Multiple, bilateral and painful ear nodules of the anthelices: a variant of chondrodermatitis nodularis?

ARTICLE

A case of a distinctive clinicopathologic condition of the ear cartilage is presented, characterized by multiple, bilateral and painful nodules of the anthelices without epidermal involvement. Histologically, there was a peri-chondrial lymphohistiocytic infiltrate and a small focus of degenerate, basophilic cartilage as well as cystic chondromalacia containing an amorphous mass. This condition is both clinically and histopathologically distinct from other causes of ear nodules, although the lesions seen in our patient exhibit features of chondrodermatitis nodularis helices and therefore could well be a variant of the latter.

Key words: anthelix, chondrodermatitis nodularis, ear cartilage, ear nodules, weathering nodules.

Nodular dermatoses of the ear cartilage commonly include chondrodermatitis nodularis helicis (CNH) [1-3], elastic nodules [4], gouty tophi [5], pseudocyst of the auricle [6] as well as benign and malignant neoplasms of both dermis and epidermis. In the present report, we describe a distinctive condition of the ear cartilage, characterized by multiple, bilateral, painful and recurrent nodules of the anthelices. This condition is both clinically and histopathologically distinct from other forms of ear nodules, although it shares similarities with "typical" CNH.

Case report

In March 1996, a 32-year-old male presented to our dermatology department with multiple, whitish nodules of both anthelices which were painful to the touch. The lesions had gradually appeared and extended over the past 5 years. The patient could not recall any precipitating event, such as local trauma, pressure (e.g., extensive cell phone use) or a cold injury. The patient's occupation and hobbies were not obviously relevant either. On examination, the lesions were firm, well-defined, globular or oval nodules, about 3 to 6 mm in diameter, without any apparent epidermal involvement (Fig. 1). Physical examination was otherwise without pathological findings and routine laboratory data showed normal values.

A biopsy specimen from one nodule confirmed that the epidermis was normal. The dermis and thickened peri-chondrium showed a continuous lymphohistiocytic inflammatory infiltrate. On the posterior aspect of the underlying cartilage, there was a small focus of degenerate, basophilic cartilage and cystic chondromalacia containing an amorphous mass (Fig. 2). There were no urate crystals. Elastic tissue staining (resorcin-fuchsin) was unrevealing and von Kossa stain did not show calcifications.

Subsequently, the patient was treated using a modified procedure according to Converse and Stenstrom: perichondrial cartilage scratching at the superior crus and antihelical fold, mattress sutures applied to the perichondrium and soft tissues at the medial surface. A second histological specimen from the resected tissue exhibited perichondrial fibrosis with lymphohistiocytic infiltrate and some necrotic cells in the dermis (not shown), consistent with the diagnosis chondrodermatitis nodularis helicis (CNH). The cosmetic result was good, however, the nodules recurred after 4 years. The patient has thus far declined further surgical treatment.

Discussion

CNH is a common, intensely painful lesion of the ear [1-3]. Most are unilateral and solitary but bilateral and multiple lesions have been reported [7]. Bilateral occurrence is rare, from 6 to 10% [1, 8]. The etiology is unknown, although cold injury, actinic damage [9], pressure necrosis and local trauma have been proposed [7]. A case of CNH with an autoantibody to denatured type II collagen has recently been described [10]. There is debate as to whether the multiple and only slightly painful nodules on the anthelix of elderly women represent the same condition as CNH [8]. It was argued that CNH was due to defects in the cartilage, whereas chondrodermatitis nodularis anthelicis was caused by outside pressure [8]. A differential study of nodules of the helix and anthelix indicated that helix nodules were unilateral and rarely multiple, but anthelix nodules were more frequently bilateral and multiple [8]. The nodules are situated more frequently on the helix in males and on the anthelix in females, especially when the latter have a sharp bend of their anthelix [8, 11].

Histopathological characteristics are irregular acanthosis with hyperkeratosis and parakeratosis abutting an ulcerated area filled with necrotic dermal debris and covered by a crust [2]. The dermis shows degenerated collagen, surrounded by a richly vascularized granulation tissue and a lymphohistiocytic inflammatory cell infiltrate [3]. In CNH, there is usually a central ulcer or depression that contains a crust or scale [1, 2]. Our patient did not exhibit any epidermal involvement (Fig. 1).

