eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > Infectious Diseases

Scrofula

Author: John E McClay, MD, Assistant Professor, Department of Otolaryngology, Division of Pediatric Otolaryngology, Children's Medical Center, University of Texas Southwestern Medical School
Coauthor(s): Michael R Lewis, MD, Associate Professor, University of Texas Health Center at Tyler
Contributor Information and Disclosures

Updated: Oct 30, 2008

Introduction

Background

Tuberculosis (TB) is the oldest documented infectious disease. In the United States, pulmonary tuberculosis (TB) accounts for most tuberculosis (TB) cases. Scrofula is the Latin word for brood sow, and it is the term applied to tuberculosis (TB) of the neck. Cervical tuberculosis (TB) is usually a result of an infection in the lymph nodes, known as lymphadenitis. Extrapulmonary tuberculosis (TB), such as scrofula, is observed most often in individuals who are immunocompromised, accounting for up to 50% of these cervical infections.

Scrofula has been known to afflict people since antiquity, and during the Middle Ages, the king's touch was thought to be curative. In modern times, surgery has played a pivotal role in the diagnosis and treatment of scrofula. Over the past several decades, however, surgical intervention has played a decreasing role because it has been fraught with persistent disease and complications. As in pulmonary tuberculosis (TB), antituberculous chemotherapy has become the standard of care for scrofula, and newer diagnostic techniques (eg, fine-needle aspiration) have replaced more invasive methods of tissue harvesting.

Today, approximately 95% of mycobacterial cervical infections in adults are caused by Mycobacterium tuberculosis and the rest are caused by atypical mycobacterium or nontuberculous mycobacterium (NTM). In children, this trend is reversed, with 92% of cases due to atypical mycobacterium. NTM was first recognized as a cause of cervical adenitis in 1956. More than 50 species have now been identified, of which one half are recognized as pathogenic. Recent statistics indicate an increase in the prevalence and isolation of cervical lymphadenitis caused by NTM, far outnumbering tuberculosis (TB) as the cause of chronic cervical adenitis in children. The distinction has both diagnostic as well as therapeutic implications. Historically, scrofula was a term used to describe tuberculosis (TB) adenitis; however, NTM adenitis is included in the following text for completeness.

Pathophysiology

M tuberculosis is an obligate aerobe, non–spore forming, slender rod. Humans are its only reservoir. Transmission is from person to person via respiratory route by inhalation of small aerosols. After a short period of replication in the lungs, silent dissemination occurs through the lymphohematogenous system to extrapulmonary sites including the cervical lymph nodes.

NTM differs from M tuberculosis in several respects: (1) person-to-person transmission generally does not occur, and (2) NTM species are ubiquitous in nature and not necessarily pathogenic or equated with disease. The oral cavity may serve as a common portal of entry because the disease primarily occurs in children who have a propensity to put contaminated objects in their mouth.

Frequency

United States

Lymphadenitis is the primary manifestation of tuberculosis (TB) in 5% of the immunocompetent population, with the cervical lymph nodes providing the site of infection in two thirds of cases. In people with human immunodeficiency virus (HIV), cervical lymphadenitis may represent one third of the total presentations. Since 1985, the first increase in incidence since 1882 has occurred in the United States because of (1) increased immigration from endemic countries, (2) rising population of those infected with HIV, (3) worsening urban social conditions, and (4) abandonment of rigid tuberculosis (TB) control programs.

Currently 2-10% of mycobacterial infections in the United States are due to other NTM.

International

In impoverished countries where tuberculosis (TB) is endemic, tuberculosis (TB) continues to be a major health concern.

Mortality/Morbidity

  • Tuberculosis (TB): The mortality rate approaches 20% with multidrug-resistant pulmonary tuberculosis (TB). Statistics are not available for isolated cervical lymphadenitis.
  • NTM: Recent statistics indicate an increase in prevalence and isolation of cervical lymphadenopathy caused by NTM. Because NTM is not generally reportable, the true incidence is difficult to determine.

Race

  • Tuberculosis (TB): Incidence is increased in indigent, Asian, Hispanic immigrant, Native American, and Eskimo populations.
  • NTM: People of all races are affected, with a white predominance.

Sex

  • Tuberculosis (TB): The female-to-male ratio is 2:1.
  • NTM: The female-to-male ratio is 1.3:1.

Age

  • Tuberculosis (TB) affects people of all ages.
  • NTM affects children aged 1-5 years.

