Lymphangitis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Vishal Devarkonda, M.B.B.S[2]

Lymphangitis is an inflammation of the lymphatic channels. Lymphangitis is an ancient disease. The exact origins of the disease are not clearly known. There is no classification system established for lymphangitis. However, based on the etiology, lymphangitis can be broadly classified into infectious and non-infectious causes. The etiology varies depending on the clinical form of lymphangitis. Lymphangitis is inflammation of the lymphatic system, a system that is comprised of small vessels of the immune system that carry lymphatic fluid. The fluid is filtered in the lymph nodes and spleen, drained into the subclavian veins through thoracic duct and finally returned back to the circulatory system. Differential diagnosis of lymphangitis includes contact dermatitis, cellulitis, thrombophlebitis, fasciitis and myositis. Incidence, prevalence and demographics of lymphangitis are poorly studied. Trauma, chronic disorders and infections of the skin are the common risk factors involved in the development of lymphangitis. Depending on etiology, lymphangitis has a broad spectrum of natural history, complications, prognosis, clinical presentation and physical findings. The presence of certain non-specific laboratory findings, in the presence of lymphangitis, may be suggestive of certain underlying causes. Examples of tests that may demonstrate abnormal findings include CBC, ESR, CRP, and serology titers. The mainstay of therapy in lymphangitis is antimicrobial therapy. Supportive therapy includes analgesics, anti-inflammatory agents, and warm compresses. Surgery is indicated in certain cases of nodular lymphangitis that are complicated by abscess and lymphedema in addition to appropriate medical therapy. There is no established primary prevention for lymphangitis.

Lymphangitis is an ancient disease. The exact origins of the disease are not clearly known. Specific historical perspective for individual infections are discussed in detail separately.

There is no classification system established for lymphangitis. Based on the etiology, lymphangitis can be broadly classified into:

• Lymphangitis due to infectious etiology
• Lymphangitis due to non-infectious etiology

The etiology varies depending on the clinical form of lymphangitis.

Lymphangitis is inflammation of the lymphatic system, a system that is comprised of small vessels of the immune system that carry lymphatic fluid. The fluid is filtered in the lymph nodes and spleen, drained into the subclavian veins and finally returned back to the circulatory system.

The presentation of red streaks on the skin in lymphangitis can also be a presentation of conditions such as contact dermatitis, cellulitis, thrombophlebitis, fasciitis and myositis. Clinical findings like red linear streaks proximal to a distal site of infection, tender regional lymphadenopathy and fever, along with a history suggestive of infection by causative organisms of lymphangitis helps in differentiating lymphangitis from other conditions.^[1]

Incidence, prevalence and demographics of lymphangitis are poorly studied. Specific epidemiological and demo-graphical data for individual infections are discussed in detail separately.

Trauma, chronic disorders and infections of the skin are the common risk factors involved in the development of lymphangitis.

Depending on etiologies, lymphangitis has a broad spectrum of natural history, complication, and prognosis. Specific natural history, complications and prognosis for individual infections are discussed in detail separately.

Depending on etiologies, lymphangitis has a broad spectrum of clinical presentation. Specific history and symptoms for individual infections are discussed in detail separately.^{[1][2][3][4][5][6][7][8]}

Depending on etiologies, lymphangitis has spectrum of physical findings. Specific physical examination findings for individual infections are discussed in detail separately.^{[1][4][5][6][7][8][2][9]}

There are no diagnostic lab findings associated with lymphangitis. The presence of certain non-specific laboratory findings, in the presence of lymphangitis, may be suggestive of certain underlying causes. Examples of tests that may demonstrate abnormal findings include CBC, ESR, CRP, and serology titers.^[1]

There are no diagnostic X-ray findings associated with lymphangitis.

There are no diagnostic CT findings associated with lymphangitis.

There are no other diagnostic findings associated with lymphangitis.

The mainstay of therapy in lymphangitis is antimicrobial therapy. Supportive therapy includes analgesics, anti-inflammatory agents, and warm compresses. Specific anitmicrobial treatment for individual infections are discussed in detail separately.

Certain cases of nodular lymphangitis that are complicated by abscess and lymphedema require surgical intervention in addition to appropriate medical therapy.

Lymphangitis is most often caused by recurrent skin infections due to group A beta-hemolytic streptococci and prophylactic administration of antibiotics can reduce the risk of these skin infections complicating into lymphangitis.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Vishal Devarkonda, M.B.B.S[2]

There is no established classification system for lymphangitis. However, it may be classified by etiology as infectious or non infectious, or by the clinical form of the infection as acute or chronic.

There is no established classification system for lymphangitis. However, based on the etiology, lymphangitis can be broadly classified into:

• Lymphangitis due to infectious etiology
• Lymphangitis due to non-infectious etiology

Based on the clinical form, lymphangitis due to infection can be further classified into:

• Acute Lymphangitis
• Chronic Granulomatous Lymphangitis
  • Filarial Lymphangitis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Vendhan Ramanujam M.B.B.S [2]Vishal Devarkonda, M.B.B.S[3]

Lymphangitis is inflammation of the lymphatic system, a system that is comprised of small vessels of the immune system that carry lymphatic fluid. The fluid is filtered in the lymph nodes and spleen, drained into the subclavian veins through thoracic duct and finally returned back to the circulatory system. Depending on the etiologies, lymhangitis has a spectrum of pathophysiology.

