Chondrodermatitis Nodularis Helicis

Background

Chondrodermatitis nodularis chronica helicis (CNH) is a common, benign, painful condition of the helix or antihelix of the ear. Chondrodermatitis nodularis chronica helicis more often affects middle-aged or older men, but cases are also reported in women. In a 2006 report by Rex et al, of 74 patients treated for chondrodermatitis, 72.9% of patients were men while 16.2% were women.^[1] Pediatric cases of chondrodermatitis nodularis chronica helicis have been reported, and one was reviewed by Grigoryants et al.^[2]

An additional case has been reported in a 9-month-old infant. History associated with this case indicated the infant slept on the ear of occurrence, where she developed 2 nodules. The infant recently had started sleeping on a hard pillow, which contributed to the lesion. An excisional biopsy of this lesion at 9 months showed histologic features consistent with chondrodermatitis nodularis. The papule resolved with a change in sleeping position over 6 months.^[3]

Clinical images of chondrodermatitis nodularis chronica helicis are below.

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Classic chondrodermatitis nodularis chronica helicis on the superior helix.

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Close-up view of classic chondrodermatitis nodularis chronica helicis.

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Pathophysiology

The exact cause of chondrodermatitis nodularis chronica helicis is unknown, although most authorities believe it is caused by prolonged and excessive pressure. Several anatomic features of the ear predispose persons to the development of this condition. The ear has relatively little subcutaneous tissue for insulation and padding, and only small dermal blood vessels supply the epidermis, dermis, perichondrium, and cartilage. Dermal inflammation, edema, and necrosis from trauma, cold, actinic damage, or pressure probably initiate the disease. In most cases, focal pressure on the stiff cartilage most likely produces damage to the cartilage and overlying skin. Anatomic features of the ear, as listed above, prevent adequate healing and lead to secondary perichondritis. The right ear is more commonly involved. A 2009 report concluded that specific perichondrial arteriolar changes may be the cause of chondrodermatitis nodularis chronica helicis.^[4]

Although most authors in the past have regarded chondrodermatitis nodularis chronica helicis as an idiopathic disorder with no systemic associations, exceptions to this have been noted. Chondrodermatitis nodularis chronica helicis may occasionally be associated with autoimmune or connective-tissue disorders, including autoimmune thyroiditis, lupus erythematosus, dermatomyositis, and scleroderma. Such cases may be more common in pediatric or young adult female patients. A 2009 report detailed chondrodermatitis nodularis chronica helicis in monozygotic twins, suggesting a possible hereditary factor.^[5]

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Etiology

The cause of chondrodermatitis nodularis chronica helicis is not certain; however, pressure, cold, actinic damage, and repeated trauma have been implicated. Sleeping on the affected side is usually an important etiologic factor. Injury to the underlying cartilage and/or skin from pressure appears to be a primary etiologic factor.

In cases of chondrodermatitis nodularis chronica helicis associated with systemic autoimmune disorders, evidence indicates that microvascular injury may be an important underlying cause. Interestingly, in one such patient, chondrodermatitis nodularis chronica helicis was the first clinical manifestation of the patient’s autoimmune disorder. Local factors, such as pressure and repeated trauma, likely compound the microvascular injury and subsequent ischemia of the underlying cartilage involved in chondrodermatitis nodularis chronica helicis.

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Epidemiology

Frequency

The exact incidence of chondrodermatitis nodularis chronica helicis is unknown. Newcomer et al found chondrodermatitis nodularis chronica helicis to be the most common condition of the external ear seen in their clinic.^[6] The incidence in patients age 60-80 years is predominantly male, while cases presenting in young females appear to be associated with evidence of underlying systemic illness in some instances.^[7]

Race

Chondrodermatitis nodularis chronica helicis occurs most commonly in fair-skinned individuals with severely sun-damaged skin; however, it can occur in persons of any races.

Sex

Although chondrodermatitis nodularis chronica helicis mostly occurs in men, 10-35% of cases involve women.

Age

Chondrodermatitis nodularis chronica helicis can occur in patients of any age but mostly affects middle-aged to older individuals. Age at onset is similar in men and women.

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Prognosis

The prognosis for patients with chondrodermatitis nodularis chronica helicis (CNH) is excellent, although long-term morbidity is common. Spontaneous resolution is the exception; remissions may occur, but chondrodermatitis nodularis chronica helicis usually continues unless adequately treated.

