Skip to main content

Advertisement

Log in

A Myriad of Scleroma Presentations: The Usual and Unusual

  • Original Paper
  • Published:
Head and Neck Pathology Aims and scope Submit manuscript

Abstract

To prospectively study a series of scleroma patients with a focus on socioeconomic status, clinical presentation, pathology, microbiology, and treatment. A total of 52 patients diagnosed with scleroma were included in the study. Demographic data of the patients were collected and clinical, radiographic, and histopathologic findings were analyzed. Biopsies from atypical patients were sent for electron microscopic examination. Patients were treated both medically and surgically with clinical follow-up to determine outcomes and the incidence of recurrence. Fifty-two patients were included in the study. There were 23 males (44.2%) and 29 females (55.8%). The highest incidence of scleroma was identified in individuals from poor, rural areas in the third to fourth decades of life. The nose was affected in all cases. Other affected sites included the nasopharynx, soft and hard palate, larynx and trachea, lacrimal passages, skin, and gingiva. Some patients had atypical presentations. Complete follow-up of all patients was hindered by non-compliance with clinic visits and the long duration of medical treatment. Recurrence or relapse of the granulomatous lesions after initial improvement occurred in 11 patients (21.2%) within 1 to 3 years. Scleroma is a stubborn, chronic, granulomatous bacterial disease. The diverse presentations may mimic neoplasms and other granulomatous conditions. We present cases with unusual presentations and demonstrate the transmission electron microscopic features of these lesions. Furthermore, we confirm the importance of achieving full eradication of the etiologic bacilli to prevent recurrent disease.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6

Similar content being viewed by others

References

  1. Hoffman EO, Harkin JC. The Mikulicz cell in rhinoscleroma. Am J Pathol. 1973;73:147–53.

    Google Scholar 

  2. Umphress B, Raparia K. Rhinoscleroma. Arch Path Lab Med. 2018;142:1533–6.

    Article  CAS  PubMed  Google Scholar 

  3. El Mofty A, Imam A, Botros G, Hamiltom PK, Floyd T. Scleroma in Egypt. Ann Otol Rhinol Laryngol. 1954;63:1031–6.

    Article  Google Scholar 

  4. Gafaar HA, Gafaar AH, Nour YA. Rhinoscleroma: an updated experience through the last 10 years. Acta Otolaryngol. 2007;131:440–6.

    Article  Google Scholar 

  5. Murphy KJ. Scleroma of the trachea associated with Pseudomonas pyocyanea. Thorax. 1966;21:355–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  6. Tapia Acana R. Endoscopy of the air passages with special reference to scleroma. Ann Otol Rhinol Laryngol. 1973;982:765–9.

    Google Scholar 

  7. Chatterji P. Scleroma of the antrum and ethmoid. J Laryngol Otol. 1969;83:917–23.

    Article  CAS  PubMed  Google Scholar 

  8. Badrawy B, Safwat F, Fahmy S. Affection of cervical lymph nodes in rhinoscleroma. J Laryngol Otol. 1974;88:261–9.

    Article  CAS  PubMed  Google Scholar 

  9. Hara JH, Lonser ER. Scleroma-Masquerading as intracranial meningioma. Arch Otolaryngol. 1964;79:742–5.

    Google Scholar 

  10. Bahri HC, Bassi NK, Rohatgi MS. Scleroma with intracranial extension. Ann Otol. 1972;81:856–9.

    CAS  Google Scholar 

  11. Singh A. Malignant changes in scleroma. Laryngoscope. 1972;82:444–6.

    Article  CAS  PubMed  Google Scholar 

  12. Atef A, Talaat N, El Farouk A, Qotb M, Fathi H, Ilia S. Sinus involvement of rhinoscleroma: a cause of antibiotic resistance and early recurrence after medical treatment. Egypt J Otolaryngol. 2012;28:8–11.

    Google Scholar 

  13. Avery RK, Salman SD, Baker AS. Rhinoscleroma treated with ciprofloxacin: a case report. Laryngoscope. 1995;105:854–6.

    Article  CAS  PubMed  Google Scholar 

  14. Badrawy R. The uvula sign in scleroma of the nasopharynx. Ann Otol Rhinol Laryngol. 1965;74:441–4.

    Article  CAS  PubMed  Google Scholar 

  15. Sedano HO, Carlos RB, Koutlas IG. Respiratory scleroma: a clinicopathologic and ultrastructural study. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1996;81:665–71.

    Article  CAS  PubMed  Google Scholar 

  16. Ahmed A, El-Badawy Z, Mohamed I, Abdelhameed W. Rhinoscleroma: a detailed histopathological diagnostic insight. Int J Clin Exp Path. 2015;8:8438–45.

    Google Scholar 

  17. Toppzada HH, Gafaar H, El-Ghazawi I. The role of plasma cells in rhinosc;eroma. ORL. 1977;39:1–13.

    Article  Google Scholar 

  18. Gafaar H, Harada Y. Rhinoscleroma: a scanning electrom microscopic study. ORL. 1976;38:350–7.

    Article  Google Scholar 

  19. Miller RH, Shulman JB, Canalis RF, Ward PH. Klebsiella rhinoscleromatis: a clinical and pathogenic enigma. Otolaryngol Head Neck Surg. 1979;87:212–21.

    Article  CAS  PubMed  Google Scholar 

  20. Badrawy R. The uvula sign in scleroma of the nasopharynx. Ann Otol. 1965;74:441–4.

    CAS  Google Scholar 

  21. Soni NK. Scleroma of the lower respiratory tract: a bronchoscopic study. J Laryngol Otol. 1994;108:484–5.

    Article  CAS  PubMed  Google Scholar 

  22. Afaro-Monge JM, Fernandez-Espinosa J. Scleroma of the lower respiratory tract: case report and review of the literature. J Laryngol Otol. 1994;108:161–3.

    Article  Google Scholar 

  23. Amoils CP, Shindo ML. Laryngotracheal manifestations of rhinoscleroma. Ann Otol Laryngol. 1996;106:336–40.

    Article  Google Scholar 

  24. Gaafar HA. Endoscopy of the lower respiratory tract scleroma. Endoscopy. 1983;15:297–9.

    Article  CAS  PubMed  Google Scholar 

  25. Badrawy R. Dacryoscleroma (scleroma of the lachrymal passages). An Otol Rhionl Laryngol. 1962;71:247–54.

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Samy Elwany.

Ethics declarations

Conflict of interest

All authors declare that they have no competing interests.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Elwany, S., Fattah, H.A., Mandour, Z. et al. A Myriad of Scleroma Presentations: The Usual and Unusual. Head and Neck Pathol 14, 588–592 (2020). https://doi.org/10.1007/s12105-019-01075-5

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12105-019-01075-5

Keywords

Navigation