Chondrodermatitis nodularis

Synonyms: chondrodermatitis nodularis helicis (or antihelicis - depending on the site of the lesion); chondrodermatitis nodularis chronica helicis; ear pressure sore; painful nodule of the ear; Winkler's disease

This is a painful, inflamed nodule of the ear, involving the cartilage and skin of the pinna. It is usually located at the apex of the helix.

Etiologi¹

• It is probably caused mainly by pressure, combined with the anatomy of the pinna and its blood supply:
  

  • The initial damage may be triggered by inflammation, oedema and necrosis from trauma, cold, actinic damage or pressure.

  • The pinna has relatively little subcutaneous tissue for insulation and padding. Only small dermal blood vessels supply the epidermis, dermis, perichondrium and cartilage. These features may prevent adequate healing and lead to secondary perichondritis.

• Possibly, perichondrial arteriolar changes may be involved. An autoimmune aetiology has also been implicated.²

Epidemiologi³

The condition is probably relatively common but is rarely documented in the literature. It is most common in elderly men but It affects those of all ages, gender and pigmentation.

Data suggest that the condition is likely to be localised to the helix in men and the antihelix (the curved raised ridge of cartilage in front of the helix) in women.

Anything that puts pressure on the external ear, such as headphones, may precipitate the condition, as may a tendency always to sleep on the same side.

Antihelix position

© Mikael Häggström, Public domain, via Wikimedia Commons

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Presentation⁴

History:

• It presents as a painful nodule on the helix or antihelix of the ear.

• It appears spontaneously and then usually enlarges rapidly to its maximum size and remains stable. Onset may be precipitated by pressure, trauma or cold.

Chondrodermatitis nodularis of the ear

© Klaus D. Peter, Wiehl, Germany,CC BY 3.0 DE, via Wikimedia Commons

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Skyltar

There is usually only one nodule - most often on the outer side of the helix, commonly at its apex:

• The nodule is firm, tender and well demarcated.

• Size range is 3-20 mm in diameter - usually about 6 mm in diameter.

• It is round or oval, slightly raised and firmly stuck to the underlying cartilage.

• It may appear inflamed.

• The surface appears scaly or crusty. If the crust is removed, a small raw area or core may be visible centrally.

Differentialdiagnos⁵

• Basalcellscancer.

• Skivepitelcancer.

• Keratoacanthoma.

• Aktinisk keratos.

• Elastotic nodules of the ears.

• Cystic chondromalacia.

Utredningar⁶

Biopsy is needed if the diagnosis is uncertain, particularly to exclude basal cell carcinoma or squamous cell carcinoma.

Associerade sjukdomar

The condition is benign. One study suggested an association with immune-mediated vascular compromise and necrobiotic collagenous disease such as granuloma annulare, especially in younger patients.⁷

Another report concerns a patient with systemic sclerosis and primary biliary cirrhosis (Reynold's syndrome). Such patients have an increased risk of developing skin malignancies.²

Ledning⁴

The lesion rarely resolves spontaneously and so will usually need some form of treatment if it is causing discomfort or annoyance. A trial of conservative therapy for all patients is suggested.^{1 8}

Conservative management

The aim of treatment is to reduce pressure on the site of the lesion - for example, by:

• Altering the way headphones or telephone receivers are held to the ear.

• Sleeping on the opposite side, if possible.

• Using a soft pillow, or modifying the pillow with a hole where the nodule is in contact. A doughnut-shaped pillow was effective in one study.⁸ A haemorrhoid cushion is a novel cheap alternative.⁹

• Using a corn plaster.

• A pressure-relieving device, which can be made by using foam plastic to be applied at night.⁷

• Using a protective made-to-measure silicone splint.¹

In addition:

• Avoid excessive exposure to the cold and the sun.

• Topical antibiotics for secondary infections or ulceration. Petroleum jelly may also help if the lesion is ulcerated.¹

Further management¹⁰

• Topical nitroglycerin has been beneficial in the control of symptoms and in improving appearance.¹¹

• Topical and intralesional steroids may relieve discomfort.

• Cryotherapy has been used.

• CO₂ and argon lasers have been used with benefit in a small number of patients.

• Photodynamic therapy has shown some promise and is currently being investigated.

• Kirurgi:
  

  • There are various surgical techniques used to achieve excision of the affected cartilage with reconstruction of the overlying skin.

  • Excision of the damaged area of cartilage is usually successful but recurrence can occur at the edge of the excised area.

  • Narrow elliptical excision of the skin lesion with shaving of the underlying cartilage appears to be as effective and less invasive, and to have similar recurrence rates (around 13.5% after four years or so).¹²

  • Excision by punch biopsy, with full-thickness skin grafting (the 'punch and graft' technique).¹

Prognos

• The condition is not life-threatening but can affect the quality of life.¹⁰

• Small studies suggest that many, or most, cases respond to conservative treatment.⁸

• The condition may recur after surgery. Recurrence rates are about 10-30%.¹