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Description

Nonvenereal endemic syphilis or Bejel is a chronic skin and tissue disease caused by infection by the endemicum subspecies of the spirochete Treponema pallidum. Bejel is one of the "endemic treponematoses", a group that also includes yaws and pinta. Typically, endemic trepanematoses begin with localized lesions on the skin or mucous membranes. Pinta is limited to affecting the skin, whereas bejel and yaws are considered to be invasive because they can also cause disease in bone and other internal tissues.[1][2][3]The diagnosis is based on medical history and travel history. It is treatable with penicillin or other antibiotics.[2]

Presentation

Bejel usually begins in childhood as a small patch on the mucosa, often on the interior of the mouth, followed by the appearance of raised, eroding lesions on the limbs and trunk. Periostitis (inflammation) of the leg bones is commonly seen, and gummas of the nose and soft palate develop in later stages.[3][4][2]

Causes

Although the organism that causes bejel, Treponema pallidum endemicum, is morphologically and serologically indistinguishable from Treponema pallidum pallidum, which causes venereal syphilis, transmission of bejel is not venereal in nature, generally resulting from mouth-to-mouth contact or sharing of domestic utensils, and the courses of the two diseases are somewhat different.[5][2][3]

Mechanism

As to the pathophysiology of Bejel we find it is driven by the invasive nature of the pathogen Treponema pallidum subsp. endemicum, which penetrates mucous membranes and achieves hematogenous dissemination while evading the immune system . The progression through primary/secondary stages reflects the immune systems inability to clear the multiplying spirochetes. Ultimately the destructive gummas characteristic of the late stage are not caused by the bacterias direct toxicity, but by delayed-type hypersensitivity reaction to persistent treponemal antigens, which results in granuloma formation, and tissue disfigurement.[6][7][8]

Diagnosis

The diagnosis of bejel is based on the travel history of the patient as well as laboratory testing of material from the lesions (dark-field microscopy). The responsible spirochaete is readily identifiable on sight in a microscope as a treponema. The following is also done:[9][2] FTA-ABS(fluorescent treponemal antibody absorption) test and PCR.

Differential diagnosis

As to the differential diagnosis in the affected individual we find the following:[2] Acquired syphilis, Pinta and Yaws.

Treatment

Nonvenereal endemic syphilis is treatable with penicillin or other antibiotics(azithromycin and doxycycline), resulting in a complete recovery.[2][9]

Epidemiology

Bejel is mainly found in arid countries of the eastern Mediterranean region and in West Africa, where it is known as sahel. (Sahel disease should be distinguished from "Sahel", the geographical band between the Northern Sahara and Southern Sudan.[10])[9][2]

History

In terms of the history of Bejel disease we find that it was studied and classified during the 19th and 20th centuries, as doctors stationed in the Middle East and North Africa began to investigate whether it was a local variant of syphilis or a separate disease. This work led to its recognition as a distinct, non-venereal treponematosis.[11]

Society and culture

In 19th-century Russia, physicians distinguished between the two forms: endemic syphilis which was associated with rural life, poor hygiene, and nonvenereal contact. While venereal syphilis was associated with city life, moral decline, and sexual transmission.[12][9]

References

  1. Mitjà O, Šmajs D, Bassat Q (2013). "Advances in the diagnosis of endemic treponematoses: yaws, bejel, and pinta". PLOS Neglected Tropical Diseases. 7 (10): e2283. doi:10.1371/journal.pntd.0002283. PMC 3812090. PMID 24205410.
  2. 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 "Bejel - Symptoms, Causes, Treatment | NORD". nord. Archived from the original on 24 August 2019
  3. 3.0 3.1 3.2 "Bejel, Pinta, and Yaws - Infectious Diseases". MSD Manual Professional Edition. Retrieved 5 December 2025.
  4. Albert, Daniel M.; Miller, Joan W.; Azar, Dimitri T.; Blodi, Barbara A. (27 February 2008). Principles and Practice of Ophthalmology E-Book. Elsevier Health Sciences. p. 4781. ISBN 978-1-4377-2111-9.
  5. Antal GM, Lukehart SA, Meheus AZ (January 2002). "The endemic treponematoses". Microbes and Infection. 4 (1): 83–94. doi:10.1016/S1286-4579(01)01513-1. PMID 11825779.
  6. Knoop, Floyd C. (2007). "Disease Caused by Treponema pallidum, Nonvenereal Treponematoses, and Leptospira". XPharm: The Comprehensive Pharmacology Reference: 1–4. doi:10.1016/B978-008055232-3.60894-3. ISBN 978-0-08-055232-3. Retrieved 1 December 2025.
  7. "Syphilis and Treponematosis: Background, Etiology, Pathophysiology". eMedicine. 28 October 2025. Retrieved 4 December 2025.
  8. Schaechter, Moselio; Engleberg, N. Cary; DiRita, Victor J.; Dermody, Terence (2007). Schaechter's Mechanisms of Microbial Disease. Lippincott Williams & Wilkins. pp. 263–264. ISBN 978-0-7817-5342-5. Retrieved 5 December 2025.
  9. 9.0 9.1 9.2 9.3 Marks, Michael; Solomon, Anthony W; Mabey, David C (October 2014). "Endemic treponemal diseases". Transactions of the Royal Society of Tropical Medicine and Hygiene. 108 (10): 601–607. doi:10.1093/trstmh/tru128. PMC 4162659. PMID 25157125.
  10. "Wayback Machine" (PDF). www.unhcr.org. Archived (PDF) from the original on 6 September 2025. Retrieved 5 December 2025.
  11. Kozma, Liat (2017). "Between Colonial, National, and International Medicine: The Case of Bejel". Bulletin of the History of Medicine. 91 (4): 744–771. doi:10.1353/bhm.2017.0080. ISSN 1086-3176. PMID 29276190.
  12. Engelstein, L. (1986). "Syphilis, historical and actual: cultural geography of a disease". Reviews of Infectious Diseases. 8 (6): 1036–1048. doi:10.1093/clinids/8.6.1036. ISSN 0162-0886. PMID 3541123.