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History

The classic presentation of chondrodermatitis nodularis chronica helicis (CNH) is a middle-aged to elderly man with a spontaneously appearing painful nodule on the helix or antihelix. The nodule usually enlarges rapidly to its maximum size and remains stable. Onset may be precipitated by pressure, trauma, or cold. When asked, the patient usually admits to sleeping on the affected side.

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Physical Examination

Nodules are firm, tender, well demarcated, and round to oval with a raised, rolled edge and central ulcer or crust. Removal of the crust often reveals a small channel. Color is similar to that of the surrounding skin, although a thin rim of erythema may be noted. Size may range from 3-20 mm. The right ear is affected more commonly than the left, and occasionally bilateral distribution is reported.^[8] Lesions develop on the most prominent projection of the ear. The most common location is the apex of the helix. Distribution on the antihelix is more common in women.

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Laboratory Studies

Magro et al. recommend that chondrodermatitis patients in the fourth decade of life should be investigated for underlying systemic disease. This recommendation originates from a study conducted by Magro et al that included 24 patients with chondrodermatitis at a mean age of 43 years who also had increased association with collagen-vascular disease, scleroderma, hypertension, thyroid disease, and heart disease.^[7]

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Other Tests

Performing skin biopsy and visualizing characteristic pathologic changes with light microscopy are the standard method of diagnosis.

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Procedures

Biopsy is indicated if the diagnosis of chondrodermatitis nodularis chronica helicis (CNH) is in doubt. Often, biopsy is necessary to differentiate chondrodermatitis nodularis chronica helicis from basal cell carcinoma or squamous cell carcinoma because many patients with chondrodermatitis nodularis chronica helicis have chronic actinic damage and a history of skin cancer.

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Histologic Findings

The histologic changes are similar to those seen in decubitus ulcers, but on a smaller scale. Within the central portion of a shave biopsy, the epidermis usually is ulcerated. At the periphery, intact epidermis is edematous and acanthotic. The dermis below the ulceration demonstrates homogeneous acellular collagen degeneration with fibrin deposition. Granulation tissue flanks the zone of necrosis on both sides. A focus of cartilaginous degeneration may be present, although it is usually minimal. Changes of proliferative fasciitis may rarely be noted.^[10]

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Medical Care

Medical management of chondrodermatitis nodularis chronica helicis (CNH) is often unsatisfactory. The primary goal should be to relieve or eliminate pressure at the site of the lesion.^[11] This is often difficult because of the patient's preference or necessity to sleep on the side with the lesion. A pressure-relieving prosthesis can be fashioned by cutting a hole from the center of a bath sponge.^[12] This device can then be held in place with a headband. Additionally, a self-adhering foam sponge (Reston Foam, 3M, St. Paul, Minn) can be customized and shaped into either a rectangular or crescentic shape in order to maximize offloading. The foam is then applied to the non–hair-bearing postauricular scalp during sleep. This affordable technique helps to ensure that shifting of the pressure-relieving foam is minimal.^[13]

A special prefabricated pillow is available that helps relieve pressure on the ear. For more information on this pillow, contact the following:

CNH Pillow

PO Box 1247

Abilene, TX 79604

Phone: (800) 255-7487 or (325) 672-2162

Fax: (325) 677-2410.

Topical antibiotics may relieve pain caused by secondary infections. Topical and intralesional steroids also may be effective in relieving discomfort. Collagen injections may bring relief by providing cushioning between the skin and cartilage. Cryotherapy also has been used as a treatment modality. Topical diltiazem and topical nitroglycerin 2% twice daily has been shown to relieve both symptoms and appearance.^{[14, 15]}

Nitroglycerin patches have also been used.^[16]

If specific efforts to relieve pressure are unsuccessful, surgical approaches almost always are needed.^[17]

A series of photos demonstrating the self-adhering foam offloading technique is shown below.

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Self-adhering foam in a curvilinear shape is placed on the non–hair-bearing postauricular scalp.

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A rectangular piece of self-adhering foam is placed on the non–hair-bearing postauricular scalp.

