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Lymphangitis


BASICS


DESCRIPTION


Acute or chronic inflammation of lymphatic channels due to a skin breach or local trauma. Presents as red, tender streaks along lymphatic channels and extending to regional lymph nodes ‚  
  • May result from compromised lymphatic drainage due to surgical procedures
  • May be infectious or noninfectious

ETIOLOGY AND PATHOPHYSIOLOGY


  • Acute infection
    • Usually caused by group A Ž ²-hemolytic Streptococcus
    • Less commonly caused by:
      • Staphylococcus aureus
      • Pasteurella multocida
      • Erysipelothrix
      • Spirillum minus (rat bite disease)
      • Pseudomonas
      • Other Streptococcus sp.
      • Immunocompromised patients can be infected with gram-negative rods, gram-negative bacilli, or fungi.
      • In fresh water exposures, Aeromonas hydrophila
  • Nodular lymphangitis
    • Also known as sporotrichoid lymphangitis
    • Presents as painful or painless nodular subcutaneous swellings along lymphatic vessels
    • Lesions may ulcerate with accompanying regional lymphadenopathy.
    • Typical of infections from the following: Sporothrix schenckii, Nocardia brasiliensis, Mycobacterium marinum, leishmaniasis, tularemia, and systemic mycoses
    • Pathology may show granulomas.
  • Noninfectious granulomatous lymphangitis
    • Rare-acquired lymphedema of the genitalia in children
    • May be due to atypical Crohn disease or sarcoidosis (1)[C]
  • Filarial lymphangitis
    • Mosquito bites transmit parasites causing inflammation and dilatation in the lymphatic vessels; can predispose to secondary bacterial infection
    • Usually caused by nematodes Wuchereria bancrofti. Other causes are Brugia malayi and Brugia timori.
  • Lymphangitis due to surgery
    • May occur after surgical procedures and lymph node dissection
  • Cutaneous lymphangitis carcinomatosa is rare. Represents ¢ ˆ ¼5% of all skin metasteses; caused by occlusion of lymphatic channels of dermis by neoplastic cells (2)
  • Sclerosing lymphangitis of the penis
    • Swelling around coronal sulcus of penis usually resulting from vigorous sexual activity or masturbation

RISK FACTORS


  • Impaired lymphatic drainage due to surgery, nodal dissection, or irradiation
  • Diabetes mellitus
  • Chronic steroid use
  • Peripheral venous catheter
  • Varicella infection
  • Immunocompromising condition
  • Human, animal, or insect bites
  • Fungal, bacterial, or mycobacterial skin infections
  • Any trauma to the skin
  • IV drug abuse
  • Residence in endemic areas of filariasis

GENERAL PREVENTION


  • Reduce chronic lymphedema with compression devices or by treating underlying process
  • Insect repellant
  • Proper wound and skin care

COMMONLY ASSOCIATED CONDITIONS


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

DIAGNOSIS


HISTORY


  • History of trauma to skin, cut, abrasion, or fungal infection
  • Systemic symptoms:
    • Malaise
    • Fever and chills
    • Loss of appetite
    • Headache
    • Muscle aches
  • Travel to a tropical region or region with known filariasis

PHYSICAL EXAM


Local signs: ‚  
  • Erythematous, macular linear streaks from site of infection toward the regional lymph nodes
  • Tenderness and warmth over affected skin or lymph nodes
  • May have blistering of affected skin
  • Fluctuance, swelling, or purulent drainage
  • Nodular lymphangitis can present with subcutaneous swellings along the lymphatic channels.
  • Sporotrichosis may present with papulonodular lesions that may ulcerate.
  • Sites may be nonpainful.

DIFFERENTIAL DIAGNOSIS


  • Superficial thrombophlebitis
    • Thrombus or infection within the thrombosis (septic thrombophlebitis)
  • Contact dermatitis
  • Allergic reaction: less likely to be allergic if >24 hours after exposure (e.g., insect bite)
  • Lymphangitis carcinomatosa
  • Malignancy-related inflammation

DIAGNOSTIC TESTS & INTERPRETATION


  • CBC may show leukocytosis; blood smear may show filarial infection.
  • Blood or wound cultures
  • Biopsy cultures
  • FNAC for filariasis of testiculoscrotal swelling but not for other superficial locations (3)

Initial Tests (lab, imaging)
Plain radiology unnecessary; may consider lymphangiography for lymphedema (4)[C] ‚  
Diagnostic Procedures/Other
  • Swab, aspirate, and/or biopsy primary site, purulent discharge, nodule or distal ulcer for culture, acid fast staining, histology, and microscopy
  • Blood cultures if systemically ill
  • Serology (e.g., Francisella tularensis, histoplasma)
  • Blood film/smear (e.g., filaria)
  • Lymphangiography to determine lymphedema or lymphatic obstruction

