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Chondrodermatitis nodularis helicis is considered to be an idiopathic degenerative process involving the upper dermis of the auricular rim.

The lesion is characterized by the presence of small, intensely painful nodules on the free border of the helix of the ear with unknown origin.

Patients are generally middle-aged or elderly males. There are no associated systemic disorders with this condition.

Associations of Chondrodermatitis nodularis helicis  with underlying trauma and sun exposure have been postulated as potential inciting factors.

The lesions are discrete, grey to red in color, oval shaped with raised rolled edges, and a central ulcer or depression which often contains a crust or scale.

The characteristic histopathologic features are epithelial hyperplasia, collagen degeneration, focal fibrinoid necrosis, and inflammatory components. Image Link (humpath.com)

The differential diagnoses for chondrodermatitis nodularis chronica helicis  are relapsing polychondritis and idiopathic cystic chondromalacia.

(Pseudocyst of the auricle: a review of 21 cases. Otolaryngol Head Neck Surg. 1986 Mar;94(3):360-1.Pseudocyst of the auricle (benign idiopathic cystic chondromalacia) is rare. Only 20 cases have been previously reported in the literature. Our study retrospectively reviews 21 additional cases. We conclude that idiopathic cystic chondromalacia can occur in both sexes, in all races, and at any age. The differential diagnoses for idiopathic cystic chondromalacia are relapsing polychondritis and chondrodermatitis nodularis chronica helicis. Recurrence of idiopathic cystic chondromalacia is uncommon following adequate local treatment.)

                  

Neural hyperplasia in chondrodermatitis nodularis chronica helicis. 2006 Nov;55(5):844-8.

BACKGROUND: Chondrodermatitis nodularis chronica helicis (CNCH) is a common condition characterized by tender nodules of the helix, of uncertain mechanism. The pain is not explained by current physiopathological hypotheses.
OBJECTIVE: We sought to investigate possible nerve hyperplasia in CNCH.
METHODS: Thirty-seven cases of clinically typical CNCH were submitted to conventional microscopy after surgical excision and analyzed with immunohistochemistry using PS100 and neurofilament antibodies. As controls, we investigated 25 tumors of the ear with the same methods.
RESULTS: Large nerves (>0.1 mm in diameter) were seen in 8 of 37 cases; these were close to the cartilage or at the upper part of the ulceration. Increased numbers of small nerve sections (0.01-0.06 mm in diameter) were seen in 35 of 37 cases, with more than 20 sections per low-power field in 18 cases. Nerve hyperplasia was not observed in tumors of the ear, even in ulcerated cases.
LIMITATIONS: Hypothesis based on morphologic observations. 
CONCLUSIONS: Nerve hyperplasia is present in CNCH, but is often masked by intense vascular and inflammatory reactions. This finding may explain the induction of pain by light pressure, whereas ulcerated auricular malignant tumors are generally painless. Certain authors believe that CNCH is an equivalent of prurigo nodularis, which interestingly also shows nerve hyperplasia.

Chondrodermatitis nodularis helicis as a marker of internal syndromes associated with microvascular injury. J Cutan Pathol. 2005;32(5):329-33.

Chondrodermatitis nodularis helicis (CNH) is held to be an idiopathic degenerative process involving the upper dermis of the auricular rim. Chondrodermatitis typically occurs in elderly men where associations with underlying trauma and sun exposure have been postulated as potential inciting triggers. Its association as a marker of systemic disease is not well established.
We describe 24 patients with CNH, in whom there were also significant underlying diseases largely associated with vascular injury including those of immune-based etiology and/or conditions which have been previously linked with granuloma annulare, another necrobiotic process of collagen. These patients with concomitant systemic disease were characteristically younger compared to the classic demographics described for CNH.
In some cases, chondrodermatitis may represent an ischemic necrobiotic disorder of collagen, potentially defining an important sign of underlying systemic disease in younger patients.

Juvenile chondrodermatitis nodularis helicis: a case report and literature review.Pediatr Dermatol. 2003 Nov-Dec;20(6):488-90.

Chondrodermatitis nodularis helicis (CNH) is a disorder that affects adults. Only one case of juvenile CNH has been reported, in an 8-year-old child who suffered from dermatomyositis.
We report another child with juvenile CNH who was not afflicted with dermatomyositis or other systemic disorders. The clinical and histologic evaluations demonstrated CNH on the helix of the right ear in a 16-year-old Caucasian girl who was otherwise healthy. Serologic analysis ruled out an underlying autoimmune disorder.
We conclude that juvenile CNH is extremely rare and may occur in patients without dermatomyositis or other systemic disorders.

Chondrodermatitis nodularis chronica helicis. Arch Fam Med.1999 Sep-Oct; 8(5):445-7.

