Chondrodermatitis nodularis chronica helicis
Synonyms and keywords:Chondrodermatitis nodularis helicis, winkler disease, ear pressure sore, chondrodermatitis nodularis auricularis

# Overview
Chondrodermatitis nodularis chronica helicis (also known as "Chondrodermatitis nodularis helicis":782) (CNCH) is a small, nodular, tender, chronic inflammatory lesion occurring on the helix of the ear, occurring most often in men.:610

# Historical Perspective
- Chondrodermatitis nodularis chronica helicis was first discovered by Max Winkler, a dermatologist, in 1915, in a paper titled Knotchenformige Erkrankung am Helix . 
- in 1925, Foerster further developed the clinical, microscopic and treatment options for CNCH.

# Pathophysiology
- The pathophysiology of Chondrodermatitis nodularis chronica helicis remains unclear. It may be due to an inflammatory condition from chronic pressure on helix of the ear, but can occur on anti-helix too.
- CNCH is seen mostly in elderly men. There are few cases seen in women and young, especially when CNCH is associated with autoimmune or connective tissue disorders. 
- The anatomy of ear pinna can contribute to the pathophysiology of CNCH. The pinna has limited blood supply and little subcutaneous tissue, which easily lead to formation of sore with pressure or trauma or cold temperature. 
- In 2009, Perichondrium vasculitis theory explained the above anatomical features. Because of vasculature and subcutaneous tissue padding limitations, there is easy development of ischemia and necrosis of underlying cartilage, causing a severe inflammatory condition, leading to CNCH. 
- There could be a possibility of genetic relation to the disease, as observed in one case study of monozygotic twins. 
- On gross pathology, CNCH looks like any other sore/nodule. Microscopically, it shows hyperkeratosis, parakeratosis with epithelial hyperplasia, dermal tissue damage with underlying cartilage destruction and dermal vessel proliferation. 

# Causes
- Few causes of CNCH include chronic pressure, trauma, anatomic features, temperature changes or actinic damage to the ear. Prolonged pressure seemed the most commonly reported cause. Sleeping on the one side can cause this pressure.
- Other causes can be continuous use of headphones/ ear phones/ cell phones, hearing aids, or can be idiopathic. There are some theories on association with autoimmune disorders and connective tissue diseases like scleroderma, autoimmune thyroiditis, lupus erythematosus, or dermatomyositis. 

# Differentiating Chondrodermatitis nodularis chronica helicis from other Diseases
- Chondrodermatitis nodularis chronica helicis can present as painful nodular lesion with central crust, so it must be differentiated from Basal cell carcinoma which is generally painless and with more vascularity.
- It must be differentiated from other diseases that cause a bump on the ear, such as:
- Actinic Keratosis
- Keratoacanthoma
- Squamous cell carcinoma
- Gouty tophi

# Epidemiology and Demographics
- The disease is much common than it is reported.

## Age
- CNCH is more commonly observed among patients aged 50-80 years old, but has been seen in young adults and children too.  Unilateral occurrence is more common.

## Gender
- CNCH is seen mostly in elderly men, but may occur with women and children too.

## Race
- There is no racial predilection for CNCH, but is seen more commonly in fair skinned with severe sun exposure.

# Risk Factors
- Common risk factors in the development of CNCH are pressure, trauma, sun damage, and systemic conditions.

# Natural History, Complications and Prognosis
- Early clinical features include just a painful sore. Spontaneous resolution is rare.
- Prognosis is generally good with treatment, although long term morbidity is common. Remissions can be seen when undertreated.

# Diagnosis

## History and Symptoms
- CNCH mostly presents as a spontaneously appearing round to oval tender nodule with raised edges on helix or anti-helix with a central crust/ulcer, commonly on ear of usually sleeping side, with nocturnal pain.
- Like said, this is the most common presentation, but it can also present as bilateral lesion, or with diurnal pain.
- It usually grows and becomes stable after reaching it's maximum size.

## Physical Examination
- Chondrodermatitis nodularis chronica helicis. Adapted from Dermatology Atlas.
- Chondrodermatitis nodularis chronica helicis. Adapted from Dermatology Atlas.
- Chondrodermatitis nodularis chronica helicis. Adapted from Dermatology Atlas.
- Chondrodermatitis nodularis chronica helicis. Adapted from Dermatology Atlas.
- Chondrodermatitis nodularis chronica helicis. Adapted from Dermatology Atlas.
- Chondrodermatitis nodularis chronica helicis. Adapted from Dermatology Atlas.
- Chondrodermatitis nodularis chronica helicis. Adapted from Dermatology Atlas.
- Chondrodermatitis nodularis chronica helicis. Adapted from Dermatology Atlas.
- Chondrodermatitis nodularis chronica helicis. Adapted from Dermatology Atlas.
- Chondrodermatitis nodularis chronica helicis. Adapted from Dermatology Atlas.

## Laboratory Findings
- There are no specific laboratory findings associated with CNCH.
- Skin biopsy may be necessary in conditions to differentiate it from carcinomatous lesions.

## Other Diagnostic Studies
- Investigating for the systemic illness( autoimmune diseases, connective tissue diseases or chronic non-communicable diseases) for underlying associations to the disease is indicated in some studies.

# Treatment
Medical therapy and Surgery are the treatment options. Surgical procedures have less chance of recurrence of the disease.

## Medical/Conservative Therapy
- The mainstay of therapy is to avoid/eradicate the triggers of CNCH, like pressure, trauma or solar damage. Pressure-relieving prosthesis is the most cost-effective method. They are available in various forms like foam sponge or foam bandages, doughnut pillows or CNH pillows, or a home-made prosthesis. 
- Intralesional and topical steroids are reported to be used in treating CNCH as a second line, with a treatment success rate of 30-40%, especially with an initial benefit. 
- Local collagen injections may provide cushioning and insulation, and corrects the deformity contour. 
- Topical antibiotics are used for secondary bacterial infections.
- Carbon dioxide laser vaporizes the nodule and underlying cartilage. One study reports 92% cure rate with it. Immediate pain relief, and excellent cosmetic results. 
- Nitroglycerin gel has been used by some to help reverse the cartilage necrosis by vasodilating arteriolar smooth muscles.
- Photodynamic therapy improves blood flow to the lesion and provides cytotoxic effect on the lesion.

## Surgery
- Various surgical procedures have been implemented over years in the treatment of CNCH, which popularly include cartilage excision, curettage and wedge excision. Surgery is still the gold standard for the treatment of CNCH, with a success rate of 89%-96% with wedge excision.

## Prevention
- Effective measures for the primary prevention of CNCH include avoiding ear pressure/ pressure offloading techniques.