Have you noticed a painful lump on the outside of your ear? Is this particularly bad at night, and does it ever bleed or leak watery fluid? If the answer to any of these questions is yes, you may be suffering from a condition called Winkler's disease. This article will help inform you about what this is, and how it is managed.

What is Winkler's Disease?

Winkler's disease is a condition that causes painful lumps to form on the skin and cartilage on the outermost fold of the outer part of the ear known as the helix.¹ It is more commonly seen in middle to later-aged men² with fairer skin types, with 70-90% of cases reported to be in this group.³ Typically, the right side is affected in men, and the left side in women, although both ears can be affected in either sex.

Are there other names for Winkler's disease?

Winkler's disease also known as  Chondrodermatitis Nodularis Helicis, which can be translated to mean an area of inflammation affecting the tissues that comprise the outside of the ear. Some sources may refer to Chondrodermatitis Nodularis Chronica Helicis; this is the same condition, with chronica referring to a condition lasting longer than a few weeks.

Is Winkler's disease a serious condition?

Crucially, Winkler's disease is a benign condition;4 this means it is not associated with cancerous changes, however several more serious conditions can sometimes mimic this, so seek a prompt medical review if you have any concerns.

What causes Winkler's disease?

No clear reason has been identified for why individuals develop Winkler's disease.⁵ Probable causes include long-term sun exposure,⁶ age, individual anatomy (especially having prominent ears), injury to the ear and excess persistent pressure applied. Sources of such pressure can include:

• Preferential sleeping on a particular side
• Long term headphone use⁷
• Hearing aid use
• Certain headgear (especially that rests on or over the ears)

In certain patient groups, particularly in women under the age of 40, Winkler's disease may occur alongside other autoimmune conditions, the risk of which is increased if the individual is also a smoker[8].

How would I know that I was suffering from Winkler's disease?

Common signs and symptoms that one may be suffering from Winkler's disease are as follows:⁹

• A nodule (lump)
  • Located on the upper outer part of the ear, more commonly on the helix (outer fold) in males and the antihelix (inner fold) in females
  • Measures about 5mm
  • Firm to touch
  • Round, with raised edges and a brown or yellow crust on the top
  • Appear quite quickly
• Pain over the nodule, especially at night and on contact
• Watery fluid (known as exudate) leaking from the nodule, usually if the crust is knocked off
• Bleeding from the nodule

Why does Winkler's disease cause pain?

Although the exact cause is unclear, a recent theory suggested a process of perichondrial vasculitis is the likely reason for tenderness over the nodule.¹⁰ The concept is that long-term pressure causes the blood vessels on the outside of the ear to become narrowed. The ear is made up of a flexible material called cartilage, which even healthy tissue usually has poor blood supply. As a result of this, the narrowing of a few existing blood vessels results in not enough blood supply to meet the demands of the surrounding tissues. This causes small parts of the tissue to ‘suffocate’ (ischaemia) and in some cases ‘die’ (necrosis). The ischaemia and necrosis trigger an inflammatory response from the body’s immune system, which causes the nodule characteristic of Winkler's disease to form. The chemicals released, both from the dying tissues in the ear and from the inflammatory response, trigger a pain response to be sent to the brain, making the nodules sore to touch.

How is Winkler's disease diagnosed?

The healthcare provider will discuss the patient’s symptoms as listed above and examine accordingly. Often a diagnosis can be made on clinical assessment alone, but in some cases of uncertainty a biopsy can be performed, which gives a definitive diagnosis¹¹ (removing a small piece of the affected area and sending it to a specialist histopathological laboratory, where this is assessed under a microscope).

Does my clinician need to check my bloods?

Usually not. However, clinicians may want to perform blood tests, particularly if there is a suspicion of an underlying and undiagnosed autoimmune condition, as seen in connective tissue disorders like systemic sclerosis or primary biliary cholangitis.¹² In these cases, a nodule of Winkler's disease may be a sign of the presence of these other disorders.

Are there any other diseases that can look similar to Winkler's disease?