Chondrodermatitis nodularis chronica helicis is a painful nodule of the external ear. These uncommon lesions are most often encountered on the helix in white men older than 40 years, although they also rarely occur on the antihelix in women. The lesions frequently present with exquisite tenderness that interferes with sleep. While the cause of this dermal inflammatory process is not known, long-term trauma or sun damage may play a role. Recurrences often complicate treatment if all sites of inflammation are not eradicated. Surgical treatment is generally recommended, either by wide excision or by deep shave and treatment of the underlying cartilage.

Chondrodermatitis nodularis chronica helicis et antihelicis. Br J Plast Surg. 1996 Oct;49(7):473-6.

A retrospective study of 50 patients (25 male, 25 female) suffering from chondrodermatitis nodularis chronica is presented. There was equal distribution between male and female, with the nodule being situated on the helix in 36 cases (23 male, 13 female), and on the antihelix in 18 cases (4 male, 14 female).
Four patients had bilateral lesions. All the patients complained of severe pain in the affected ear when they slept on it at night. Of the 54 ears in this study, 23 had undergone previous surgery for the complaint. These recurrences occurred when either skin alone, or a disproportionately large piece of skin relative to cartilage, was excised.
A treatment technique is described involving minimal skin excision combined with extensive cartilage resection. There has been no recurrence following our technique and postoperative deformity has been minimal.

Chondrodermatitis nodularis helicis occurring with systemic sclerosis--an under-reported association? Clin Exp Dermatol. 1994 May;19(3):219-20.

Three cases of chondrodermatitis nodularis helicis (CDNH) in patients with the limited form of systemic sclerosis are described.
Two of the three cases were noted during regular follow-up visits and the third case was found as part of a survey of the 21 other patients with systemic sclerosis routinely seen in the department.
While CDNH is known to have several predisposing causes there are no reports in the literature of this condition occurring in association with systemic sclerosis.

The surgical management of chondrodermatitis nodularis chronica helicis. J Dermatol Surg Oncol. 1991 Nov;17(11):902-4.

Surgical management of chondrodermatitis nodularis chronica helicis by curettage is described. The curet directs the dissection.
The necrotic cartilage is soft and is removed easily. The endpoint is reached when the curet is repelled by firm, elastic cartilage.
Contours remain normal because the skin is tented over the defect supported by the remaining cartilage.

Chondrodermatitis nodularis chronica helicis treated with curettage and electro-cauterization: follow-up of a 15-year material. Acta Derm Venereol. 1983;63(1):85-7.

During the 15-year period from 1965 to 1979 a total of 142 cases of chondrodermatitis nodularis chronica helicis were diagnosed. 32% were women, a higher proportion than in earlier materials.
The treatment was principally curettage followed by electrocauterization. 78 patients were re-examined after an average lag of 7.1 years. The relapse rate was 31%.
This simple surgical technique seems equal to the more elaborate procedures in respect of recurrence rate and more satisfactory from a cosmetic point of view.

Chondrodermatitis nodularis helicis: a transepidermal perforating disorder.J Cutan Pathol. 1980 Apr;7(2):70-6.

Chondrodermatitis nodularis helicis is a chronic disorder occurring exclusively on the ear. It is most common in the sixth decade, and two thirds of the patients are men.
Although it has no single characteristic histologic feature, a diagnosis can be made with certainty with a clinical history and a combination of morphologic changes seen in the cartilage and overlying skin.
The natural history and histology are similar to a group of disorders characterized by transepithelial elimination known as the perforating dermatoses.

Chondrodermatitis helicis: a clinical re-evaluation and pathological review. Laryngoscope. 1976 Sep;86(9):1402-12.

Chondrodermatitis helicis, or painful nodule of the ear, is an uncommon benign aural lesion which is seen and treated by dermatologists and otolaryngologists. Because of the sparcity of reports in the recent literature, our experience with 50 patients over a 10-year period is presented.
The diagnosis is based on history and physical examination and biopsy need be performed only to confirm the diagnosis in atypical cases. Patients are generally middle-aged or elderly males. There are no associated systemic disorders with this condition. The lesions are discrete, grey to red in color, oval shaped with raised rolled edges, and a central ulcer or depression which often contains a crust or scale. The lesion is typically painful and tender and, for this reason, the patient seeks help shortly after the onset of symptoms. The characteristic histopathologic features are epithelial hyperplasia, collagen degeneration, focal fibrinoid necrosis, and inflammatory components.
Clinically, the lesion is misdiagnosed in the majority of instances and is presumably, therefore, treated inappropriately. It should be stressed that this is a benign condition and initial management should be aimed at conservative therapy with local steroid injection or conservative non-deforming surgery.
Wide excision, including removal of the underlying cartilage with appropriate reconstrictive closure should be reserved for the conservative treatment failures.
 

                 

 

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