• Lymphatic vessels are thin walled, valved structures situated in deep dermis and sub-dermis tissues.^[1]
• Lymph follows the following pattern of drainage: ^[1]
  • Lymph from tissues drain into the regional lymph nodes via afferent lymphatics, then to right lymphatic duct and thoracic duct via efferent lymphatics, and finally into venous circulation via one of the subclavian veins.

Settings involved in pathogenesis of disease are: ^[2][3]

• Normal Lymphatic vessels:- Invasion of microorganisms either from traumatized skin, or from a distal site of infection into the lymphatic vessels that leads to inflammation of the vessel.
• Anatomical abnormalities resulting in obstruction of lymphatic channels or damage to the lymphatic vessels, as seen in setting of surgery, malignancy, and trauma.^[4]

Depending on etiologies, lymphangitis has spectrum of pathophysiology:

Acute lymphangitis, develops in setting of distal trauma, resulting in infection. Infection, if not contained locally, may spread through lymphatic channels resulting in lymphangitis.^[2][3]

Chronic granulomatous lymphangitis, develops in the setting of minor trauma to extremities (e.g., wood splinter or rose bush). Distal infection spreads through an indolent process, resulting in subcutaneous nodules along the course of lymphatic channels.^[2][3]

Filarial lymphangitis develops when thread like adult filarial worms resides in lymphatic channels and lymph nodes, resulting in inflammatory changes to the lymphatics.^[5]

Lymphangits carcinomatosa is a dreadful condition, that develops due to extensive lymphatic permeation and embolization of lymphatic capillary by tumor cells.^[6][7]

• The exact pathophysiology of sclerosing lymphangitis remains unclear. However, many patients report increase in sexual activity before the appearance of lesion, indicating idiopathic nature of the disease.^[8]

Light microscopic and electron microscopy examination reveal:^[8]

• Sclerosis and hypertrophy of lymphatic vessels along with mild perilymphatic round cell infiltrate.
• Marked edema and thickening of lymphatic vessels both with and without thrombus formation.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Vendhan Ramanujam M.B.B.S [2]Vishal Devarkonda, M.B.B.S[3]

The etiology varies depending on the clinical form of lymphangitis. The most common causes of acute lymphangitis include group A streptococcus, Staphylococcus aureus, and Pasteurella multocida. The most common causes of chronic granulomatous lymphangitis include Sporotrichosis, Nocardia brasiliensis, Mycobacterium marinum, and Brugia spp.

The etiology varies depending on the clincal form of lymphangitis. Causes of lymphangitis include:^{[1][2][3][4][5][4][6][7][8][9][10]}

• Group A streptococcus
• Staphylococcus aureus
• Pasteurella multocida

• Sporothrix schenckii
• Sporotrichosis
• Mycobacterium marinum
• Nocardia brasiliensis,
• Leishmania braziliensis
• Francisella tularensis

• Wuchereria bancrofti
• Brugia malayi
• Brugia timori
• Brugia pseudomallei

• Aeromonas hydrophila
• Herpes simplex virus
• Ricettsia sibirica monogolotimonae
• Lymphogranuloma venerum
• Erysipelothrix
• Erysipelothrix rhusiopathiae
• Bacillus anthracis
• Fusobacterium nucleatum
• Peptostreptococcus
• Prevotella
• Pseudomonas
• Rhodococcus equi
• Rickettsia africae
• Spirillum minus

• Nocardia
• Nocardia asteroides
• Mycobacterium chelonae
• Leishmania guyanensis
• Leishmania major
• Leishmania mexicana
• Leishmania panamensis
• Leishmania tropica
• Leishmania
• Mycobacterium kansasii
• Mycobacterium tuberculosis^[11][12]
• Blastomyces dermatitidis
• Coccidioides immitis
• Cryptococcus neoformans
• Histoplasma capsulatum
• Botryomycosis
• Streptococcus pyogenes

• Lymphangitis carcinomatosa^[5]
• Crohn’s disease ^[13]
• Radiation
• Venom of Scolopendra ^[14]
• Sclerosing lymphangitis^[15][16]
• Tuberculin skin test^[17]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Vendhan Ramanujam M.B.B.S [2]Vishal Devarkonda, M.B.B.S[3]

The presentation of red streaks on the skin in lymphangitis can also be a presentation of conditions such as contact dermatitis, cellulitis, thrombophlebitis, fasciitis and myositis. Clinical findings like red linear streaks proximal to a distal site of infection, tender regional lymphadenopathy and fever, along with a history suggestive of infection by causative organisms of lymphangitis helps in differentiating lymphangitis from other conditions.^[1]