There are other conditions that can mimic Winkler's disease. In particular, although it is benign,  Winkler's disease nodules can look very similar to certain forms of skin cancer such as basal cell carcinoma  and squamous cell carcinoma,¹³ so the patient is assessed by a professional with the appropriate expertise.

What are the management options available to individuals with Winkler's disease?

It is possible to group the commonly used methods to treat Winkler's disease into three groups: conservative management, medical management and surgical intervention. In addition, there are several other treatment groups that have become accessible to the public that do not fall neatly into these categories; these are listed as miscellaneous. Discussion with the healthcare provider will allow the patient to choose the method most appropriate for self.

Conservative management

This relies on making simple adaptations to one’s life, to allow the body to deal with the nodule without the need to use any medicinal drugs or invasive surgical interventions. As a general rule, this involves the use of various forms of padding to take pressure away from the nodule. It can include:¹⁴

• Self-adherent foam sponges
• Foam bandages
• Doughnut or U-shaped pillows,¹⁵ for use when sleeping

Medical management

Sometimes conservative methods alone may not be enough. In this case, there are pharmaceutical agents that can be used to help clear up any nodules, such as:

• Hyaluronic acid injections¹⁶ – a component naturally found in skin and cartilage, that is directly injected into the nodule in 1-2 doses, with some studies suggesting a near immediate improvement in symptoms and complete resolution within a few weeks.
• Nitroglycerine topical treatments¹⁷ - an ointment applied twice daily.
• Topical and intralesional steroids¹⁸ – still occasionally used, but recent evidence to suggest benefit unless used alongside conservative management options.

Surgical management

In certain circumstances, the healthcare provider may recommend surgical removal of a nodule as a definitive cure.¹⁹ The exact techniques vary between treatment centres, but some common methods that may be used are:

• Primary excision (removal) of the nodule,²⁰ which can be performed with cartilage trimming or with cartilage sparing depending on the extent of cartilage involvement.
• Punch and graft technique²¹ – the nodule is removed with a punch biopsy (a small stick about half the width of a pencil, with a metal ring at the end) and, if necessary, a small skin graft can be placed over the raw area with tissue from behind the ear.

Miscellaneous

Certain treatment centres may also offer additional management options.²² These are highly specialised, often with limited amounts of research behind them and are typically only used where other therapeutic interventions have failed. Some examples include:

• Photodynamic therapy²³ – applying a chemical over the affected area, then exposing this to light from a specialised lamp emitting specialised wavelengths of light to remove the disease from this area.
• LASER ablation treatments²⁴ - using a carbon dioxide LASER beam to destroy the target lesion, and letting the raw area heal. A course of antibiotics is typically given to prevent infection after the procedure.
• Cryoablation²⁵ – use of liquid nitrogen on the affected area to freeze the nodule.

Can Winkler's disease come back after treatment?

It can take time for Winkler's disease to fully disappear. The majority of cases will resolve with conservative methods alone, but this can take several months. Although the surgical methods offer the most rapid solution, they are invasive, can lead to damage to surrounding structures and scarring, and there is a risk of recurrence of up to 30%.²⁶

Living with Winkler's Disease

Although it can be uncomfortable, the symptoms of Winkler's disease can be managed with adherence to the discussed treatment options. It is especially important to avoid anything that can cause long-term pressure to the ears. This could mean switching from headphones to earphones, using headwear that exerts less pressure over the ear and using appropriate sun protection for your skin. If one preferentially sleeps on a particular side, you don’t need to avoid this, however investing in a donut pillow or another similar pressure-relieving device can help to protect your ears at night.

Remember trying a conservative or medical intervention which is not helping, or have recently had a surgical intervention and experiencing a recurrence of symptoms, contact the healthcare provider for reassessment.

Summary

Winkler's disease is a benign condition that causes small painful lumps to form on the outside of the ear. It is likely the result of long-term pressure to the affected area cutting off blood supply and causing uncomfortable inflammation to this area. In addition to pain, there may also be small amounts of watery fluid or blood from the affected area. Management options include padding to remove pressure from the area, medical therapies and surgical options. Discussion with the healthcare provider can help too find the best solution.