Chondrodermatitis nodularis chronica helicis in monozygotic twins

Chan, Heidi P.; Neuhaus, Isaac M.; Maibach, Howard I.

doi:10.1111/j.1365-2230.2008.02915.x

Cited by 16 publications

(11 citation statements)

References 3 publications

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“…Cartilage degeneration: Winkler believed that CNCH occurs secondary to cartilage degenerative changes, which act as an inflammatory stimulus to the skin [ 17 ]. Also, degenerative changes in the cartilage and its overlying skin, as a result of pressure necrosis, are thought to be the most common etiological factor [ 12 ].…”

Section: Reviewmentioning

confidence: 99%

Review of the Etiopathogenesis and Management Options of Chondrodermatitis Nodularis Chronica Helicis

Salah¹,

Urso²,

Khachemoune³

2018

Cureus

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Chondrodermatits nodularis chronica helicis (CNCH), first described by Max Winkler in 1915, presents as a sore nodule on the helix or antihelix of the external ear. In this paper, we review the etiopathogenesis and management options of CNCH. This condition has a multifactorial etiology; however, sustained pressure from sleeping on one side is the favored theory. Currently, there are many surgical and non-surgical methods of treating CNCH. Most practitioners recommend conservative measures first in their patients, such as pressure-relieving prostheses, prior to surgical treatment. Surgery is the gold standard of therapy with cartilage and wedge excisions yielding recurrence rates of about 10%. Carbon dioxide laser and photodynamic therapy are newer treatment modalities for CNCH, yet they have recurrence rates similar to conservative therapy. In conclusion, due to the high rates of CNCH recurrence, wedge resection is the suggested treatment for CNCH after conservative measures fail.

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Section: Reviewmentioning

confidence: 99%

Review of the Etiopathogenesis and Management Options of Chondrodermatitis Nodularis Chronica Helicis

Salah¹,

Urso²,

Khachemoune³

2018

Cureus

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“…Three of the seven documented cases of pediatric CNH occurred in children with underlying connective tissue disease, and researchers have proposed this as a predisposing factor for the development of CNH in these cases. Researchers have also proposed that genetic and developmental abnormalities of the ear may contribute to the development of CNH . We expect that the imbalance of genes controlling growth on chromosome 11p15 contributes to the development of CNH, although the details of how these genes affect chondrodermatitis development is still unclear.…”

Section: Discussionmentioning

confidence: 99%

Pediatric chondrodermatitis nodularis helicis (CNH) in a child with Beckwith‐Wiedemann syndrome (BWS)

Fix

Cornejo

Duffy

et al. 2019

Pediatric Dermatology

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Chondrodermatitis nodularis helicis is an idiopathic degenerative process that presents as a painful nodule, papule, or ulcer on the helix or antihelix. It predominantly affects adults and is thought to be associated with trauma to the ear. We describe a case of pediatric chondrodermatitis nodularis helicis occurring in a child with a history of Beckwith-Wiedemann syndrome that was successfully treated with an excisional biopsy and relief from a recurrent source of pressure on the ear. | 389Pediatric Dermatology BRIEF REPORT to be associated with trauma to the ear, most commonly from pressure while sleeping, though reports exist of CNH triggered by hats, helmets, telephones, and headphones. 1 Vascular insufficiency, underlying connective tissue disease, thermal, and actinic damage have also been proposed to contribute to CNH. 2 Chondrodermatitis nodularis helicis is predominantly a disease of adults, with only 7 reported cases in the pediatric literature (Table 1). Three of the seven documented cases of pediatric CNH occurred in children with underlying connective tissue disease, and researchers have proposed this as a predisposing factor for the development of CNH in these cases. Researchers have also proposed that genetic and developmental abnormalities of the ear may contribute to the development of CNH. 3,4 We expect that the imbalance of genes controlling growth on chromosome 11p15 contributes to the development of CNH, although the details of how these genes affect chondrodermatitis development is still unclear. Common BWS findings of ear pitting or ear creases were absent in this patient. However, as this child had larger-than-average ears and left-sided lateralized overgrowth, we hypothesize that persistent pressure from headphones on his enlarged ear predisposed him to developing CNH. An extensive clinical differential diagnosis for CNH includes calcinosis cutis, gout tophi, reactive perforating collagenosis, verruca vulgaris, basal cell carcinoma, squamous cell carcinoma, actinic keratosis, and weathering nodule of the ear. Although CNH in a pediatric patient is rare, it should be considered in any young patient presenting with a tender papule or nodule on the external ear. When CNH is suspected, providers should try to identify any contributing factors, with the understanding that their absence does not preclude the diagnosis.Surgical removal is the first line treatment for suspected CNH, as it allows histopathologic assessment of the lesion in question and has a higher cure rate than common nonsurgical techniques like pressure relief or topical nitroglycerine. 6,7 The efficacy and safety of other nonsurgical techniques like topical or intralesional steroids, collagen injection, cryotherapy, laser therapy, platelet-rich plasma, topical bacitracin, and photodynamic therapy are less well studied. 7