• Contact dermatitis: Contact dermatitis is characterized by onset of symptoms like itching, burning and pain within minutes to hours following exposure to an environmentally relevant allergen and is associated with clinical findings like macular erythema, fissures, or vesicles. Clinical evidences like distal site of infection and tender regional lymphadenopathy are absent.
• Myositis: Typical cutaneous lesions along with symmetric proximal muscle weakness, elevated serum muscle enzymes, electromyographic finding of myopathic changes, and characteristic muscle biopsy abnormalities are characteristic of myositis.^[2]
• Necrotizing fasciitis: Gradual in onset and later rapid in progression of clinical features like fever, pain, swelling, crepitus, and foul-smelling wound drainage following trauma, surgery, or spread of an infection from a near by tissue or organ, along with signs of systemic toxicity and elevated serum creatine kinase levels are suggestive of necrotizing fasciitis. Surgical exploration establishes the diagnosis.^[3]
• Septic thrombophlebitis: Septic thrombophlebitis presents with clinical manifestations like fever, erythema, tenderness and purulent drainage at the site of an involved vessel in the setting of an intravascular catheter and persistent bacteremia. Further laboratory studies and radiological evidence establishes the diagnosis and differentiates it from lymphangitis.^[4]
• Superficial thrombophlebitis: Clinical findings like erythema, tenderness, induration, or palpable nodular cords along the course of a superficial vein is suggestive of superficial thrombophlebitis. Further evaluation with duplex ultrasound or laboratory studies helps in establishing the diagnosis while differentiating it from lymphangitis.^[5]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Vishal Devarkonda, M.B.B.S[2]

Lymphangitis being a manifestation of wider concurrently occurring spectrum of manifestations or pathologies. The epidemiology and demographics of this individual manifestation are poorly studied

Lymphangitis being a manifestation of wider concurrently occurring spectrum of manifestations or pathologies. The epidemiology and demographics of this individual manifestation are poorly studied. Epidemiology and demographics of lymphangitis carcinomatosis and sclerosing lymphangitis are discussed below:

Lymphangitis carcinomatosis occurs in 6-8% of the intra-thoracic metastasis from a malignant disease.^[1]

Higher prevalence of lymphangitis carcinomatosis is reported in males, when compared to females(60:40) and younger population.^[1]

Epidemiology and demographics

Incidence and prevalence of sclerosing lymphangitis is poorly studies. Demographically, it mostly occurs in younger male population between 20-40 years of age.^[2]

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Vendhan Ramanujam M.B.B.S [2]Vishal Devarkonda, M.B.B.S[3]

Lymphangitis being a manifestation of wide concurrently occurring spectrum of manifestations or pathologies. The risk factors vary from etiology to etiology.

Lymphangitis being a manifestation of wide concurrently occurring spectrum of manifestations or pathologies. The risk factors vary from etiology to etiology.

Common risk factors for infectious causes of lymphangits include:^{[1][2][3][4][5][6][7][8]}

• Any trauma to the skin
• Chronic skin disorders such as psoriasis or eczema
• Diabetes mellitus
• Human, animal, or insect bites
• Immunocompromised hosts
• Lymphedema
• Skin and soft-tissue infections
• Surgery
• Treatment of breast cancer
• Venous catheterization
• Travel to filaria endemic area

Risk factors for sclerosing lymphangitis include:^[9]

• Vigorous sexual acitvity

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Vishal Devarkonda, M.B.B.S[2]

Lymphangitis being a manifestation of wide concurrently occurring spectrum of manifestations or pathologies. Natural history, complications and prognosis varies from etiology to etiology.

Lymphangitis being a manifestation of wide concurrently occurring spectrum of manifestations or pathologies. Natural history, complications and prognosis varies from etiology to etiology. In general, the natural history of lymphangitis based on the classification follows:^{[1][2][3][4][5][6][7]}

Natural history of lymphangitis due to infectious etiology varies widely from organism to organism. Generally, if left untreated or body defenses weren’t able to overcome the pathological nature of organism, could lead to bacteremia, sepsis, septic shock, multi-organ failure, and death.

Lymphangits carcinomatosis, being a metstatic malignant condition, commonly results in respiratory failure even with treatment.

Being a self-limiting and self-resolving condition, If left untreated, it undergoes spontaneous resolving in weeks.^[8]

Complications of lymphangitis varies from etiology to etiology.

• Cellulitis
• Pneumonia
• Endocarditis
• Osteomyelitis
• Meningitis
• Septic artheritis
• Peritonitis
• Visceral abscess
• Septic shock
• Multi-organ failure

Most common complications of lymphangitis carcinmatosa are:^[16]

• Respiratory failure
• Right Heart failure

Prognosis of lymphangitis varies from etiology to etiology

With appropriate anitmicrobial treatment, most attacks of lymphangitis with infectious etiology resolve within days to weeks.^{[17][18][19][20][21][22][23]}

Being a manifestation of metastatic primary tumor. Lymphangitis carcinomatosa has extremely poor prognosis with 50% mortality within 3 months of onset of first respiratory symptoms.^[16]

Sclerosing lymphangitis, is self- limiting and self- resolving condition, with excellent prognosis.^[24]

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