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Chondrodermatitis Nodularis Helicis

Key Points

  • Chondrodermatitis nodularis helicis (CNH) is an uncommon, strikingly painful lesion on the helix or antihelix of the ear, which often appears as a crusted ulceration mimicking a cutaneous malignancy.
  • Diagnosis may be made by clinical exam and the symptom of pain, but biopsy of commonly performed to exclude cutaneous malignancies
  • Treatment is aimed at protecting the area with local measures, with a number of topical, intralesional, and ultimately surgical treatment approaches available

Introduction

Chondrodermatitis nodularis helicis (CNH) (or chondrodermatitis nodularis chronica helicis) is a painful lesion on the outer ear that is markedly more common in men than women, with a ratio approaching 10:1. CNH tends to occur in older patients, typically in patients in their 50s, 60s, or 70s. While the overall incidence is unknown, CNH is likely more common than recognized in the literature, due to underreporting. CNH occurs as a very painful ulceration, which may be crusted, arising almost invariably on the helix or antihelix of the ear. Some CNH lesions may appear dome shaped or have a raised rim, which may appear inflamed and erythematous. Typical lesions are small, ranging from 3 to 10mm in size. The vast majority appear as ulcerations, frequently with a thick serous crust. CNH is almost always unilateral, and patients often note the lesions are present on the side of their head that they sleep on. Due to their clinical appearance, CNH lesions are often mistaken for non-melanoma skin cancers (either basal cell or squamous cell carcinomas). Unlike those cutaneous malignancies, CNH is a benign entity. Pain is a critical feature, as CNH is invariably symptomatic, though patients may note a variety of types of pain arising from their lesion.

The differential diagnosis is primarily non-melanoma skin cancers (basal and squamous cell carcinoma). Other entities to consider include solar (actinic) keratoses, cutaneous horns, verruca vulgaris (warts), gouty tophi, or other cutaneous malignancies.

A small skin biopsy is sometimes performed to confirm the diagnosis and exclude those common cutaneous malignancies. Histologically, CNH is defined by increased vasculature, often seen at the edges of the ulcer, with variable amounts of mixed inflammatory cells. Cartilage, when present, may appear degenerated.

The precise cause of CNH is unknown; most feel that the lesion results from repetitive pressure and trauma on an area of photo-damaged skin. While the etiology of CNH is unknown, many have speculated that this may be a form of localized tissue ischemia, either due to local vascular factors (arteriole narrowing), external pressure or trauma, or cold-induced vasospasm. Most patients to not recall a specific inciting trauma, but external factors likely play a role in the disease. It is suspected that minor trauma, such as sustained localized pressure from pillows, telephones, headsets, or other activities of daily living may be sufficient to incite CNH.

Treatment is generally conservative, at least initially. Some patients will experience spontaneous improvement, however, it is more common that patients who seek physicians for this condition will experience persistent lesions if untreated, and most patients experience recurrences.

Initial therapy includes advising patients to avoid pressure to the area. Patients should use a soft pillow, including potentially a pillow with a gap for the ear or a pressure-offloading “doughnut.” They should avoid using telephones on that side and avoid headphones. There is limited data for a variety of local approaches, including light cryotherapy, topical corticosteroids, intralesional corticosteroids, or intralesional collagen/dermal fillers. Recently, topical nitroglycerin has been demonstrated to help some cases of CNH in a small study. Surgical options have long been the standard for recalcitrant cases. Certain lasers, typically ablative lasers, have been used. Curettage and electrodestruction has also been reported to help. In recurrent, refractory cases, surgical excision of the lesion and associated sub-ulcer cartilage is often very effective.

Initial Evaluation

Differential diagnosis

Basal cell carcinoma

Squamous cell carcinoma

Solar (actinic) keratosis

Treatment

First-line therapy: All patients should be counseled to protect the ear and avoid repeated external trauma. Initial therapy includes advising patients to avoid pressure to the area. Patients should use a soft pillow, including potentially a pillow with a gap for the ear or a pressure-offloading “doughnut.” Over-the-counter or custom-made protective ear prostheses exist. Patients may also attempt to use a sponge or foam padding, with a hole cut for the affected ear, which can be held in place by a headband or gentle wrap They should avoid using telephones on that side and avoid headphones. Conservative management alone with protective padding and trauma avoidance can lead to dramatic improvement, with some studies showing close to 90% of CNH lesions healing with this approach. Patients who fail to respond should be questioned about adherence, as noncompliance is a frequent cause of treatment failure with this approach.

