Repeated pressure can lead to vascular changes, but underlying vascular disease also can predispose a person to CNH at a younger age. A striking case of bilateral CNH was reported in an 8-year-old girl with a known history of dermatomyositis.7 Furthermore, in 24 patients with CNH (mean age, 43 years), Magro et al9 observed an association between CNH and collagen vascular disease, scleroderma, hypertension, thyroid disease, and heart disease, with a higher incidence of any of these medical problems in younger patients. Therefore, screening all patients presenting with CNH, particularly those younger than their fourth decade, for underlying vasculopathy and an autoimmune connective tissue disorder is advised.9
Other findings of CNH reported in the literature include loss of elastic fibers in the central area of degenerated dermal collagen and nerve hyperplasia, which might account for pain.6 Many of the biopsies in cases of CNH reported in the literature also demonstrate perichondrial fibrous thickening, mild chronic inflammation, and degenerative changes in collagen, including hyalinization and rarely necrosis and calcification. Skin at the periphery of the lesion usually contains granulation tissue, with a mild to moderate inflammatory infiltrate and dilated vessels extending beyond the lesion.2
Genetics might play a role in the disorder, which is suggested by the occurrence of CNH in monozygotic twins10 and in an otherwise healthy 16-year-old adolescent girl with CNH of the right ear who screened negative for underlying connective tissue disease—serologic tests included antinuclear antibody, anti-Sm, anti-SCL-70, anti-Ro, anti-La, and rheumatoid factor—but who had a family history of a maternal grandmother with CNH, also on the right side.6
In the present case, there was no family history or signs and symptoms of underlying systemic disease at the time of diagnosis. The social history revealed excessive occupational sun exposure, habitually wearing a tight visor, and frequent cell phone use, all of which might have contributed to CNH.
Management
Medical management is geared toward relieving pressure at the site of the lesion, which was accomplished by use of an off-loading, ring-shaped, foam pillow at night in a 9-month-old girl with CNH, in which the smaller of her 2 left-sided lesions completely resolved by 6-month follow-up.8 However, it often is difficult to achieve adequate relief of pressure because of the patient’s preference for holding a cell phone to a particular ear or unconscious sleeping habits that perpetuate lesions. There are many creative physical interventions to offload aggravating pressure from the area during sleep. A prosthesis can be fashioned by cutting a hole from the center of a bath sponge and securing it with a headband,11 or a crescentic or rectangular piece of self-adhering foam sponge can be applied to the non–hair-bearing postauricular scalp during sleep.12 Topical antibiotics might relieve pain caused by secondary infection.
Surgical intervention, with or without placement of a full-thickness skin graft, is the mainstay of therapy. Excision was performed in 3 previously reported pediatric cases, with no recurrence reported at 6- to 24-month follow-up. Other treatments employed to varying effect include topical and intralesional steroids, collagen injection, cryotherapy, nitroglycerin paste 2% twice daily,13 and electrodesiccation and curettage.14 In adults, if full resolution is desired, multiple surgeries might be required to remove underlying protuberant cartilage; however, this strategy is not without risk of complication, including formation of adjacent cartilaginous nodules that can become site(s) of CNH recurrence due to a change in pressure points.
Conclusion
Although uncommon, CNH can present on the ears of young patients. A causal link between underlying vasculopathy and CNH has yet to be determined, but the association discovered by Magro et al9 merits obtaining a more detailed rheumatologic history and examination, followed by serologic testing (if indicated). Once the diagnosis of CNH is determined, patient education is paramount to prevent recurrence. Increased awareness of habits that inflict persistent repetitive trauma or pressure to the site—from sleeping patterns to cell phone use—will help to extinguish the behavior and therefore the lesion.