Are You Confident of the Diagnosis?
Characteristic findings on physical examination
Chondrodermatitis nodularis helicis (CNH) is a small, nodular, inflammatory lesion found on the outermost portion of the ear. Most often, the lesions are found on the upper helical rim or middle to lower antihelical rim, but always at the most prominent or protuberant point of the ear. On physical examination, lesions appear to be skin-colored to erythematous dome shaped firm papules, usually measuring 2-4 mm in diameter (Figure 1). The center of the lesion can have a central depression, scale, crust or shallow ulcer (Figure 2). There may be some surrounding inflammation. Occasionally, there is just an erosion or ulcer.
Lesions often arise spontaneously without a history of preceeding known trauma. However, the two most important questions to ask in a patient encounter are:
Is the lesion tender?
Which side do you lie on when you sleep?
A key diagnostic feature is the exquisite tenderness to palpation or pressure that is often associated with CNH. This pain is often the reason the patient presents for evaluation. It may be exacerbated by hats, headphones or other devices that come into contact with the ear. Most commonly, the patient reports that the pain interferes with their ability to sleep at night. The chronic pressure from lying on the preferred side may also play a role in the pathogenesis of the lesion itself.
Expected results of diagnostic studies
Histopathology shows a well-circumscribed area of acanthosis, parakeratosis and hypergranulosis. A central ulceration with disruption of the epidermis may be present. The underlying dermis shows an area of eosinophilic necrosis of collagen surrounded by granulation tissue.
The dermal inflammation is seen extending to a thickened perichondrium. Epithelioid cells may be present and vascular proliferation may be present. This can have a similar histologic appearance to a decubitus ulcer but on a much smaller scale. In addition, peripheral solar elastosis is common (Figure 3A and Figure 3B).
Cases of CNH can be diagnosed based on the classic clinical appearance, location, and tenderness. Biopsy may be performed to confirm the diagnosis and rule out skin cancer, especially when there is extensive ulceration.
The differential diagnosis includes cutaneous malignancies such as squamous cell carcinoma and basal cell carcinoma. Though both can be inflamed and even ulcerated, they are rarely exquisitely tender. They also continue to slowly grow whereas CNH lesions reach a mature size and stop growing.
Actinic keratoses and cutaneous horns are often scaly and hyperkeratotic but are not ulcerated, inflamed or tender. Weathering nodules are asymptomatic, white to skin-colored and present along the free margin of the helix. They are bilateral and multiple in most people and can cause a scalloped appearance to the helical rim. Biopsy lacks epidermal disruption and inflammation.
Additional entities to consider include:
-Verrucae: verrucous surface with pinpoint capillary hemorrhages, rarely tender and rarely found on the ear
-Keratoacanthomas: rapidly growing, classic endophytic growth with squamous metaplasia on pathology
-Calcinosis cutis: multiple firm, white, yellow papules/nodules, chalky material. Look for underlying systemic disease or overt trauma, rarely isolated to one ear.
-Elastotic nodules: bilateral, semitranslucent, appear as aggregates of granular white-to-pink material usually with an overlying “orange peel” surface, associated with early actinic damage. Elastic stain can help distinguish these on biopsy
-Gouty tophi: white firm nodules with associated inflammation and tenderness on the helix, often multiple and with a history of untreated gout.
-Reactive perforating collagenosis: flesh-colored, umbilicated, dome-shaped papules or nodules as large as 10 mm in diameter with an adherent, keratinous plug, often pruritic and rarely located on the ear
-Pseudocyst of the auricle: typically presents unilaterally as a cystic enlargement of the anterior and lateral aspects of the auricle, most commonly in the scaphoid and triangular fossae. There is painless, fluctuant swelling which develops over 4-12 weeks.
Who is at Risk for Developing this Disease?