There is limited data for a variety of local approaches, including light cryotherapy, topical corticosteroids, intralesional corticosteroids, or intralesional collagen/dermal fillers. Recently, topical nitroglycerin has been demonstrated to help some cases of CNH in a small study. Surgical options have long been the standard for recalcitrant cases. Certain lasers, typically ablative lasers, have been used. Curettage and electrodestruction has also been reported to help. In recurrent, refractory cases, surgical excision of the lesion and associated sub-ulcer cartilage is often very effective.

Chondrodermatitis nodularis helicis (failing conservative approach)

First steps

  • Topical corticosteroids may help in some cases, with most data existing for mid-to high-potency topical steroids (betamethasone valerate 0.1%).
  • Intralesional triamcinolone injections may be helpful; while a variety of concentrations have been reported (5 mg/cc to 40 mg/cc, 0.1 to 0.2 mL injected), given the thin skin of the area and frequent solar damage, we suggest starting with the lower concentrations (5 mg/cc, 0.1 mL).
  • Topical nitroglycerine 2% has been shown to help moderate numbers of patients in a small case series, with patients experiencing improvement within weeks to months.

Ancillary steps

  • Maintain pressure off-loading protective devices and trauma avoidance throughout therapeutic intervention.

Subsequent steps

  • Surgical options include a number of variations of definitive lesion removal, ranging from simple excision to excision of the nodule and paring or excision of the altered sub-ulcer cartilage (wedge resection), which may require a flap or other tissue manipulation to cover the affected collagen; some surgeons utilize cartilage replacement or grafting as well.

Pitfalls

  • Lesions that fail to respond to therapy may represent alternative diagnoses, and physicians should consider biopsy and histologic evaluation to confirm the diagnosis in resistant cases.
  • Recurrences are common; when they recur, physicians should revisit the counseling regarding protective measures and ensure patients are working to prevent repetitive trauma that can incite lesions of CNH.
  • Conservative management and topical therapy is very effective, but traditionally the mainstay of therapy in recalcitrant cases remains surgical; it is important to treat the underlying cartilage as well as the ulcer in these cases.

Clinical Case

Case 1

  • 70-year-old healthy man
  • No significant past medical history
  • Review of systems is noncontributory
  • Social history is noncontributory; does not smoke or drink alcohol
  • Presents for management of 4-month painful erosion on the helical rim of his left ear

Initial evaluation

  • Healthy appearing male
  • Tender mildly erythematous dome-shaped papule with central ulceration and crusting
  • Diagnosis: chondrodermatitis nodularis helicis
  • Recommend pressure-offloading “doughnut” ear prosthesis, softer pillow, sleeping on the opposite side, and avoiding use of telephone pressing up against that ear or the use of headphones
  • Follow-up in two to three months

Follow-up evaluation

  • Patient notes some relief but the lesion persists
  • Biopsy to exclude non-melanoma skin cancer: pathology consistent with chondrodermatitis nodularis helicis
  • Intralesional triamcinolone 5 mg/cc is injected into the site
  • Follow-up if fails to resolve
  • Additional treatment options include: topical nitroglycerin, surgical therapy

References

Flynn V, Chisholm C, Grimwood R (2011) Topical nitroglycerin: a promising treatment option for chondrodermatitis nodularis helicis. J Am Acad Dermatol, 65:531-536.

Moncrieff M, Sassoon EM (2004) Effective treatment of chondrodermatitis nodularis chronica helicis using a conservative approach. Br J Dermatol, 150:892-894.

Thompson LDR (2007) Chondrodermatitis nodularis helicis. Ear Nose Throat, 8:734-735.

Upile T, Patel NN, Jerjes W, Singh NU, Sandison A, Michaels L (2009) Advances in the understanding of chondrodermatitis nodularis chronica helices: the perichondrial vasculitis theory. Clin Otolaryngol, 34:147-150.

Wagner G, Liefeith J, Sachse MM (2011) Clinical appearance, differential diagnoses, and therapeutical options of chondrodermatitis nodularis chronica helicis Winkler. J Dtsch Dermatol Ges, 9:287-291.

Zuber TJ, Jackson E (1999) Chondrodermatitis nodularis chronica helicis. Arch Fam Med, 8:445-447.