Incidental Inguinal Tubercular Lymphadenitis in a female of Androgen Insensivity Syndrome- A rarest of rare presentation

Androgen insensitivity syndrome (AIS) is a rare X-linked recessive disorder occurring in phenotypic women with male genotype (46, XY) resulting due to mutation in the X chromosome. On the other hand, tuberculosis (TB) is a chronic granulomatous infection caused by Mycobacterium tuberculosis, an acid...

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Main Authors: Paridhi, Shalini Bahadur, Madhuvan Gupta, Shalini Shukla, Shivani Kalhan, Deepak Goel, Deeksha Singh, Vivek Gupta
Format: Article
Language:English
Published: Association of Clinical Pathologists of Nepal 2023-07-01
Series:Journal of Pathology of Nepal
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Online Access:https://www.nepjol.info/index.php/JPN/article/view/51477
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Summary:Androgen insensitivity syndrome (AIS) is a rare X-linked recessive disorder occurring in phenotypic women with male genotype (46, XY) resulting due to mutation in the X chromosome. On the other hand, tuberculosis (TB) is a chronic granulomatous infection caused by Mycobacterium tuberculosis, an acid-fast bacillus. We report a case of a 22-year-old unmarried female presenting with bilateral inguinal pain and primary amenorrhea, which, on comprehensive evaluation, revealed complete female external genitalia and intra-abdominal testes. The karyotype was 46 XY, hence a diagnosis of complete AIS was made. One of the ‘probable testes’ removed surgically was an inguinal lymph node with tuberculous lymphadenitis. Isolated inguinal tuberculous lymphadenopathy, by itself, is rare. Co-occurrence of Complete AIS and inguinal tuberculous lymphadenopathy is a highly unusual and rare association that has not been documented in the literature reviewed.
ISSN:2091-0797
2091-0908