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“…2 CN has shown evidence of association with autoimmune and connective tissue disorders, and may have a familial propensity. 3 Treatment of CN, once the diagnosis is established, is aimed at comfort, cosmesis, and prevention of recurrence. Offl oading the pressure of sleeping on one ear is essential, and numerous devices have been used: sponges with holes cut in them, oval corn cushions, and even medical-device pads that fasten around the head with hook-and-loop closures and surround the ear.…”

Section: Discussionmentioning

confidence: 99%

A persistent, painful ear wound

Overcash¹

2014

Journal of the American Academy of Physician Assistants

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CASEA 74-year-old woman presented to a dermatology offi ce with complaints of a painful area on the helix of the right ear. The lesion intermittently heals and recurs and is made worse by sleeping on her right side. She has been sleeping primarily on her right side for over a decade, due to shoulder problems. She denied any recent trauma, cold exposure, sunburn, or additional lesions. She attempted to cure the lesion with triple antibiotic ointment, topical antifungal creams, and tea tree oil, all without success. Her past medical history is remarkable for multiple nonmelanoma skin cancers and actinic keratoses, and she was worried this was a skin cancer "like my husband had on his ear."Physical examination reveals signifi cant photodamage on the face, ears, arms, and upper chest. On the lower crus of the antihelix, exactly where it is overlapped by the root of the helix (Figure 1), she has a 3 mm, fi rm, tender nodule with a 1 mm central ulcer (Figure 2). THE MOST LIKELY DIAGNOSIS IS • relapsing polychondritis • chondrodermatitis nodularis • squamous cell carcinoma • basal cell carcinoma DISCUSSIONThe patient's age, history of sleeping on one side, location of the lesion, and clinical appearance all support a diagnosis of chondrodermatitis nodularis (CN). CN is also called chondrodermatitis nodularis chronica helicis et antihelicis, as it is chronic and occurs both on the helix and on the antihelix. Some lesions present with a central crusty core, although it is often excoriated by the patient, leaving only a small ulcer.Squamous cell carcinomas are also commonly painful and present on a sun-damaged ear, so this must be ruled out via a full-thickness biopsy if the diagnosis is in question or a lesion persists despite treatment. Basal cell carcinoma also occurs in sun-damaged skin, and may have central ulceration, but usually is not painful. Relapsing polychondritis also causes a painful red ear, but would not be limited to a small pressure-bearing area. To rule out skin cancer, and for therapeutic benefi t, a deep saucerization shave biopsy was performed. Care was taken to avoid leaving any jagged edges or fragmented cartilage, and the base of the lesion was not curetted.Histopathology revealed irregular epidermal hyperplasia with hyperkeratosis and hypergranulosis, degeneration of the connective tissue beneath the zone of hyperplasia, and a mild infl ammatory infi ltrate of mononuclear cells. Histopathology of CN often includes visualization of a large central crater fi lled with acellular necrotic debris, fi brin, and infl ammatory cells; acanthotic hyperkeratotic epidermis around the crater; fi brin and eosinophilic dermal collagen under the crater; and edema of the surrounding viable dermis. 1 Signs of sun damage often are present, such as telangiectasia, and signs of chronic infl ammation of ear cartilage such as fi brosis and even ossifi cation of cartilage.CN is caused by continuous pressure on the fragile rim of cartilage inside the helix, especially in the setting of thin overlying skin. It is most comm...

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Chondrodermatitis nodularis chronica helicis in monozygotic twins

Cited by 16 publications

References 3 publications

Review of the Etiopathogenesis and Management Options of Chondrodermatitis Nodularis Chronica Helicis

Review of the Etiopathogenesis and Management Options of Chondrodermatitis Nodularis Chronica Helicis

Pediatric chondrodermatitis nodularis helicis (CNH) in a child with Beckwith‐Wiedemann syndrome (BWS)

A persistent, painful ear wound

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