TREATMENT


GENERAL MEASURES


  • Hot, moist compresses to affected area
  • If lymphedema is involved, compression garments and weight loss may help.
  • Abstinence from sexual activity (for sclerosing lymphangitis)

MEDICATION


  • Treat common organisms empirically. Use culture and susceptibility to guide subsequent antibiotic treatment (5)[B].
  • If mild disease, use outpatient oral antibiotics.
  • If no improvement after 48 hours of oral antibiotics, reassess and consider IV antibiotics and/or hospitalization.
  • If systemic involvement, start IV antibiotics.
  • If necrotizing fasciitis due to group A Ž ²-hemolytic Streptococcus is suspected, treat aggressively with antibiotics and surgical intervention.

First Line
  • Antibiotics for group A streptococcal infection
    • Amoxicillin (if patient known to have only group A Streptococcus)
      • Dosing
        • Adults
          • Mild to moderate: 500 mg PO q12h
          • Severe: 875 mg PO q12h or 500 mg PO q8h
        • Children <3 months: 30 mg/kg/day PO divided q12h
        • Children ≥3 months, ≤40 kg
          • Mild to moderate: 25 mg/kg/day PO divided q12h or 20 mg/kg/day divided q8h
          • Severe: 45 mg/kg/day PO divided q12h or 40 mg/kg/day divided q8h
        • Children ≥40 kg same as adult dosing
      • Common adverse effects
        • Diarrhea
      • Serious adverse effects
        • Anaphylaxis, Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN)
      • Drug interactions
        • Methotrexate, venlafaxine, warfarin, hormonal contraceptives
      • Contraindications
        • Hypersensitivity to penicillin
  • Ampicillin/sulbactam
    • Dosing
      • Adults and children ≥40 kg: 1.5 to 3 g (ampicillin + sulbactam component) IV/IM q6h
      • Children <40 kg: 200 mg/kg/day IV infusion, in divided doses q6h; maximum 8 g ampicillin per day
    • Common adverse effects
      • Diarrhea, injection site reactions
    • Serious adverse effects
      • Clostridium difficile diarrhea, pseudomembranous enterocolitis
    • Drug interactions
      • Hormonal contraceptives
    • Contraindications
      • Hypersensitivity reactions
  • Ceftriaxone
    • Dosing
      • Adults: 1 to 2 g IV/IM q24h
      • Children: 50 to 75 mg/kg/day IV/IM once daily or in divided doses q12h; maximum 2 g/day
    • Common adverse effects
      • Injection site reactions, diarrhea
    • Serious adverse effects: same as amoxicillin or ampicillin
    • Drug interactions
      • Do not administer calcium-containing solutions in the same IV line.
    • Contraindications
      • Hypersensitivity to cephalosporins
      • Concurrent calcium-containing IV fluids
      • Increased risk of kernicterus, salt precipitation in lungs and kidneys in neonates <28 days (use cefotaxime instead)
  • Cephalexin
    • Dosing
      • Adults: 500 mg PO q12h
      • Children: 25 to 50 mg/kg/day divided q12h
    • Common adverse effects
      • Diarrhea
    • Serious adverse effects
      • SJS, TEN, interstitial nephritis, renal failure, pseudomembranous enterocolitis, anaphylaxis
    • Contraindications
      • Hypersensitivity to cephalosporins
  • Azithromycin (if penicillin or cephalosporin allergy)
    • Dosing
      • Adults: 500 mg PO on day 1 followed by 250 mg/day PO on days 2 to 5
      • Children ≥2 years: 12 mg/kg/day PO (maximum dose: 500 mg/day) once daily for 5 days (FDA off-label use for skin infections in children)
    • Common adverse effects
      • Abdominal pain, nausea, vomiting, diarrhea, headache
    • Serious adverse effects
      • Prolonged QT interval, torsades de pointes, liver failure, Lambert-Eaton syndrome, myasthenia gravis, corneal erosion, anaphylaxis
    • Drug interactions
      • Nelfinavir, warfarin, other medications with potential to prolong QT interval
    • Contraindications
      • Hepatic dysfunction or cholestatic jaundice with prior treatment
      • Hypersensitivity to macrolide (azithromycin, erythromycin, clarithromycin)
  • Diethylcarbamazine, ivermectin, albendazole, and doxycycline are used to treat filarial infection.
  • Acetaminophen or ibuprofen (NSAIDs) for pain and fever

SURGERY/OTHER PROCEDURES


  • Incision and drainage of abscess if present
  • Necrotizing fasciitis needs surgical evaluation and likely debridement
  • Nodular lymphangitis may benefit from I&D
  • With severe lymphedema, consider surgical drainage