Another variant of CNH has recently been reported in people who frequently use cell phones: the nodule may be on the tragus, since the phone is held in such a way as to press only on the lower part of the ear [12]. Cartilage excision alone produces a long-term cure rate of 84% for helix and 75% for anthelix lesions [13]. The modified Converse-Stenstrom approach employed in this patient consists of scoring of the anthelical cartilage on its anterior surface in a subcutaneous position [14, 15].

Elastic nodules of the ear are asymptomatic pearly papules that usually occur bilaterally on both anthelices [4]. Contrary to our histologic findings, however, the elastic fibres would show hyperplasia, thickening, curling, and branching [4]. Moreover, the lack of any clinical, histological or laboratory evidence of hyperuricaemia rules out gouty tophi. Kavanagh and colleagues described clinicopathologically distinctive ear nodules on the free margin of the helices in elderly Caucasian males which they termed "weathering nodules" [16]. In most of the patients the nodules were also multiple and bilateral, but, unlike in our patient, asymptomatic. Histologically, these nodules comprised a spur of fibrous tissue with a focus of cartilage neoplasia, whereas we observed cartilage degeneration and cystic chondromalacia.

Taken together, these findings lend support to the view that our observation represents a distinctive clinicopathologic condition of the ear cartilage. However, the lesions seen in our patient exhibit clinical and histopathologic features reminiscent of CNH and could therefore well be a variant of the latter.

References

1. Newcomer VD, Steffen CG, Sternberg TH, et al. Chondrodermatitis nodularis chronica helicis. Arch Dermatol Syph 1953; 68: 241-55.

2. Metzger SA, Goodman ML. Chondrodermatitis helicis: a clinical re-evaluation and pathological review. Laryngoscope 1976; 86: 1402-12.

3. Shuman R, Helwig EB. Chondrodermatitis helicis. Am J Clin Pathol 1954; 24: 126-44.

4. Weedon D. Elastotic nodules of the ear. J Cutan Pathol 1981; 8: 429-33.

5. Strak TW, Hirokawa RH. Gout and its manifestations in the head and neck. Otolaryngol Clin North Am 1982; 15: 659-64.

6. Cohen PR, Grossman ME. Pseudocyst of the auricle. Case report and world literature review. Arch Otolaryngol Head Neck Surg 1990; 116: 1202-4.

7. Lawrence CM. The treatment of chondrodermatitis nodularis with cartilage removal alone. Arch Dermatol 1991; 127: 530-5.

8. Barker LP, Young AW, Sachs W. Chondrodermatitis of the ears: a differential study of nodules of the helix and anthelix. Arch Dermatol 1960; 81: 53-63.

9. Goette DK. Chondrodermatitis nodularis chronica helicis: a perforating necrobiotic granuloma. J Am Acad Dermatol 1980; 2: 148-54.

10. Yoshinaga E, Enomoto U, Fujimoto N, et al. A case of chondrodermatitis nodularis chronica helicis with an autoantibody to denatured type II collagen. Acta Derm Venereol 2001; 81: 137-8.

11. Munnoch DA, Herbert KJ, Morris AM. Chondrodermatitis nodularis chronica helicis et antihelicis. Br J Plast Surg 1996; 49: 473-6.

12. Elgart ML. Cell phone chondrodermatitis. Arch Dermatol 2000; 136: 1568.

13. Hudson-Peacock MJ, Cox NH, Lawrence CM. The long-term results of cartilage removal alone for the treatment of chondrodermatitis nodularis. Br J Dermatol 1999; 141: 703-5.

14. Baumgartner PH. A technical hint for the correction of prominent ears, based on the method of Converse. Plast Reconstr Surg 1966; 37: 66-8.

15. Stenstrom SJ, Heftner J. The Stenstrom otoplasty. Clin Plast Surg 1978; 5: 465-70.

16. Kavanagh GM, Bradfield JWB, Collins CMP, et al. Weathering nodules of the ear: a clinicopathological study. Brit J Dermatol 1996; 135: 550-4.

Article accepted on 6/5/02