Photodynamic therapy is promising, although multiple treatment sessions may be required.^{[17, 18]}

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Surgical Care

Various procedures have been used in the treatment of chondrodermatitis nodularis chronica helicis. These procedures include wedge excision, curettage, electrocauterization, photodynamic therapy, carbon dioxide laser ablation, and excision of the involved skin and cartilage.^{[19, 20, 21]} In general, the recurrence rate is high unless the underlying focus of damaged cartilage is removed and the pressure relieved. Treatment with cartilage removal alone, as described by Lawrence, provides excellent curative, functional, and cosmetic results.^[22]

Perform the procedure to remove cartilage with the patient under local anesthesia using 0.5-1% buffered lidocaine with epinephrine 1:200,000.

For lesions on the helix, make an incision on either side of the nodule running along the rim of the helix. Make the incision where the scar can be best hidden. Bluntly dissect and reflect the skin from the perichondrium to reveal the helix cartilage. Trim the cartilage immediately under the ulcer with a flat shaving technique using a scalpel to a depth of approximately 3 mm. The remaining cartilage must be smooth to touch because rough cartilage may produce pressure points. After hemostasis is achieved, reapproximate and suture the skin.

For lesions on the antihelix, raise a 3-sided flap that is approximately 25 mm wide and 15 mm long, with its attached margin directed toward the helix. Expose the perichondrium-covered cartilage, and excise cartilage with a scalpel until all edges are smooth to touch. Obtain hemostasis, and reapproximate and suture the flap. Conservation of the normal tissue is important for esthetic outcome.^[23]

Another variation for treating lesions on the antihelix involves excising the affected cartilage and then closing the defect with bilateral advancement flaps that are based inferiorly and superiorly along the antihelix.^[24]

Rajan et al reported a novel approach to the surgical treatment of chondrodermatitis for small, localized lesions. The area is anesthetized with 1% lidocaine with epinephrine. A punch biopsy instrument is used, the diameter of which is such that the lesion is encompassed by the punch. The punch is applied perpendicular to the skin surface and advanced until a deep punch of the underlying cartilage is cut. The specimen of skin is excised and sent for histopathological evaluation. The posterior auricular donor site is chosen for reasonable skin color match. The same size punch tool is used to harvest the full-thickness skin graft, and the donor site is closed with interrupted sutures. The graft is sutured with 6-0 suture after proper preparation of the graft.^[25]

Cognetta et al described the "triangular window technique," which combines surgical treatment and biopsy in the same procedure. A triangular-shaped incision is made around the affected cartilage (the apex directed posteriorly with the wide base hinge located at the anterior helical rim). The affected cartilage is removed and the triangular-shaped skin is sutured to its original position. A 2-mm punch biopsy is then taken in the center of the cutaneous triangular window to be submitted to pathology and to serve as a "weep hole" for any drainage.^[26]

In addition, Affleck, in an editorial comment, reiterates that full wedge excision of chondrodermatitis nodularis is often not necessary and can be avoided in favor of more conservative excision.^[27] Conservative excision of the helical rim allows for repair with a chondrocutaneous helical rim advancement flap, as described by Ramsey et al, which leads to superior esthetic outcome and simpler reconstruction for helical rim defects.^[28]

These simple surgical procedures provide excellent curative, functional, and cosmetic results with low morbidity and recurrence rates. If the disease recurs, the procedures may be repeated without causing deformity to the ear.

A series of surgical images from a single patient is below.

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Chondrodermatitis nodularis chronica helicis on the antihelix.

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Taken during surgery, this photo demonstrates reflection of the skin, which reveals the underlying perichondrium and cartilage. After the cartilage is....

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Taken 6 months after surgery.

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Consultations

Dermatologists, dermatologic surgeons, and Mohs micrographic surgeons are knowledgeable about this condition and the treatments described above.

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Activity

If trauma, pressure necrosis, cold, or sun exposure is suspected as an exacerbating factor, then reduction of exposure is beneficial. If the patient sleeps on the affected side, then changing sides or using pressure-relieving pillows or pads may be helpful. Such measures often are difficult for the patient, and surgery may be the desired alternative.

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Complications

While surgical intervention is a mainstay of therapy, multiple surgeries may be necessary. At times, removal of underlying protuberant cartilage results in adjacent protuberances that can be site(s) of recurrence of chondrodermatitis nodularis chronica helicis (CNH), owing to a change in pressure points.

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