactive in 1:8 dilutions and was later confirmed with Treponema pallidum hemagglutination (TPHA).\nHer parents and siblings (one elder and two younger) were screened for syphilis. Parents were VDRL reactive in 1:4 dilutions, elder sibling (female, 11 years old) was VDRL reactive in 1:8 dilutions and all were confirmed with TPHA. The two younger siblings were VDRL non-reactive. Patient's father gave a history of unprotected, premarital sexual contact, with an unknown female, 14 years back. Three weeks later he developed a genital ulcer for which he was treated and the ulcer healed without any scar. Patient's mother had six deliveries. Her first male baby was delivered 12 years back which died on the same day because of seizures. The second female baby was delivered 2 years later which also died on the 10 th day due to low birth weight. Her third female child (11 years old) is the elder sibling of the patient, who had non-development of upper central incisors []. Her fourth child was the patient. Fifth and sixth children were clinically and serologically normal []. All were home deliveries without any antenatal care.\nRoutine blood investigations, cerebrospinal fluid (CSF) analysis, Mantoux test, chest X-ray, X-ray skull and long bones were normal for the patient. Computed tomography (CT) scan of paranasal sinus (PNS) showed perforation of palate [] with bilateral maxillary and nasal polyps []. Her parents refused for biopsy of polyps. From the above history, clinical examination and investigations, diagnosis of late congenital syphilis with stigmata was made. The patient was treated with injection benzathaine penicillin 50,000 international units/kg body weight, deep intramuscular, once weekly, for 3 weeks, after test dose. The elder sibling and parents were also treated with injection benzathaine penicillin 24 lakh international units, once weekly, for 3 weeks. They were advised to come for regular follow-up.