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Chondrodermatitis Nodularis Helicis
Updated: Jan 15, 2010
Introduction
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.
Classic chondrodermatitis nodularis chronica helicis on the superior helix.
Close-up view of classic chondrodermatitis nodularis chronica helicis.
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
Frequency
United States
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
Mortality/Morbidity
Spontaneous resolution is the exception; remissions may occur, but chondrodermatitis nodularis chronica helicis usually continues unless adequately treated.
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.
Clinical
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.
Physical
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.
Causes
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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| Follow-up: Chondrodermatitis Nodularis Helicis |
| Multimedia: Chondrodermatitis Nodularis Helicis |
| References |
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References
Rex, J., Ribera, M., Bielsa, I., et al. Narrow Eliptical Excision and Cartilage Shaving for Treatment of Chondrodermatitis Nodularis. Dermatologic Surgery. 2006;32:400-404.
Grigoryants V, Qureshi H, Patterson JW, Lin KY. Pediatric chondrodermatitis nodularis helicis. J Craniofac Surg. Jan 2007;18(1):228-31. [Medline].
Tsai TH, Lin YC, Chen HC. Infantile chondrodermatitis nodularis. Pediatr Dermatol. May-Jun 2007;24(3):337-9. [Medline].
Upile T, Patel NN, Jerjes W, Singh NU, Sandison A, Michaels L. Advances in the understanding of chondrodermatitis nodularis chronica helices: the perichondrial vasculitis theory. Clin Otolaryngol. Apr 2009;34(2):147-50. [Medline].
Chan HP, Neuhaus IM, Maibach HI. Chondrodermatitis nodularis chronica helicis in monozygotic twins. Clin Exp Dermatol. Apr 2009;34(3):358-9. [Medline].
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Affleck AG. Surgical treatment of chondrodermatitis nodularis chronica helicis: conservation of normal tissue is important for optimal esthetic outcome. J Oral Maxillofac Surg. Oct 2008;66(10):2194. [Medline].
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Further Reading
Keywords
chondrodermatitis nodularis helicis, chondrodermatitis nodularis chronica helicis, CNH, CNH pillow, ear lesion, helix lesion, antihelix lesion, ear sore, pressure sore on ear, external ear lesion, dermatomyositis, autoimmune thyroiditis, lupus erythematosus, dermatomyositis, scleroderma, treatment, diagnosis
