Description

• Acute or chronic inflammation of lymphatic vessels that typically presents as red, tender streaks rapidly extending proximally to regional lymph nodes
• May be infectious or noninfectious; commonly occurs on an extremity due to skin infection
• Lymphatic filariasis (elephantiasis) most common cause worldwide; bacterial infection most common cause in North America

Epidemiology

Prevalence
Estimated 51 million cases of lymphatic filariasis as of 2018; down from 120 million in 1997

Etiology and Pathophysiology

• Acute infectious lymphangitis
  • Most commonly caused by skin breakdown with secondary infection, typically bacterial
  • Streptococcus pyogenes (group A β-hemolytic Streptococcus) most common cause in immunocompetent
  • Consider gram-negative bacteria or fungi in immunocompromised patients
  • Less commonly caused by: Staphylococcus aureus, Pasteurella multocida (cat, dog, or other animal bite), Bartonella henselae (cat bite, cat scratch disease), Erysipelothrix (fish exposure), Spirillum minus (rat bite disease), Pseudomonas sp., other Streptococcus sp., Aeromonas hydrophila (fresh water exposures), Bacillus anthracis (cutaneous anthrax), Parapoxvirus (occupational milker’s nodule), herpes simplex virus (herpetic whitlow), Chlamydia trachomatis (lymphogranuloma venereum)
• Chronic lymphangitis
  • Filarial lymphangitis-typically affects extremities but can result in genital lymphatic involvement
    • Lymphatic filariasis (elephantiasis) most common cause of lymphangitis worldwide
    • Inflammation of lymph vessels due to presence and death of parasite transmitted via mosquito bite
    • Usually caused by nematodes (helminth) Wuchereria bancrofti; less commonly, Brugia malayi and Brugia timori
    • Endemic areas: Asia, Africa, the Western Pacific, the Caribbean, and South America
  • Nodular lymphangitis
    • Also known as sporotrichoid lymphangitis or lymphocutaneous syndrome
    • Painful or painless nodular subcutaneous swellings along lymphatic vessels
    • Lesions may ulcerate with accompanying regional lymphadenopathy
    • Usually does not develop as rapidly as acute lymphangitis and may not present with systemic symptoms
    • Typical of infections from: Sporothrix schenckii, Nocardia brasiliensis, Mycobacterium marinum, Leishmania sp., Franciscella tularensis, and systemic mycoses (coccidioidomycosis, blastomycosis, histoplasmosis)
    • Pathology may show granulomas.
  • Granulomatous intestinal lymphangitis: rare; associated with Crohn disease
  • Lymphangitis secondary to compromised lymphatic drainage
    • Following surgical procedures, trauma, malignancy, or radiation
    • Congenital or acquired anatomic abnormalities-elephantiasis nostras verrucosa (in setting of congestive heart failure and obesity)
  • Cutaneous lymphangitis carcinomatosa or neoplastic lymphangitis: rare presentation of skin metastasis—~5% of all skin metastases; caused by neoplastic occlusion of dermal lymphatic vessels
    • Associated cancers include breast (most common), lung, stomach, pancreas, and rectal.
• Acute noninfectious lymphangitis
  • Sclerosing lymphangitis of the penis: swelling around coronal sulcus of penis as a result of vigorous sexual activity or masturbation
  • Insect or spider bite

Risk Factors

• Skin infection—bacterial, viral, fungal
• Impaired lymphatic drainage
• Peripheral venous catheter; IV drug abuse
• Skin trauma including human, animal, insect, or spider bites
• Diabetes mellitus
• Immunocompromising condition
• Residence in filariasis endemic areas

General Prevention

• Proper wound and skin care
• Reduce chronic lymphedema with compression devices or by treating underlying processes.
• Insect repellent; arthropod bite precautions
• Chemoprevention (albendazole, ivermectin, diethylcarbamazine citrate) in endemic areas for lymphatic filariasis

Commonly Associated Conditions

• Cellulitis, erysipelas
• Lymphedema
• Filarial infection (W. bancrofti)
• Prior lymph node dissection
• Tinea pedis (athlete’s foot)
• Sporotrichosis