Chondrodermatitis Nodularis Helicis


Article Author:
Gunjan Gupta


Article Editor:
Talel Badri


Editors In Chief:
Ahmad Al Aboud
Jayakar Thomas
Pramod Nigam


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Trevor Nezwek
Radia Jamil
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Steve Bhimji
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beata Beatty
Nazia Sadiq
Hajira Basit
Phillip Hynes
Tehmina Warsi


Updated:
6/4/2019 7:22:18 PM

Introduction

Chondrodermatitis nodularis chronica helicis (CNH) is a benign inflammatory condition that affects the skin and cartilage of the pinna. 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.[1][2]

This condition commonly affects the helix of the pinna, though in certain cases, the antihelix also may be affected. For this reason, some have suggested renaming the condition chondrodermatitis nodularis auricularis. Other synonyms for the condition are chondrodermatitis nodularis chronica helicis, ear pressure sores, and chondrodermatitis nodularis anti helicis.[3][4]

Etiology

The exact etiology behind the development of CNH remains unclear.  Most believe it is caused by chronic and excessive pressure on the pinna. Same-side sleeping usually causes this pressure. Other causes of pressure include the continuous and prolonged use of hearing aids, headphones, and other headgear. CNH also may be associated with autoimmune and connective tissue disorders like autoimmune thyroiditis, lupus erythematosus, dermatomyositis, and scleroderma, especially among young females. Anatomical features like the grossly protruding helix or antihelix of affected individuals also have been considered as contributory factors by some. Additional predisposing factors include repeated trauma, actinic damage, and exposure to cold weather.[5][6]

Furthermore, pinna has certain peculiar anatomical features which make it vulnerable to the development of CNH. These include little subcutaneous tissue and limited blood supply. Little subcutaneous tissue offers minimal cushioning and padding to underlying cartilage. This increases the chances of pressure and cold-induced damage. Limited blood supply further adds to the problem by causing a delay in the healing process and enhancing the chances of ischemia.[7]

Epidemiology

CNH is probably a disease of common occurrence, but it is infrequently documented in the literature. Therefore, the exact incidence of CNH remains unknown. This disease commonly affects men of middle-age or elderly but may affect women and younger adults. CNH can occur in all the ethnicities; however, it is more frequent among fair-skinned people with a history of chronic sun exposure.

Pathophysiology

The exact pathomechanism behind CNH development remains unknown; however, several theories have been proposed. Perichondrial vasculitis theory, reported in 2009 is the latest among all. According to this theory, the 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.[8][9]

Histopathology

The classical histopathology shows hyperkeratosis, parakeratosis with adjacent hyperplasia of the epithelium, and substantial destruction of dermal tissue which is usually lined by sclerosis. Other features include the proliferation of dermal vessels and destruction of underlying cartilage.[10][9]

History and Physical

Symptoms

CNH patients typically present with the complaint of a spontaneously appearing painful nodule on the helix or antihelix of the pinna. CNH is mainly located on the ear of the usual sleeping side, predominantly the right one. CNH classically presents unilaterally. Nevertheless, bilateral ear involvement may also be observed. Previously mentioned predisposing factors usually precipitate onset. The nodule grows rapidly and then stabilizes after attainment of maximum size. Nocturnal pain is the most frequent symptom with only a few cases presenting with daytime pain, occurring with touching.

Physical Examination

Physical examination reveals a single oval or round shaped nodule with raised, rolled edges and an ulcer or crust in its center. The lesion has a diameter of 4 mm to 6 mm and is typically surrounded by an erythematous area. The most common site is the apex of the helix. The nodule is firm, tender and usually fixed to the auricular cartilage on palpation. Removal of the central crust reveals a small channel. Other associated features include bleeding and exudate on the removal of the crust. Distribution on the antihelix is more common among females than among males. Location on rare sites like the external auditory canal and posterior part of the pinna has been reported.

Evaluation

Diagnosis of CNH can begin with a clinical examination. Histopathological confirmation by skin biopsy is the substitute method of diagnosis. Some recommend investigations to rule out systemic illness like scleroderma, thyroid disease, and other collagen vascular diseases especially in young adults presenting under 40 years of age.