Lesions are most common in middle-aged white males with prevalence ratios between females to males of between 1:5 and 1:10. They are most common after the age of 40 years, with 90% of cases being reported between the ages of 50 and 80 years of age. Interestingly, most men present with lesions on the helix whereas most women and non-white men tend to have CNH nodules on the antihelix (Figure 4). Nodules are more common on the right ear, likely related to a higher prevalence of sleeping on the right side.
Several cases of childhood CNH have been reported as have isolated case studies in infants. CNH has also been reported in families including one case of monozygotic twins who both presented with CNH lesions within 36 days of each other. However, no genetic basis has been found.
Certain practices such as sleeping on the affected ear, extensive phone or cell phone usage, large ear pieces worn by telephone operators, and headphones have all been linked to cases of CNH. It has been observed that most patients have short hair at the time of diagnosis.
What is the Cause of the Disease?
The exact etiology of CNH is unknown. Predisposing factors include actinic damage, cold exposure, trauma, local ischemia, and, occasionally, radiotherapy. Many believe that the lesion is produced by ischemic necrosis of the dermis and generally occurs on the side the patient favors for sleep. The pressure generated during sleep is suspected as a major etiologic factor again linking this to a more classic pressure injury. There is lack of subcutaneous fat and limited vasculature in this area that can contribute to the vascular compromise. This causes dermal cartilage degeneration.
Some authors have argued that CNH may actually represent a type of perforating disorder. The idea is that the body attempts to eliminate the damaged dermal collagen transepidermally.
More recently, some investigators have tried to explain the pathogenesis by changes within the vasculature itself. A review of 16 cases showed arteriolar narrowing in the perichondrium region of the pinna most remote from the blood supply (the helix). There were several areas of vessel thickening and hyalinization. Systemic disease and frostbite are two conditions that are thought to cause arteriolar narrowing in the perichondrium of helix. This causes cartilage necrosis and extrusion, leading to severe local inflammation.
Why does it hurt? Although likely a secondary phenomenon, cases of CNH have been found to have evidence of nerve hyperplasia or increased small nerves adjacent to the involved cartilage. This was absent in control lesions and skin cancers. This could help to explain the exquisite tenderness often seen in patients with CNH. Interestingly, Ackerman et al. suggested that CNH is the “auricular analogue of prurigo nodule,” which can also have hypertrophied nerves.
Systemic Implications and Complications
CNH is generally not considered a sign of an underlying systemic disease process and there is no tendency to malignant change. However, there have been some reports of associated underlying systemic diseases, in particular, connective tissue diseases, in which CNH can be an associated sign or even presenting feature.
Such cases may be more common in pediatric or young adult female patients. In cases associated with systemic disease (autoimmune thyroiditis, lupus erythematosus, dermatomyositis, and scleroderma) microvascular injury may be an important underlying cause. On biopsy, baseline changes of CNH plus vasculopathic changes including vascular thrombosis and ectasia may be present.
Immune-based vasculopathy was thought to be involved in cases of dermatomyositis and scleroderma with CNH. The authors of this study recommend that CNH patients in the fourth decade or less of life should be investigated for underlying systemic disease. However, there are no specific recommendations regarding the extent of laboratory and other investigations that should be done.
Treatment options are summarized in Table I.
|Medical Treatment||Surgical Procedures||Physical Modalities|
|Modified CNH pillow||Excision of skin with partial cartilage excision||Carbon dioxide laser ablation|
|Topical Steroids||Shave of nodule with curettage of base|
|Intralesional Steroids||Punch excision|
|Topical antibiotics||Wedge resection|
|Collagen injections||Electrodessication and Curettage|
Optimal Therapeutic Approach for this Disease
Listen to the patient and get a sense of the evolution of this lesion. How quickly did it appear? Has it continued to grow or has it stabilized? Is it painful? Does it bleed? Is there a history of non-melanoma skin cancer in the past? If there are any concerns about the diagnosis, or it is acting more like a skin cancer, a shave biopsy would be recommended. Identify the age of the patient and determine if there are any other symptoms that could suggest an underlying systemic condition.
