Scrofula: Symptoms, Types, Causes and Treatment

Scrofula, once known as the “King’s Evil,” is a fascinating and complex disease rooted deeply in medical history. Primarily affecting the lymph nodes in the neck, scrofula is most often caused by infection with mycobacteria. Its diagnosis and treatment can be challenging, as it often mimics other diseases and presents in various forms. This article will explore the symptoms, types, causes, and treatments of scrofula, synthesizing evidence from historical and modern clinical sources.

Symptoms of Scrofula

Scrofula is notorious for its subtle onset and variable presentation, particularly in children and young adults. Recognizing the symptoms early is crucial for prompt diagnosis and effective management. Let’s explore the key clinical features and how they may differ depending on the underlying mycobacterial cause.

Symptom	Description	Frequency/Population	Source(s)
Lymphadenopathy	Enlarged, often unilateral neck lymph nodes	Most common, esp. in children	4 5 6
Skin Changes	Dusky/purplish hue, thinning, flaking	Progression of disease	4
Suppuration	Development of draining sinuses, abscesses	Advanced/untreated cases	4 6
Systemic Symptoms	Fever, weight loss, night sweats	More common in TB scrofula	5 6

Table 1: Key Symptoms

Overview of Symptomatology

• Lymphadenopathy is the hallmark of scrofula, typically appearing as painless, slow-growing swellings in the neck. These are usually unilateral and most commonly found in the submandibular and cervical regions 4 5.
• Skin changes develop as the lymph nodes enlarge and approach the skin, resulting in discoloration, thinning, and flaking. If untreated, the overlying skin may break down 4.
• Suppuration occurs when the affected lymph nodes become fluctuant, adhere to the skin, and eventually drain, forming chronic non-healing sinuses 4 6.
• Systemic symptoms such as fever, weight loss, and night sweats are more likely in cases caused by Mycobacterium tuberculosis, but are rare in nontuberculous mycobacterial (NTM) scrofula, especially in children 4 5 6.

Detailed Symptom Breakdown

Localized Lymph Node Swelling

• Most patients present with a single, painless, enlarged lymph node.
• The node may feel rubbery at first and gradually becomes firmer or matted to surrounding tissue.
• In children, the submandibular and submental lymph nodes are most commonly involved 4 5.

Skin Manifestations

• Overlying skin may become shiny, develop a bluish or purplish hue, and eventually thin out.
• Flaking and breakdown of the skin are signs of progression, often leading to the formation of fistulas or draining sinuses 4.

Progression to Suppuration

• Without intervention, the lymph node may soften (fluctuate), rupture through the skin, and drain pus.
• Chronic draining sinuses can persist, sometimes leading to scarring or poor cosmetic outcomes 4 6.

Systemic and Constitutional Symptoms

• Fever, night sweats, and weight loss are classic in tuberculous scrofula but are usually absent in NTM cases, especially in young children 4 5 6.
• The absence of these symptoms can help differentiate NTM scrofula from other causes of lymphadenitis.

Types of Scrofula

Understanding the different types of scrofula is essential for accurate diagnosis and effective treatment. The disease can be broadly classified based on the causative mycobacterium and clinical presentation.

Type	Causative Agent	Distinguishing Features	Source(s)
Tuberculous	Mycobacterium tuberculosis	Systemic symptoms, good response to antibiotics	2 5 6
Nontuberculous	Atypical mycobacteria (e.g., M. avium complex, M. scrofulaceum)	Children, localized, poor antibiotic response	1 2 4 5
Complicated	Multiple concurrent forms	Simultaneous or sequential involvement of different organs	3

Table 2: Types of Scrofula

Types in Detail

Tuberculous Scrofula

• Caused by Mycobacterium tuberculosis, often in association with pulmonary or systemic TB.
• More likely to present with systemic symptoms (fever, weight loss, night sweats).
• Responds well to standard antitubercular drug therapy 2 5 6.

Nontuberculous (Atypical) Scrofula

• Caused by non-tuberculous mycobacteria (NTM), most commonly Mycobacterium avium complex, M. scrofulaceum, M. kansasii, and others 1 4.
• Predominantly affects young children (1–5 years old).
• Presents as isolated, slowly progressive, painless lymphadenopathy with rare systemic symptoms.
• Less responsive to standard antibiotics; often requires surgical intervention 4 5.
• Diagnosis can be confirmed by culture, though not all cases are culture-positive 4.

Complicated and Mixed Forms

• Historical accounts describe cases with multiple manifestations: skin, bones, eyes, and lymph nodes involved simultaneously or sequentially 3.
• These complex presentations illustrate the systemic nature of scrofula and its potential for varied clinical forms.

Causes of Scrofula

The underlying cause of scrofula is infection by mycobacteria, but the specific pathogen and route of infection can significantly influence the presentation and treatment response.

