Practice Essentials
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. [1, 2, 3] 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. [4] The etiology of CNH is unknown and likely multifactorial. Most authorities believe it is caused by prolonged and excessive pressure.The thinning skin and cartilage seen with aging may be a contributing factor. [5]
Pediatric cases of chondrodermatitis nodularis chronica helicis have been reported, and one was reviewed by Grigoryants et al. [6] 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. [7]
Clinical images of chondrodermatitis nodularis chronica helicis are below.
Treatment is initially conservative and includes pressure avoidance or pressure relief by sleeping on the opposite side, padding of the ear with sponges or foam, and use of a donut pillow. [5] 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. Other interventions include topical nitroglycerin gel, cryotherapy, and photodynamic therapy. Wedge resection should be considered when the lesion recurs despite multiple attempts of less invasive interventions. If specific efforts to relieve pressure are unsuccessful, surgical approaches almost always are needed. [8]
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 contribute to the disease, especially in the case of actinic damage. 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. [10]
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.
In a case series of 215 patients with CNH, 15.34% were also diagnosed with chronic inflammatory and autoimmune diseases (CADs). The most frequent CADs reported were polymyalgia rheumatica (3%), psoriasis/psoriatic arthritis (2%), rheumatoid arthritis (1%), CREST syndrome, vitiligo, and chronic dermatitis (< 1%). [11]
A 2009 report detailed chondrodermatitis nodularis chronica helicis in monozygotic twins, suggesting a possible hereditary factor. [12]
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. Cases of CNH resulting from cell phone use have been reported. [13]
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.
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. [14] 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. [15]
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.
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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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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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 removed, the flap is reapproximated and sutured.
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Taken 6 months after surgery.
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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.
