Chondrodermatitis Nodularis Helicis 

  • Author: Victor J Marks, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jan 15, 2010
 

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 helicClassic chondrodermatitis nodularis chronica helicis on the superior helix. Close-up view of classic chondrodermatitis nodularClose-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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Epidemiology

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.

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Contributor Information and Disclosures
Author

Victor J Marks, MD  Associate, Department of Dermatology, Section Chief, Dermatologic Surgery, Geisinger Health System

Victor J Marks, MD is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Micrographic Surgery and Cutaneous Oncology, American College of Physicians, American Medical Association, and Pennsylvania Medical Society

Disclosure: Nothing to disclose.

Coauthor(s)

Russell Scott Akin, MD  Fellow, Department of Dermatology, Geisinger Health System

Russell Scott Akin, MD is a member of the following medical societies: Alpha Omega Alpha

Disclosure: Nothing to disclose.

Christine A Papa, DO  Instructor, Department of Dermatology, Kennedy Memorial Health System, University of Medicine and Dentistry of New Jersey

Disclosure: Nothing to disclose.

Specialty Editor Board

Kelly M Cordoro, MD  Assistant Professor, Pediatric and Adult Dermatology, Department of Dermatology, University of California at San Francisco

Kelly M Cordoro, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Association of Professors of Dermatology, Dermatology Foundation, Medical Society of Virginia, National Psoriasis Foundation, Society for Pediatric Dermatology, and Women's Dermatologic Society

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Edward F Chan, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine

Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

  1. 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.

  2. Grigoryants V, Qureshi H, Patterson JW, Lin KY. Pediatric chondrodermatitis nodularis helicis. J Craniofac Surg. Jan 2007;18(1):228-31. [Medline].

  3. Tsai TH, Lin YC, Chen HC. Infantile chondrodermatitis nodularis. Pediatr Dermatol. May-Jun 2007;24(3):337-9. [Medline].

  4. 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].

  5. Chan HP, Neuhaus IM, Maibach HI. Chondrodermatitis nodularis chronica helicis in monozygotic twins. Clin Exp Dermatol. Apr 2009;34(3):358-9. [Medline].

  6. Newcomer VD, Steffen CG, Sternberg TH, Lichtenstein L. Chondrodermatitis nodularis chronica helicis; report of ninety-four cases and survey of literature, with emphasis upon pathogenesis and treatment. AMA Arch Derm Syphilol. Sep 1953;68(3):241-55. [Medline].

  7. Magro CM, Frambach GE, Crowson AN. Chondrodermatitis nodularis helicis as a marker of internal disease [corrected] associated with microvascular injury. J Cutan Pathol. May 2005;32(5):329-33. [Medline].

  8. Oelzner S, Elsner P. Bilateral chondrodermatitis nodularis chronica helicis on the free border of the helix in a woman. J Am Acad Dermatol. Oct 2003;49(4):720-2. [Medline].

  9. Cribier B, Scrivener Y, Peltre B. Neural hyperplasia in chondrodermatitis nodularis chronica helicis. J Am Acad Dermatol. Nov 2006;55(5):844-8. [Medline].

  10. Moncrieff M, Sassoon EM. Effective treatment of chondrodermatitis nodularis chronica helicis using a conservative approach. Br J Dermatol. May 2004;150(5):892-4. [Medline].

  11. Kromann N, Hoyer H, Reymann F. Chondrodermatitis nodularis chronica helicis treated with curettage and electrocauterization: follow-up of a 15-year material. Acta Derm Venereol. 1983;63(1):85-7. [Medline].

  12. Lawrence CM. The treatment of chondrodermatitis nodularis with cartilage removal alone. Arch Dermatol. Apr 1991;127(4):530-5. [Medline].

  13. 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].

  14. Rajan N, Langtry JA. The punch and graft technique: a novel method of surgical treatment for chondrodermatitis nodularis helicis. Br J Dermatol. Oct 2007;157(4):744-7. [Medline].

  15. 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].

  16. Ramsey ML, Marks VJ, Klingensmith MR. The chondrocutaneous helical rim advancement flap of Antia and Buch. Dermatol Surg. Nov 1995;21(11):970-4. [Medline].

  17. Abell E. Inflammatory diseases of the epidermal appendages and of cartilage. In: Lever WF, ed. Lever's Histopathology of the Skin. 8th ed. Philadelphia, Pa: Lippincott-Raven; 1997:416-7.

  18. Arndt KA. Chondrodermatitis helicis. In: Fitzpatrick TB, Eisen AZ, Wolff K, et al, eds. Dermatology in General Medicine. 4th ed. New York, NY: McGraw-Hill; 1993:1149-51.

  19. Heffner DK, Hyams VJ. Cystic chondromalacia (endochondral pseudocyst) of the auricle. Arch Pathol Lab Med. Aug 1986;110(8):740-3. [Medline].

  20. Lawrence CM. Chondrodermatitis nodularis. In: Arndt KA, LeBoit PE, Robinson JK, Wintroub BU, eds. Cutaneous Medicine and Surgery: An Integrated Program in Dermatology. Vol 1. Philadelphia, Pa: WB Saunders; 1996:507-11.

  21. Long D, Maloney ME. Surgical pearl: surgical planing in the treatment of chondrodermatitis nodularis chronica helicis of the antihelix. J Am Acad Dermatol. Nov 1996;35(5 Pt 1):761-2. [Medline].

  22. Moschella SL, Cropley TG. Diseases of the mononuclear phagocytic system: The so-called reticuloendothelial system. In: Moschella SL, Hurley HJ, eds. Dermatology. Vol 1. Philadelphia, Pa: WB Saunders; 1992:1061-2.

  23. Requena L, Aguilar A, Sánchez Yus E. Elastotic nodules of the ears. Cutis. Dec 1989;44(6):452-4. [Medline].

 
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Classic chondrodermatitis nodularis chronica helicis on the superior helix.
Close-up view of classic chondrodermatitis nodularis chronica helicis.
Chondrodermatitis nodularis chronica helicis on the antihelix.
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.
Taken 6 months after surgery.
 
 
 
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