5.1.3 Lymphangitis

Grading & Level of Importance: B

ICD-11

BD91

Synonyms

None.

Epidemiology

No clear statistical data available.

Definition

Inflammation along the lymphatic vessels.

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Inflammation along the lymphatic pathways between a site of cutaneous inflammation / infection / infarction and the regional lymph nodes.

Aetiology & Pathogenesis

Most common causes are bacterial and mycotic leading to ascending infection of lymphatics draining to regional lymph nodes. Non-infectious inflammatory processes, such as an arthropod bite or sting (filariasis and onchocerciasis), can also cause lymphangitis/lymphadenitis. Tumor cell invasion causes lymphangitis carcinomatosa.

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Most common cause is bacterial infection, leading to ascending infection of lymphatics draining to regional lymph nodes. It is usually a complication of soft tissue bacterial infections.

Lymphangitis most often results from an acute streptococcal infection of the skin. Less often, it is caused by a staphylococcal infection. Lymphangitis may be a sign that a skin infection is getting worse, initiating bacteriaemia and sepsis.

Non-infectious inflammatory processes, such as an arthropod bite or sting (filariasis and onchocerciasis), can also cause lymphangitis/lymphadenitis. Lymphangitis has also been seen in patients with Crohn’s disease.

Tumor cell infarction causes lymphangitis carcinomatosa. Breast, lung, stomach, pancreas, rectal, and prostate cancers are common types of tumors that can lead to lymphangitis.

Signs & Symptoms

Linear, tender erythema between the primary skin lesion (entry point for infection, site of bite or sting, tumor invasion) and the regional lymph nodes.

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Linear, tender erythema between the primary skin lesion (entry point for infection, site of bite or sting, tumor infarction) and the regional lymph nodes and throbbing pain along the affected area. Additionally, fever and chills, enlarged and tender lymph nodes, malaise, headache, loss of appetite and muscle aches may occur as general symptoms.

Localisation

Could appear elsewhere, mostly on extremities, sometimes trunk.
Special location: sulcus beneath glans penis (sclerosing lymphangitis).

Classification

  • Bacterial lymphangitis
    • Infestation-induced lymphangitis
    • Sclerosing lymphangitis (of the penis)
    • Lympangitis carcinomatosa
  • Mycotic lymphangitis: sporotrichosis

Laboratory & other workups

Markers for inflammation, bacterial and fungal swabs of entry site, regional lymph node sonography, tumor staging.

Dermatopathology

Depending on cause, widening of vessels and accumulation of neutrophils and positive staining for bacteria or nodular lymphatic vessels like in sporotrichosis (PAS stain). In cancerous lymphangiitis the lumen of vessels is filled with tumor cells which can be stained by immunohistochemistry according to suspected tumor origin.

Course

Acute. Usually responds rapidly to antibiotic therapy. In tumor association chronic progressive. Lymph vessels may become occluded and lymphedema occurs.

Complications

The primary site can evolve into an abscess. Development of cellulitis or sepsis. Chronic lymphedema (Filariasis, relapsing erysipelas).

Diagnosis

Clinical findings, microbiology. Possibly duplex sonography to exclude superficial thrombophlebitis or tumor.

Differential Diagnosis

Superficial thrombophlebitis, Mondor's disease.

Prevention & Therapy

Antibiotic and antimycotic therapy, intravenously in severe cases. Start with empiric regimen based on likely causes and adjust with culture results. Treat the entry site (incision, debridement, drainage, disinfection). In severe cases: bed rest, thromboprophylaxis with fractionated heparin. Topical: cool compresses.

 

Treatment of infestation, tumor surgery (primary tumor) or systemic antitumor therapy (metastases).

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Antibiotic and antimycotic therapy, intravenous in severe cases.

Start with empiric regimen based on likely causes and adjust with culture results. Treat the entry site (incision, debridement, drainage, disinfection). In severe cases: bed rest, thromboprophylaxis with fractionated heparin. Topical: cool compresses.

Treatment of infestation, tumor surgery (primary tumor) or systemic antitumor therapy (metastases).

Pain medication, anti-inflammatory medication, surgery to drain any abscesses that may have formed, surgical debridement, or removal, of a node (if it causes obstruction) may be required in severe complicated cases.

Special

Special location: sulcus beneath glans on penis (sclerosing lymphangitis). Differential diagnosis: Mondor’s disease.

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