Advertisement

A Myriad of Scleroma Presentations: The Usual and Unusual

  • Samy ElwanyEmail author
  • Hesham Abdel Fattah
  • Zeyad Mandour
  • Ahmed Soliman Ismail
  • Mostafa Abdelnabi
Original Paper

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.

Keywords

Rhinoscleroma Granuloma Nose Klebsiella Tracheostomy 

Notes

Compliance with ethical standards

Conflict of interest

All authors declare that they have no competing interests.

References

  1. 1.
    Hoffman EO, Harkin JC. The Mikulicz cell in rhinoscleroma. Am J Pathol. 1973;73:147–53.Google Scholar
  2. 2.
    Umphress B, Raparia K. Rhinoscleroma. Arch Path Lab Med. 2018;142:1533–6.CrossRefPubMedGoogle Scholar
  3. 3.
    El Mofty A, Imam A, Botros G, Hamiltom PK, Floyd T. Scleroma in Egypt. Ann Otol Rhinol Laryngol. 1954;63:1031–6.CrossRefGoogle Scholar
  4. 4.
    Gafaar HA, Gafaar AH, Nour YA. Rhinoscleroma: an updated experience through the last 10 years. Acta Otolaryngol. 2007;131:440–6.CrossRefGoogle Scholar
  5. 5.
    Murphy KJ. Scleroma of the trachea associated with Pseudomonas pyocyanea. Thorax. 1966;21:355–8.CrossRefPubMedPubMedCentralGoogle Scholar
  6. 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. 7.
    Chatterji P. Scleroma of the antrum and ethmoid. J Laryngol Otol. 1969;83:917–23.CrossRefPubMedGoogle Scholar
  8. 8.
    Badrawy B, Safwat F, Fahmy S. Affection of cervical lymph nodes in rhinoscleroma. J Laryngol Otol. 1974;88:261–9.CrossRefPubMedGoogle Scholar
  9. 9.
    Hara JH, Lonser ER. Scleroma-Masquerading as intracranial meningioma. Arch Otolaryngol. 1964;79:742–5.Google Scholar
  10. 10.
    Bahri HC, Bassi NK, Rohatgi MS. Scleroma with intracranial extension. Ann Otol. 1972;81:856–9.Google Scholar
  11. 11.
    Singh A. Malignant changes in scleroma. Laryngoscope. 1972;82:444–6.CrossRefPubMedGoogle Scholar
  12. 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. 13.
    Avery RK, Salman SD, Baker AS. Rhinoscleroma treated with ciprofloxacin: a case report. Laryngoscope. 1995;105:854–6.CrossRefPubMedGoogle Scholar
  14. 14.
    Badrawy R. The uvula sign in scleroma of the nasopharynx. Ann Otol Rhinol Laryngol. 1965;74:441–4.CrossRefPubMedGoogle Scholar
  15. 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.CrossRefPubMedGoogle Scholar
  16. 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. 17.
    Toppzada HH, Gafaar H, El-Ghazawi I. The role of plasma cells in rhinosc;eroma. ORL. 1977;39:1–13.CrossRefGoogle Scholar
  18. 18.
    Gafaar H, Harada Y. Rhinoscleroma: a scanning electrom microscopic study. ORL. 1976;38:350–7.CrossRefGoogle Scholar
  19. 19.
    Miller RH, Shulman JB, Canalis RF, Ward PH. Klebsiella rhinoscleromatis: a clinical and pathogenic enigma. Otolaryngol Head Neck Surg. 1979;87:212–21.CrossRefPubMedGoogle Scholar
  20. 20.
    Badrawy R. The uvula sign in scleroma of the nasopharynx. Ann Otol. 1965;74:441–4.Google Scholar
  21. 21.
    Soni NK. Scleroma of the lower respiratory tract: a bronchoscopic study. J Laryngol Otol. 1994;108:484–5.CrossRefPubMedGoogle Scholar
  22. 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.CrossRefGoogle Scholar
  23. 23.
    Amoils CP, Shindo ML. Laryngotracheal manifestations of rhinoscleroma. Ann Otol Laryngol. 1996;106:336–40.CrossRefGoogle Scholar
  24. 24.
    Gaafar HA. Endoscopy of the lower respiratory tract scleroma. Endoscopy. 1983;15:297–9.CrossRefPubMedGoogle Scholar
  25. 25.
    Badrawy R. Dacryoscleroma (scleroma of the lachrymal passages). An Otol Rhionl Laryngol. 1962;71:247–54.CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of OtolaryngologyAlexandria UniversityAlexandriaEgypt

Personalised recommendations