Treatment / Management

There are several therapeutic options for the management of CNH. These can be broadly divided into conservative treatment and surgical excision.

Conservative Treatment

  1. Pressure-relieving prosthesis/padding is a cost-effective and inexpensive method. The primary goal of this prosthesis is to relieve or eliminate pressure on the lesion. These are available in various forms like foam sponge that is self-adhering to the postauricular area, foam bandages attached to the head and doughnut pillows for sleeping. The cure rate achieved by this method varies among different studies, mainly depending on patient compliance.
  2. Some institutes have used topical and intralesional steroids; however, the cure rate achieved with steroid use is unsatisfactory.
  3. Local collagen injection may provide relief by offering cushioning and insulation (like subcutaneous tissue).
  4. Photodynamic therapy has been found promising; however, a disadvantage is the requirement for multiple treatment sessions.
  5. Carbon dioxide/argon laser is another treatment modality. The patient is treated with laser, and later the wound is allowed to heal by secondary intention.
  6. Topical application with 2% nitroglycerine twice daily has been shown to relieve both symptoms and appearance. Nitroglycerine patches may be used as a substitute.
  7. Some also recommend cryosurgery. 
  8. Electrocauterization/ curettage is another treatment modality.

Surgery

Many techniques have emerged over the years for surgical excision of CNH. These include large excision followed by ear reconstruction using skin grafts or local flaps, removal of the lesional skin and cartilage, as well as techniques of cartilage-only removal with skin-sparing. Most of the studies agree that the simple approach with primary excision and meticulous trimming of cartilage is preferred as the method of choice.

Differential Diagnosis

CNH may be confused with a malignant skin condition like basal cell carcinoma due to its nodular appearance and central crusting. Larger and inflamed lesions may mimic squamous cell carcinoma. Gouty tophi is another differential diagnosis; however, unlike CNH,  tophi are multiple and occur on other sites like finger and toes.

Keratoacanthoma grows much faster than CNH, and classical resolution after a few months is the characteristic feature which helps to differentiate this condition. CNH lesions with predominant keratosis may resemble seborrheic or keratotic dermatitis.

Prognosis

CNH has a good prognosis, although long-term morbidity is common. Spontaneous resolution is extremely rare. Remission is commonly seen among inadequately treated cases.

Enhancing Healthcare Team Outcomes

CNH is best managed by a multidisciplinary team including ENT nurses. CNH is a relatively common disorder which is not well recognized. It is believed to be due to pressure on the ear from a variety of causes. The basic feature is a painful ear, usually at the pinna. Over the years, many treatments have been devised for this condition including surgery. To improve outcomes. clinicians should first try pressure offloading remedies first. Surgery to remove any obvious lesion should be the last resort. Because of the lack of trials, there is no consensus on which treatment works; hence one should educate the patient on avoiding ear pressure first, which does appear to provide symptom relief.


Interested in Participating?

We are looking for contributors to author, edit, and peer review our vast library of review articles and multiple choice questions. In as little as 2-3 hours you can make a significant contribution to your specialty. In return for a small amount of your time, you will receive free access to all content and you will be published as an author or editor in eBooks, apps, online CME/CE activities, and an online Learning Management System for students, teachers, and program directors that allows access to review materials in over 500 specialties.

Improve Content - Become an Author or Editor

This is an academic project designed to provide inexpensive peer-reviewed Apps, eBooks, and very soon an online CME/CE system to help students identify weaknesses and improve knowledge. We would like you to consider being an author or editor. Please click here to learn more. Thank you for you for your interest, the StatPearls Publishing Editorial Team.

Chondrodermatitis Nodularis Helicis - Questions

Take a quiz of the questions on this article.

Take Quiz
Regarding chondrodermatitis nodularis chronica helices, which is untrue?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
An elderly patient presents with a solitary tender nodule on the antihelix. The lesion appeared spontaneously and has been present for several years. Punch biopsy reveals dermal inflammation and cartilage necrosis. Which of the following is among the treatment options available for this condition?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A 70-year-old man from Rochester, United States presented to otologist with a complaint of a nodule on the outer margin of right pinna which causes severe pain while sleeping. He was a known case of presbyacusis and has used a behind the ear hearing aid right side for past 10 years. The nodule was biopsied and identified as chondrodermatitis nodularis helicis. Which of the following might be a predisposing factor for the disease?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
A 61-year-old male presented with a 10-month history of a painful nodule on right auricle. The pain was severe enough to wake him several times at night. There was no history of any trauma. The clinical diagnosis of chondrodermatitis nodularis (CNH) helicis is made after thorough physical examination. Which of the following is not a clinical feature of CNH?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up
Which of the following is not a challenging diagnosis for chondrodermatitis nodularis helicis?



