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Onchocerciasis

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Overview & Agent - River Blindness Basics

  • Onchocerciasis (River Blindness): Chronic parasitic disease caused by filarial nematode Onchocerca volvulus.
  • Causative Agent: Onchocerca volvulus (filarial nematode).
    • Adult worms reside in subcutaneous nodules.
    • Microfilariae (larval stage) migrate through skin and eyes.
  • Vector: Female Simulium blackfly.
    • Breeds in fast-flowing rivers and streams (hence "river blindness").
    • Transmission occurs via repeated bites of infected blackflies.
  • Endemic Areas: Primarily Africa; also Latin America and Yemen.

Simulium blackfly, vector of Onchocerciasis

Onchocerca volvulus harbors endosymbiotic bacteria, Wolbachia pipientis, which contribute to the inflammatory response and are a target for therapy (doxycycline).

Pathogenesis & Clinical - Worm's Wrath

  • Pathogenesis:

    • Migrating microfilariae (MF) in skin & eyes → main pathology.
    • Adult worms reside in subcutaneous nodules (onchocercomas).
    • MF death (spontaneous or post-treatment, e.g., ivermectin) → triggers inflammatory response (Mazzotti reaction, classically severe with DEC, milder with ivermectin).
    • Chronic inflammation → dermal atrophy, fibrosis, depigmentation.
  • Clinical Manifestations:

    • Dermatological:
      • Intense Pruritus: often the earliest and most troublesome symptom.
      • Onchodermatitis (various forms):
        • Acute Papular OD (APOD): scattered, itchy papules.
        • Chronic Papular OD (CPOD): larger, flatter, hyperpigmented papules.
        • Lichenified OD (LOD/Sowda): hyperkeratotic, hyperpigmented, intensely itchy plaques; often unilateral on a lower limb.
      • Skin Atrophy: thin, wrinkled skin ("cigarette paper" or "lizard skin").
      • Depigmentation: mottled depigmentation, classically "leopard skin" appearance, especially on shins.
      • Onchocercomas: firm, non-tender, palpable subcutaneous nodules, typically over bony prominences. Onchocerciasis "leopard skin" on shins
    • Ocular ("River Blindness"):
      • Punctate keratitis ("snowflake" opacities) - early, often reversible.
      • Sclerosing keratitis → corneal opacity & blindness.
      • Anterior uveitis, iridocyclitis → glaucoma, cataracts.
      • Chorioretinitis, optic neuritis & atrophy.
    • Systemic:
      • Lymphadenopathy, lymphoedema → "hanging groin", elephantiasis.
      • Cachexia, musculoskeletal pain.

⭐ "Leopard skin" (spotted depigmentation, typically on the shins) is a characteristic late cutaneous manifestation of onchocerciasis, resulting from chronic inflammation and loss of melanin pigment where microfilariae have died in the dermis.

Diagnosis - Spotting the Squirmers

  • Skin Snips (Gold Standard):
    • Biopsy (iliac crest, scapula, calf).
    • Saline incubation, microscopy for motile microfilariae (mf).
    • Quantify mf density.
  • Ophthalmological Exam:
    • Slit-lamp: Punctate keratitis, iridocyclitis, optic atrophy.
  • Nodulectomy:
    • Excision & histology of nodules for adult worms.
  • Antibody Detection (Serology):
    • ELISA for IgG4 (Ov16 antigen).
    • Useful for travelers, early/light infections.
  • PCR:
    • On skin snips; highly sensitive.
  • Mazzotti Test (Rarely Used): ⚠️
    • Oral DEC provokes reaction. Risk of severe effects.
  • DEC Patch Test:
    • Topical DEC; localized skin reaction. Safer.
  • Eosinophilia: Common, non-specific.

⭐ Skin snips remain the cornerstone for diagnosing onchocerciasis, allowing direct visualization of microfilariae. Two snips, from iliac crest or gluteal region, are typically taken.

Management & Prevention - Banishing Blindness

  • Goals: Halt disease progression, alleviate debilitating symptoms (pruritus, eye lesions), interrupt transmission.
  • Pharmacotherapy:
    • Ivermectin (DOC): 150 mcg/kg PO, q6-12 months.
      • Potent microfilaricidal: Rapidly ↓ skin/eye microfilariae, reducing lesions & symptoms.
      • ⚠️ Mazzotti reaction: Common (fever, rash, pruritus); manage symptomatically (antihistamines).
    • Doxycycline: 200 mg/day PO for 4-6 weeks.
      • Targets Wolbachia endosymbionts; sterilizes/kills adult worms (macrofilaricidal effect).
  • Surgical: Nodulectomy (adjunctive; for accessible nodules, reduces local worm burden).
  • Prevention (Elimination Focus):
    • Mass Drug Administration (MDA): Community-wide Ivermectin (CDTI).
    • Vector Control: Larviciding Simulium (blackfly) breeding sites.

    ⭐ Ivermectin is microfilaricidal, reducing morbidity. Doxycycline targets Wolbachia, providing macrofilaricidal effects (sterilizing/killing adult worms), essential for interrupting transmission and achieving elimination.

High-Yield Points - ⚡ Biggest Takeaways

  • Caused by Onchocerca volvulus, transmitted by Simulium blackflies breeding in fast-flowing rivers.
  • Leads to "River Blindness" via sclerosing keratitis, chorioretinitis, and optic atrophy.
  • Skin: intense itching, papular onchodermatitis, leopard skin, subcutaneous nodules (onchocercomata).
  • Diagnosis: skin snips (microfilariae), slit-lamp exam (ocular microfilariae).
  • Treatment: Ivermectin is drug of choice; Mazzotti reaction (fever, rash) with DEC.
  • Doxycycline targets Wolbachia endosymbionts, leading to adult worm sterility/death.

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Practice Questions: Onchocerciasis

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In a patient presented with a fever and a positive filarial antigen test, what is the next appropriate method of management?

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_____ are blue/grey-coloured macules on the skin characteristically seen in Pediculosis pubis

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_____ are blue/grey-coloured macules on the skin characteristically seen in Pediculosis pubis

Maculae cerulae

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Onchocerciasis – NEET-PG Dermatology Notes | Oncourse