If the lesion is found in the absence of other symptoms, inquire about sleeping habits or devices that may come into contact with the ear. Explain that the lesion is related to pressure or sleeping on the affected side. If the history points to CNH, get a sense of how much this lesion interferes with daily living. If it is an incidental finding or minimally bothersome, conservative measures can be discussed such as a modified pillow or potent topical steroids.
A pressure-relieving prosthesis can be made by cutting a hole from the center of a bath sponge. This can then be held in place with a headband. One study using this type of device resulted in an 87% cure rate within 1 month of treatment. A special prefabricated pillow is available through the following address: CNH pillow, PO Box 1247, Abilene, TX, 79604. Phone (800) 255-7487 or (325) 672-2162 and Fax: (325) 677-2410. If there is associated telephone or headphone use, this must be changed.
Potent topical steroids can be applied to the area twice daily to help decrease inflammation. However, this would need to be done in conjunction with changes in pressure. Always instruct patients to apply the steroid only to the affected area, as high-potency steroids can cause skin atrophy. Some patients may even prefer to have the area injected with intralesional steroids prior to surgical treatment.
Corticosteroid injections have been reported to produce some benefit, usually using an injection of 0.1-0.2 ml of triamcinolone acetonide (10-40mg/ml). Many who receive corticosteroid injections often go on to need excision, but it is advocated by some as an initial treatment option.
If the lesion is exquisitely tender, interferes with sleep or the patient desires more definitive treatment, surgical excision is recommended and is the gold standard of treatment. Typically, only one cartilage surface is damaged beneath the underlying nodule, and some physicians advocate a partial excision procedure involving the cartilage rather than a wedge excision of the auricle.
A narrow elliptical excision or an incision creating a flap to expose the affected deep structures can be done, followed by shaving the affected rough cartilage so that is smooth with the adjacent unaffected cartilage. If there is a punctum or central ulcer, it should be included in the ellipse or incision. Shave excision with curettage of the underlying cartilage and punch excision of the nodule and affected cartilage have also been reported. An important point to remember when shaving the inflamed cartilage is that the remaining cartilage must be smooth to the touch because rough cartilage just roduces new pressure points.
Other options thar have been less well-documented include topical antibiotic ointments, intralesional collagen, electrodessication and curettage (ED&C), cryotherapy, and a recent study showing some efficacy using photodynamic therapy. Keep in mind, any recurrence is likely to be due to prominent cartilage if the surgical technique is not accurately performed.
Topical antibiotic ointments may reduce any pain caused by secondary infections. Complete symptom relief has been reported in five patients following injection of collagen implants. The hypothesis is that this provides improved cushion support. A recurrence rate of 31% in 142 patients using ED&C has been reported. Feathering of the edges is performed to eliminate rough edges of skin and cartilage.
Explain the etiology of this condition and the predisposing factors to the patient. Encourage them to avoid behaviors that would put them at higher risk, such as lying on that side when they sleep or using devices (headphones, etc) that put pressure on that area. Also, encourage the use of sunscreen, as it is thought that actinic damage and dermatoheliosis may also play a role.
If conservative measures are chosen for treatment, see the patient back within 2 months to ensure that they are improving. This will give you an opportunity to see the response to treatment as well as reevaluating the lesion. Is it continuing to grow? Has it changed? Do you need to biopsy now to rule out a skin cancer.
This also gives you the chance to offer different treatment options if the initial treatment has been unsuccessful. Is the lesion is more bothersome now and would the patient like surgical excision? If a surgical approach is chosen, the patient should be warned about the risks of bleeding, infection and scar with surgery. Daily dressing changes should be done and the patient should be seen back within one week to remove the sutures.
If the patient is completely healed after surgery or with conservative treatment, they can be seen back on an as needed basis. The occurrence of CNH does not necessitate periodic skin examinations, as it is not associated with malignant potential. The patient may, however, experience recurrence and can be followed up at that time.