Clinical

History

  • M tuberculosis
    • Patients report a painless, enlarging, or persistent mass.
    • Systemic symptoms include fever/chills, weight loss, or malaise in 43% of patients.
  • Nontuberculous mycobacterium
    • Chronic cervicofacial mass
    • Clinical progression of the disease
    • No constitutional symptoms
    • Poor response to conventional antibiotics
    • No history of tuberculosis (TB) exposure

Physical

  • M tuberculosis
    • Any cervical node, although anterior cervical chain is more common
    • Firm rubbery node becoming more firm and matted as disease progresses
    • Infrequently, fluctuant with draining fistula
    • Multiple masses in two thirds of patients
    • Bilateral nodes in one third of patients
  • Nontuberculous mycobacterium
    • A nontender slightly fluctuant mass is present with the overlying skin obtaining a violaceous hue. This is referred to as a cold abscess because of its lack of calor, or warmth.
    • As the lesion progresses, the skin can become adherent to the underlying mass. This stage may progress to rupture and sinus formation.

Causes

  • Cellular immunity, in particular the T-cell population, is instrumental in controlling infection. Activated T cells generate cytokines that enable tissue macrophages and monocytes to destroy the mycobacteria and form a tubercle or granuloma. Therefore, in the population with HIV, the incidence of tuberculous infection is 500 times greater than in the general population.
  • Nontuberculous mycobacterium (NTM) generally occurs in immunocompetent hosts.

More on Scrofula

Overview: Scrofula
Differential Diagnoses & Workup: Scrofula
Treatment & Medication: Scrofula
Follow-up: Scrofula
References
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References

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  5. Kennedy TL. Curettage of nontuberculous mycobacterial cervical lymphadenitis. Arch Otolaryngol Head Neck Surg. Jul 1992;118(7):759-62. [Medline].

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  22. Yuen AP, Wong SH, Tam CM, et al. Prospective randomized study of thrice weekly six-month and nine-month chemotherapy for cervical tuberculous lymphadenopathy. Otolaryngol Head Neck Surg. Feb 1997;116(2):189-92. [Medline].

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Further Reading

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Keywords

scrofula, tuberculosis, TB, cervical tuberculosis, cervical tuberculous lymphadenopathy, mycobacterial lymphadenitis, extrapulmonary tuberculosis, infectious disease, tuberculosis of the neck, neck tuberculosis, scrofula neck, lymphadenitis, antituberculous chemotherapy

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Contributor Information and Disclosures

Author

John E McClay, MD, Assistant Professor, Department of Otolaryngology, Division of Pediatric Otolaryngology, Children's Medical Center, University of Texas Southwestern Medical School
John E McClay, MD is a member of the following medical societies: American Academy of Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, and American Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Michael R Lewis, MD, Associate Professor, University of Texas Health Center at Tyler
Michael R Lewis, MD is a member of the following medical societies: American Academy of Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Lanny Garth Close, MD, Chair, Professor, Department of Otolaryngology-Head and Neck Surgery, Columbia University College of Physicians and Surgeons
Lanny Garth Close, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, American College of Physicians, American Laryngological Association, American Society for Head and Neck Surgery, and New York Academy of Medicine
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Peter S Roland, MD, Professor, Department of Neurological Surgery, Professor and Chairman, Department of Otolaryngology-Head and Neck Surgery, Director of Clinical Center for Auditory, Vestibular and Facial Nerve Disorders, Chief of Pediatric Otology, University of Texas Southwestern Medical Center; Adjunct Professor of Communicative Disorders, School of Human Development.
Peter S Roland, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Otolaryngic Allergy, American Academy of Otolaryngology-Head and Neck Surgery, American Auditory Society, American Laryngological Rhinological and Otological Society, American Neurotology Society, American Otological Society, North American Skull Base Society, and Society of University Otolaryngologists-Head and Neck Surgeons
Disclosure: Alcon labs Honoraria Speaking and teaching; GSK Honoraria Speaking and teaching; Advanced Bionics Honoraria Board membership; Cochlear corp Honoraria Board membership; Med El corp travel grants Speaking and teaching; Insight vision Consulting fee Consulting

CME Editor

Christopher L Slack, MD, Otolaryngology-Facial Plastic Surgery, Private Practice, Associated Coastal ENT; Medical Director, Treasure Coast Sleep Disorders
Christopher L Slack, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Arlen D Meyers, MD, MBA, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine
Arlen D Meyers, MD, MBA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Head and Neck Society
Disclosure: Covidien Corp Consulting fee Consulting; US Tobacco Corporation unstricted gift unknown

 
 
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