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ORGAN SYSTEMS Otolaryngeal system (ENT)

Chondrodermatitis nodularis chronica helicis (CNH)

Rare non-neoplastic inflammatory and degenerative process of the external ear, characterized by necrobiotic changes in the dermis that extend down to the perichondrium.

Rare non-neoplastic inflammatory and degenerative process of the external ear, characterized by necrobiotic changes in the dermis that extend down to the perichondrium.

History:

CNH is also known as Winkler disease, based on the name of the dermatologist who first described the condition in 1915. It was later defined in 1918 by Foerster, who also outlined the microscopic, clinical, and treatment details of the disease.


Aetiology

Risk factors:

  • Chronic and excessive pressure on the pinna:
    • Same-side sleeping (M/C cause)
    • Other causes: Continuous and prolonged use of hearing aids, headphones, and other headgear
  • Anatomical features: Grossly protruding helix/antihelix
  • Repeated trauma
  • Actinic damage
  • Exposure to cold weather

Associated conditions:

Autoimmune and connective tissue disorders, especially among young females.
  • Autoimmune thyroiditis
  • Lupus erythematosus
  • Dermatomyositis
  • Scleroderma

Perichondrial vasculitis theory (2009):

Disease process begins secondary to the mentioned predisposing factors which cause arteriolar narrowing in the region of perichondrium, farthest from the arterial supply (helix). This leads to ischemia, necrosis, and extrusion of underlying cartilage. Ultimately, foreign body reaction sets in, resulting in severe inflammatory reaction and development of CNH.

Clinical features

Spontaneously appearing painful nodule on helix/antihelix

  • Classically presents unilaterally usually on the sleeping side.
  • Nodule grows rapidly and then stabilizes after attainment of maximum size
  • Nocturnal pain (M/C feature)
Solitary umbilicated nodule with central crust | Kumar, P., & Barkat, R. (2017). Chondrodermatitis nodularis chronica helicis. Indian dermatology online journal, 8(1), 48–49. https://doi.org/10.4103/2229-5178.198767

Physical examination:

  • Single oval/round nodule with raised, rolled edges
  • Firm, tender and usually fixed to the auricular cartilage on palpation.
  • Ulcer/crust in center
  • 4-6 mm diameter, typically surrounded by an erythematous area
  • M/C site: Apex of helix
  • Removal of the central crust reveals a small channel
  • Other associated features: Bleeding and exudate on the removal of the crust
Chondrodermatitis nodularis chronica helicis. (A) Clinical image: Erythematous hyperkeratotic papule on the left anthelix. (B,C) Dermoscopy: Structureless white areas and irregular, ill-defined vessels at the periphery. At the center, keratin and a yellow-brown flat crust (B), an erosion (C). | Morgado-Carrasco, D., Fustà-Novell, X., Podlipnik, S., & Ferrandiz, L. (2019). Dermoscopic Features of Chondrodermatitis Nodularis Chronica Helicis: A Case Series. Dermatology practical & conceptual, 9(1), 52–53. https://doi.org/10.5826/dpc.0901a12

Management

  • Pressure-relieving prosthesis/padding: Relieve/eliminate pressure on lesion
  • Topical/intralesional steroids
  • Local collagen injection: Cushioning and insulation (like subcutaneous tissue)
  • Photodynamic therapy (multiple treatment sessions required)
  • Carbon dioxide/argon laser treatment: Wound allowed to heal by secondary intention.
  • Topical 2% nitroglycerine twice daily
  • Cryosurgery. 
  • Electrocauterization/ curettage
Homemade pressure-relieving device | Salah, H., Urso, B., & Khachemoune, A. (2018). Review of the Etiopathogenesis and Management Options of Chondrodermatitis Nodularis Chronica Helicis. Cureus, 10(3), e2367. https://doi.org/10.7759/cureus.2367

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