Unusual Clinical Scenarios to Consider in Patient Management
As explained above, there are some presentations in younger patients that could potentially be associated with underlying systemic disease. Look for other symptoms or systems involved to direct any further investigations.
Finally, if a lesion were to recur in a similar area, do not automatically assume that it is a CNH. Take a thorough history and consider the possibility of skin cancer.
What is the Evidence?
Zuber, , TJ, Jackson, , E. “Chondrodermatitis Nodularis Chronica Helicis”. Arch Fam Med . vol. 8. 1999. pp. 445-7. (A very concise but thorough clinical review, including management options for chondrodermatitis nodularis chronica helicis. Very good intraoperative photographs and diagrams for surgical excision.)
Long, , D, Maloney , M. “Surgical Pearl: Surgical planning in the treatment of chondrodermatitis nodularis chronica helicis of the antihelix”. J Am Acad Dermatol . vol. 35. 1996. pp. 761-2. (Very detailed article explaining the surgical technique used for lesions on the antihelix with intraoperative photographs.)
Rajan, , N, Langtry, , JAA. “The punch and graft technique: a novel method of surgical treatment for chondrodermatitis nodularis helicis”. Br J Dermatol . vol. 157. 2007. pp. 744-7. (A study of 22 patients treated with punch excision of CNH lesions on the helix. Includes a comparison table with surgical cure rates using different surgical techniques.)
Moncrieff, , M, Sassoon, , EM. “Effective treatment of chondrodermatitis nodularis helicis using a conservative approach”. Br J Dermatol . vol. 150. 2004. pp. 892-4. (This study showed an 87% cure rate of CNH lesions when a modified pillow was used. A photograph of the pillow is provided.)
Cribier, , B, Scrivener , Y, Peltre , B. “Neural hyperplasia in chondrodermatitis nodularis chronica helicis”. J Am Acad Dermatol. vol. 55. 2006. pp. 844-8. (A report of 37 cases of CNH versus nonmelanoma skin cancers that were examined microscopically. This study points out the prominence of neural proliferation in CNH lesions and states that this likely accounts for the pain associated with these lesions.)
Oelzner, , S, Elsner, , P. “Bilateral chondrodermatitis nodularis chronica helicis on the free border of the helix in a woman”. J Am Acad Dermatol. vol. 49. 2003. pp. 720-721. A case report illustrating the rare presentation of bilateral CNH lesions as opposed to the more typical unilateral presentation.
Magro, , CM, Frambach , GE, Crowson , AN. “Chondrodermatitis nodularis helicis as a marker of internal syndromes associated with microvascular injury”. J Cutan Pathol . vol. 32. 2005. pp. 329-33. (A thorough review of 24 patients with CNH in whom there were underlying systemic diseases largely associated with vascular injury. They point out the younger age of these patients and recommend workup in patients with CNH who don’t fall into the typical demographics.)
Upile , T, Patell , NN, Jerges , W. “Advances in the understanding of chondrodermatitis nodularis chronica helices: the perichondrial vasculitis theory”. Clin Otolaryngol . vol. 34. 2009. pp. 147-50. (A review of 16 confirmed cases of CNH that revealed arteriolar narrowing in the perichondrium region of the helix.)
Rogers, , NE, Farris , PK, Wang , AR. “Juvenile chondrodermatitis nodularis helicis: A case report and literature review”. Ped Dermatol. vol. 20. 2003. pp. 488-90. (A case report of a 16-year-old female with CNH to illustrate the rare cases seen in children.)
Rex, , J. “Narrow elliptical skin excision and cartilage shaving for treatment of chondrodermatitis nodularis”. Dermatol Surg. vol. 32. 2006. pp. 400-04. (Retrospective study of surgical treatment of 74 patients with CNH. Includes a table with recurrence rates based on published cases of CNH treated with different modalities.)
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