INPATIENT CONSIDERATIONS


Admission Criteria/Initial Stabilization
  • Admit for signs of serious illness: fluids if in hypotensive shock.
  • Fever, chills, systemic toxicity
  • IV antibiotics
  • ICU or surgery as indicated

Discharge Criteria
Patient can be discharged on oral antibiotics after systemic symptoms resolve. Home IV antibiotics are an option depending on clinical setting. ‚  

ONGOING CARE


FOLLOW-UP RECOMMENDATIONS


  • Elevate affected area.
  • 48-hour follow-up to ensure improvement
  • Recurrent lymphangitis should prompt workup to ascertain underlying cause (other infectious organism, anatomic abnormality, etc.).

Patient Monitoring
Close follow-up to ensure decreasing inflammation ‚  

PATIENT EDUCATION


Instruct patients on proper wound and skin care. ‚  

PROGNOSIS


  • Good prognosis for uncomplicated cases
  • Antimicrobial therapy is effective in 90% of patients.
  • Untreated, can spread rapidly, especially group A Streptococcus

COMPLICATIONS


Sepsis, cellulitis, necrotizing fasciitis, myositis ‚  

REFERENCES


11 Taylor ‚  MJ, Hoerauf ‚  A, Bockarie ‚  M. Lymphatic filariasis and onchocerciasis. Lancet.  2010;376(9747):1175 " “1185.22 Prat ‚  L, Chouaid ‚  C, Kettaneh ‚  A, et al. Cutaneous lymphangitis carcinomatosa in a patient with lung adenocarcinoma: case report and literature review. Lung Cancer.  2013;79(1):91 " “93.33 Khare ‚  P, Kala ‚  P, Jha ‚  A, et al. Incidental diagnosis of filariasis in superficial location by FNAC: a retrospective study of 10 years. J Clin Diagn Res.  2014;8(12):FC05 " “FC08.44 Falagas ‚  ME, Bliziotis ‚  IA, Kapaskelis ‚  AM. Red streaks on the leg. Lymphangitis. Am Fam Physician.  2006;73(6):1061 " “1062.55 Badger ‚  C, Seers ‚  K, Preston ‚  N, et al. Antibiotics/anti-inflammatories for reducing acute inflammatory episodes in lymphoedema of the limbs. Cochrane Database Syst Rev.  2004;(2):CD003143.

ADDITIONAL READING


  • Babu ‚  AK, Krishnan ‚  P, Andezuth ‚  DD. Sclerosing lymphangitis of penis " ”literature review and report of 2 cases. Dermatol Online J.  2014;20(7):9.
  • Edlich ‚  RF, Winters ‚  KL, Britt ‚  LD, et al. Bacterial diseases of the skin. J Long Term Eff Med Implants.  2005;15(5):499 " “510.
  • Raja ‚  A, Seshadri ‚  RA, Sundersingh ‚  S. Lymphangitis carcinomatosa: report of a case and review of literature. Indian J Surg Oncol.  2010;1(3):274 " “276.
  • Schubach ‚  A, Barros ‚  MB, Wanke ‚  B. Epidemic sporotrichosis. Curr Opin Infect Dis.  2008;21(2):129 " “133.

CODES


ICD10


  • I89.1 Lymphangitis
  • L03.91 Acute lymphangitis, unspecified
  • N48.29 Other inflammatory disorders of penis
  • B74.0 Filariasis due to Wuchereria bancrofti
  • B74.1 Filariasis due to Brugia malayi
  • B74.2 Filariasis due to Brugia timori

ICD9


  • 457.2 Lymphangitis
  • 682.9 Cellulitis and abscess of unspecified sites
  • 607.2 Other inflammatory disorders of penis
  • 125.0 Bancroftian filariasis
  • 125.1 Malayan filariasis

SNOMED


  • 1415005 Lymphangitis (disorder)
  • 8838005 Acute lymphangitis (disorder)
  • 78973009 Chronic lymphangitis (disorder)
  • 91586009 Bancroftian elephantiasis (disorder)
  • 361279005 Sclerosing lymphangitis of penis (disorder)
  • 7066000 Infectious lymphangitis (disorder)
  • 19661000 Malayan elephantiasis (disorder)

CLINICAL PEARLS


  • Lymphangitis classically presents with erythematous linear streaks of the skin from the inciting site (e.g., bite, cut, abrasion) to regional lymph nodes.
  • Patients with prior surgical lymph node dissection are predisposed to lymphangitis.
  • Patients with severe systemic symptoms should be admitted and treated with IV antibiotics.
  • Parasitic or fungal infections can cause acute or chronic lymphangitis.
  • Treatment of underlying skin infection (such as tinea pedis) may prevent recurrence.
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