You are in: eMedicine Specialties > Dermatology > PHOTO-RELATED DISEASES Chondrodermatitis Nodularis HelicisArticle Last Updated: Jul 23, 2007AUTHOR AND EDITOR INFORMATIONSection 1 of 10
  Author: Victor J Marks, MD, Associate, Department of Dermatology, Section Chief, Dermatologic Surgery, Geisinger Health System Victor J Marks 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 Coauthor(s): Christine A Papa, DO, Instructor, Department of Dermatology, Kennedy Memorial Health System, University of Medicine and Dentistry of New Jersey; Hakeem Sam, MD, PhD, FRCPC, Fellow in Procedural Dermatology (Mohs Micrographic Surgery), Division of Dermatology, Geisinger Health Center Editors: Kelly M Cordoro, MD, Fellow and Clinical Instructor, Department of Pediatric Dermatology, University of California at San Francisco; Assistant Professor (On Educational Leave), Assistant Program Director for Resident Medical Education, Department of Dermatology, University of Virginia School of Medicine; David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Director Dermatology Residency Training Program, Scott and White Clinic; Edward F Chan, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center Author and Editor Disclosure Synonyms and related keywords: CNH, CNH pillow, ear lesion, helix lesion, antihelix lesion, ear sore, pressure sore on ear, external ear lesion INTRODUCTIONSection 2 of 10
  BackgroundChondrodermatitis nodularis chronica helicis (CNH) is a common, benign, painful condition of the helix or antihelix of the ear. CNH more often affects middle-aged or older men, but cases in women occur also. Pediatric cases of CNH have been reported, and one was recently reviewed by Grigoryants et al.1 The youngest affected patient initially presented at age 3 years with a tender and persistent lesion on the right antihelix. An excisional biopsy of this lesion at age 9 years showed histologic features consistent with CNH. PathophysiologyThe exact cause of CNH 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. Focal pressure on the stiff cartilage most likely produces damage to the cartilage and overlying skin in most cases. Anatomic features of the ear, as listed above, prevent adequate healing and lead to secondary perichondritis. The right ear is more commonly involved. FrequencyUnited StatesExact incidence is unknown. Newcomer et al2 found CNH to be the most common condition of the external ear seen in their clinic. Mortality/MorbiditySpontaneous resolution is the exception; remissions may occur, but the condition usually continues unless treated adequately. RaceCNH occurs most commonly in fair-skinned individuals with severely sun-damaged skin; however, it can occur in all races. SexWhile CNH mostly occurs in men, 10-35% of cases involve women. AgeCNH can occur in patients at any age but mostly affects middle-aged to older individuals. Age at onset is similar in men and women. CLINICALSection 3 of 10
HistoryThe classic presentation 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. PhysicalNodules 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 exist. Size may range from 3-20 mm. The right ear is affected more commonly than the left, and bilateral distribution occasionally occurs. 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. CausesThe cause of CNH is not certain; however, pressure, cold, actinic damage, and repeated trauma have been implicated. Sleeping on the affected side is usually the important etiologic factor. Injury to the underlying cartilage and/or skin from pressure appears to be the most important etiologic factor. DIFFERENTIALSSection 4 of 10
Actinic Keratosis Basal Cell Carcinoma Cystic chondromalacia Elastotic nodules of the ears Keratoacanthoma Squamous Cell Carcinoma Other Problems to Be ConsideredA clinically distinctive feature of CNH is that very often the patient presents for help because of the pain associated with the skin lesions. In contrast, cutaneous tumors in the differential diagnosis are usually painless, even when ulcerated. Consistent with this observation, Cribier et al3 found histologic evidence of nerve hyperplasia or increased numbers of small nerves adjacent to the involved cartilage primarily in CNH cases but not in control tumors. WORKUPSection 5 of 10
ProceduresBiopsy is indicated if the diagnosis is in doubt. Often, biopsy is necessary to differentiate CNH from basal cell carcinoma or squamous cell carcinoma because many patients with CNH have chronic solar damage and a history of skin cancer. Histologic FindingsThe 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. TREATMENTSection 6 of 10
Medical CareMedical management is often unsatisfactory. The primary goal should be to relieve or eliminate pressure at the site of the lesion. This is often difficult because of the patient's preference or necessity to sleep on the side of the lesion. A pressure-relieving prosthesis can be fashioned by cutting a hole from the center of a bath sponge. This device can then be held in place with a headband. A special prefabricated pillow is available that helps relieve pressure on the ear. For more information on this pillow, contact: 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. If specific efforts to relieve pressure are unsuccessful, surgical approaches almost always are needed. Surgical CareVarious procedures have been used in the treatment of CNH. These procedures include wedge excision, curettage, electrocauterization, carbon dioxide laser ablation, and excision of the involved skin and cartilage. 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,4 provides excellent curative, functional, and cosmetic results.
ConsultationsDermatologists, dermatologic surgeons, and Mohs micrographic surgeons are knowledgeable about this condition and the treatments described above. ActivityIf 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. FOLLOW-UPSection 7 of 10
PrognosisThe prognosis for patients with CNH is excellent. MISCELLANEOUSSection 8 of 10
Medical/Legal PitfallsThe most important medicolegal pitfall associated with CNH may be the misdiagnosis of a true malignancy as CNH, thereby resulting in failure to perform a biopsy and to treat it as a malignancy. MULTIMEDIASection 9 of 10
REFERENCESSection 10 of 10
Chondrodermatitis Nodularis Helicis excerpt Article Last Updated: Jul 23, 2007 | ||||||||||||||||||||||||||||||||||||||||||