Click Your Answer Below


Would you like to access teaching points and more information on this topic?

Improve Content - Become an Author or Editor and get free access to the entire database, free eBooks, as well as free CME/CE as it becomes available. If interested, please click on "Sign Up" to register.

Purchase- Want immediate access to questions, answers, and teaching points? They can be purchased above at Apps and eBooks.


Sign Up

Chondrodermatitis Nodularis Helicis - References

References

Cohen PR,Erickson CP,Calame A, Painful tumors of the skin:     [PubMed]
Morgado-Carrasco D,Fustà-Novell X,Podlipnik S,Ferrandiz L, Dermoscopic Features of Chondrodermatitis Nodularis Chronica Helicis: A Case Series. Dermatology practical     [PubMed]
Darragh CT,Om A,Zwerner JP, Chondrodermatitis Nodularis Chronica Helicis of the Right Nasal Vestibule. Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.]. 2018 Nov;     [PubMed]
Di Berardino F,Zanetti D, The Direct Use of Mobile Phone and the Occurrence of Chondrodermatitis Nodularis in the Antihelix: An Exemplificative Case. Indian dermatology online journal. 2018 Nov-Dec;     [PubMed]
García-García B,Munguía-Calzada P,Aubán-Pariente J,Junceda-Antuña S,Zaballos P,Argenziano G,Vázquez-López F, Dermoscopy of chondrodermatitis nodularis helicis. Archives of dermatological research. 2018 Sep;     [PubMed]
Salah H,Urso B,Khachemoune A, Review of the Etiopathogenesis and Management Options of Chondrodermatitis Nodularis Chronica Helicis. Cureus. 2018 Mar 26;     [PubMed]
Elsensohn A,Getty S,Shiu J,de Feraudy S, Intradermal Proliferative Fasciitis Occurring With Chondrodermatitis Nodularis Helicis. The American Journal of dermatopathology. 2018 Feb;     [PubMed]
Kumar P,Barkat R, Chondrodermatitis nodularis chronica helicis. Indian dermatology online journal. 2017 Jan-Feb;     [PubMed]
Shah S,Fiala KH, Chondrodermatitis nodularis helicis: A review of current therapies. Dermatologic therapy. 2017 Jan;     [PubMed]
Juul Nielsen L,Holkmann Olsen C,Lock-Andersen J, Therapeutic Options of Chondrodermatitis Nodularis Helicis. Plastic surgery international. 2016;     [PubMed]

Disclaimer

The intent of StatPearls is to provide practice questions and explanations to assist you in identifying and resolving knowledge deficits. These questions and explanations are not intended to be a source of the knowledge base of all of medicine, nor is it intended to be a board or certification review of Dermatology-Pediatric. The authors or editors do not warrant the information is complete or accurate. The reader is encouraged to verify each answer and explanation in several references. All drug indications and dosages should be verified before administration.

StatPearls offers the most comprehensive database of free multiple-choice questions with explanations and short review chapters ever developed. This system helps physicians, medical students, dentists, nurses, pharmacists, and allied health professionals identify education deficits and learn new concepts. StatPearls is not a board or certification review system for Dermatology-Pediatric, it is a learning system that you can use to help improve your knowledge base of medicine for life-long learning. StatPearls will help you identify your weaknesses so that when you are ready to study for a board or certification exam in Dermatology-Pediatric, you will already be prepared.

Our content is updated continuously through a multi-step peer review process that will help you be prepared and review for a thorough knowledge of Dermatology-Pediatric. When it is time for the Dermatology-Pediatric board and certification exam, you will already be ready. Besides online study quizzes, we also publish our peer-reviewed content in eBooks and mobile Apps. We also offer inexpensive CME/CE, so our content can be used to attain education credits while you study Dermatology-Pediatric.