A 60-year-old woman presented with a nodule on her right antihelix. The nodule had appeared a year ago and was tender to the extent that it would awake her from sleep when she turned on it. Her past health was unremarkable. There was no history of trauma. She had been sleeping on her right side until the appearance of the painful nodule, when she began to favor sleeping on her left side.
Physical examination revealed an erythematous, dome-shaped, firm nodule with a central crust on the right antihelix. The nodule measured 2 to 3 mm in diameter, and it was tender to palpation. The rest of the physical examination findings were unremarkable.
Answer: Winkler diseaseSee the full case at Consultant360
Chondrodermatitis nodularis helicis (CNH), also known as chondrodermatitis nodularis chronicus helicis, Winkler nodules, or Winkler disease, is a benign, inflammatory, and degenerative condition that affects the skin and cartilage of the pinna, manifesting as a tender papule or nodule.1 The disorder was first described in 1915 by Max Winkler, who reported on 8 men with painful, firm, lentil- to cherry-sized nodules with central crusting on the helix.2
EPIDEMIOLOGY and ETIOPATHOGENESIS: Chondrodermatitis nodularis helicis is a common dermatologic disorder. The exact incidence is not known, because many cases are not recognized or not reported. In most cases, the age of onset is greater than 40 years, with a peak between 58 and 72 years.1,3 For lesions on the helix, the male to female ratio is approximately 10 to 1, while lesions on the antihelix are more common in women.4-6
Although the exact etiology is not known, chondrodermatitis nodularis helicis might result from local trauma, prolonged excessive pressure such as that created by constant sleeping on one side, and actinic damage, leading to arteriolar narrowing and ischemia of the auricular perichondrium, cartilage, and dermis.6,7 The ischemia may lead to necrosis of the dermis and the underlying cartilage.8 The necrobiotic dermal collagen and sometimes the underlying cartilaginous matrix are extruded through a crater-like defect in the epidermis.6 The auricle, especially the helix, is predisposed to this condition given that the local blood supply is poor and there is little supporting subcutaneous tissue for cushioning.8 Genetics might contribute to the condition given that it has been observed in monozygotic twins.4
HISTOPATHOLOGY and PRESENTATION: Histologically, the epidermis surrounding the crater is hyperkeratotic and acanthotic.6 The dermal collagen underlying the crater is homogeneous and eosinophilic and surrounded by vascular granulation tissue.6,9 There is inflammation, degeneration, and fibrosis of the underlying perichondrium.9 Degenerative changes may be seen in the cartilage.9
Typically, chondrodermatitis nodularis helicis presents as a tender, solitary, firm, well-demarcated, dome-shaped, erythematous or skin-colored papule or nodule on the pinna.10 The helix commonly is involved, followed by the antihelix, although any part of the pinna such as the antitragus, scapha, and concha also may be involved.3 The superior pole of the helix at the transition from the vertical to horizontal course of the ear margin is most commonly affected.3 The papule or nodule is attached to the underlying cartilage and is usually a few millimeters or less in diameter. The lesion may have rolled edges and a central ulceration or crust.11 The right pinna is affected more frequently than the left pinna.3,11 Bilateral occurrence or multiple lesions also have been described.3,4
DIFFERENTIAL DIAGNOSIS: The differential diagnosis includes actinic keratosis, basal cell carcinoma, keratoacanthoma, squamous cell carcinoma, eccrine spiradenoma, glomus tumor, leiomyoma, neuroma, prurigo nodularis, and gouty tophi.3
COMPLICATIONS and TREATMENT: The painful lesion often interferes with the patient’s sleep and affects the patient’s quality of life.1,11
The use of a chondrodermatitis pillow or similar pressure-relieving prosthesis, avoidance of the use of earphones and headphones, and sleeping on the unaffected side should be advised.10,11 Spontaneous resolution upon removal of pressure from the affected ear has been reported.12
For persistent lesions, surgical excision remains the gold standard of treatment, which also makes histopathologic examination possible.3,4 Other treatment options include topical nitroglycerin, topical corticosteroid, intralesional corticosteroid, perilesional collagen injection, laser ablation, curettage, electrocauterization, cryotherapy, and photodynamic therapy.1,9 In spite of treatment, the recurrence rate is high.1 Referral to a dermatologist to confirm the diagnosis and for treatment is recommended.
1. Garrido Colmenero C, Martínez García E, Blasco Morente G, Tercedor Sánchez J. Nitroglycerin patch for the treatment of chondrodermatitis nodularis helicis: a new therapeutic option. Dermatol Ther. 2014;27(5):278-280.
2. Winkler M. Knötchenförmige Erkrankung am Helix (chondrodermatitis nodularis chronicus helicis). Arch Dermatol Syph. 1915;121(2):278-285.
3. Wagner G, Liefeith J, Sachse MM. Clinical appearance, differential diagnoses and therapeutical options of chondrodermatitis nodularis chronica helicis Winkler. J Dtsch Dermatol Ges. 2011;9(4):287-291.
4. Chan HP, Neuhaus IM, Maibach HI. Chondrodermatitis nodularis chronica helicis in monozygotic twins. Clin Exp Dermatol. 2009;34(3):358-359.
5. Flynn V, Chisholm C, Grimwood R. Topical nitroglycerin: a promising treatment option for chondrodermatitis nodularis helicis. J Am Acad Dermatol. 2011;65(3):531-536.
6. Thompson LD. Chondrodermatitis nodularis helicis. Ear Nose Throat J. 2007;86(12):734-735.
7. 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. 2009;34(2):147-150.
8. Kulendra K, Upile T, Salim F, O’Connor T, Hasnie A, Phillips DE. Long-term recurrence rates following excision and cartilage rim shave of chondrodermatitis nodularis chronica helicis and antihelicis. Clin Otolaryngol. 2014;39(2):121-126.
9. Gilaberte Y, Frias MP, Pérez-Lorenz JB. Chondrodermatitis nodularis helicis successfully treated with photodynamic therapy. Arch Dermatol. 2010;146(10):1080-1082.
10. Singh M, Wilson A, Parkinson S. Two non-surgical treatments for chondrodermatitis nodularis helicis. Br J Oral Maxillofac Surg. 2009;47(4):327-328.
11. Kuen-Spiegl M, Ratzinger G, Sepp N, Fritsch P. Chondrodermatitis nodularis chronica helicis – a conservative therapeutic approach by decompression. J Dtsch Dermatol Ges. 2011;9(4):292-296.
12. Travelute CR. Self-adhering foam: a simple method for pressure relief during sleep in patients with chondrodermatitis nodularis helicis. Dermatol Surg. 2013;39(2):317-319.