Cause	Pathogen Example	Transmission/Pathogenesis	Source(s)
Tuberculous Scrofula	Mycobacterium tuberculosis	Reactivation or direct spread from pulmonary TB	2 5 6
NTM Scrofula	M. avium, M. scrofulaceum	Environmental exposure (soil, water); not person-to-person	1 4 5
Mixed/Other Causes	Various mycobacteria	Chance invasion, especially following oropharyngeal infection	1 3

Table 3: Causes of Scrofula

Detailed Exploration of Causes

Tuberculous Scrofula

• Caused by Mycobacterium tuberculosis, the same bacterium responsible for pulmonary and other forms of TB.
• Infection can occur via lymphatic spread from a primary pulmonary focus, or by direct inoculation.
• Historically prevalent, now more common in regions with high TB incidence or among immigrants from endemic areas 5 6.

Nontuberculous Mycobacterial (NTM) Scrofula

• Caused by environmental mycobacteria, such as M. avium complex, M. scrofulaceum, and M. kansasii 1 4.
• Infection occurs through environmental exposure, usually via oropharyngeal contamination entering deeper tissues, often after minor local infections 1.
• Not transmitted from person to person—no need for isolation precautions 1 4.
• More common in young children, as their developing immune systems and frequent exposure to contaminated soil/water put them at higher risk 4.

Pathogenesis and Risk Factors

• Scrofula develops when mycobacteria overcome local defenses and invade cervical lymph nodes.
• Risk factors include:
  • Age (children, especially 1–5 years old, for NTM)
  • Immunocompromised state (for both TB and NTM)
  • Exposure to contaminated environments (for NTM)
  • Residence in or immigration from TB-endemic regions (for TB scrofula) 4 5 6

Treatment of Scrofula

Treatment strategies for scrofula differ significantly depending on the causative mycobacterium. Accurate diagnosis is crucial to avoid ineffective therapies and unnecessary complications.

Treatment	Indication	Efficacy/Notes	Source(s)
Antitubercular Drugs	Tuberculous scrofula	High cure rate, standard regimens	5 6
Surgical Excision	NTM scrofula	High success, best cosmetic results	4 5
Observation	Early/small NTM cases	Sometimes effective, requires close monitoring	4
Incision & Drainage	Not recommended for NTM	Low cure, poor cosmetic outcomes	4

Table 4: Treatment Modalities

Thorough Overview of Treatment

Tuberculous Scrofula

• Medical therapy is the mainstay: multi-drug antitubercular regimens (e.g., isoniazid, rifampin, ethambutol, pyridoxine) are highly effective 5 6.
• Most patients respond well; surgery is rarely necessary except for diagnostic biopsy or complications 6.

Nontuberculous Mycobacterial (NTM) Scrofula

• Surgical excision is the preferred approach:
  • Complete removal of the affected lymph node(s) is curative in 90% of cases 4.
  • Early surgery offers the best outcomes, minimizing cosmetic defects 4.
• Medical therapy (e.g., macrolides) may be considered in selected cases or when surgery is contraindicated, but evidence is limited 4.
• Observation:
  • Some small, early cases may resolve spontaneously, but close follow-up is essential 4.
• Incision and drainage:
  • Not recommended for NTM scrofula due to high rates of persistent drainage, scarring, and poor cure rates 4.

Diagnostic and Therapeutic Procedures

• Fine-needle aspiration (FNA) can be useful for diagnosis and may be therapeutic in select cases, but complete excision is typically required for cure 4.
• Histology and culture are important for definitive diagnosis, though cultures may take weeks and are not always positive 4 6.

Special Considerations

• Surgery near the facial nerve (especially in the submandibular region) carries risks; experienced surgeons are essential 4.
• In rare cases, when surgery is not feasible (due to risk of nerve damage or parental refusal), prolonged observation or medical therapy may be indicated 4.

Conclusion

Scrofula is a disease with a rich historical legacy and ongoing clinical relevance. While its presentation and management vary depending on the underlying mycobacterial cause, modern diagnostics and targeted treatments offer high rates of cure and excellent outcomes when promptly applied.

Key Points:

• Scrofula typically presents as painless, slow-growing lymph node swelling, especially in the neck, often without systemic symptoms in children.
• The main types are tuberculous (Mycobacterium tuberculosis) and nontuberculous (atypical mycobacteria), with distinct clinical and therapeutic features.
• Causes include both human and environmental mycobacteria, with differing routes of infection and risks.
• Treatment is highly effective when tailored to the causative organism: antitubercular drugs for TB scrofula, surgical excision for NTM scrofula.
• Accurate diagnosis is essential to avoid ineffective or harmful therapies and to ensure optimal recovery and cosmetic results.

By understanding the nuanced differences in scrofula’s symptoms, types, causes, and therapies, clinicians and patients can confidently approach this ancient yet still relevant disease.