Clinical Notes : ENT

127. Cutaneous Lesions of the External Ear

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Cutaneous lesions of the outer ear

 

Anatomical uniqueness of the ear

 

The outer ear consists of the skin bearing external ear canal and the auricle.

Both are of elastic cartilage covered with skin.

It is attached to the periost and poorly vascularised.

The epidermis on the concave aspect lies on a very thin subcutis which is strongly attached to the auricular cartilage.

In contrast, the convex aspect of the outer ear has a thicker subcutis with a stronger layer of subcutaneous fat which causes a certain laxity and displaceability compared to the concave side.

 

An additional anatomical uniqueness is the high concentration of holocrine ceruminal glands in the skin of the external ear canal. The cerumen may mask existing diseases of the skin in the entrance of the external ear canal.

 

The auricle is susceptible to environmental influences and trauma.

Because of its exposed localization, the ear is particularly liable to the effects of ultraviolet (UV) light and, consequently, to pre-neoplastic and neoplastic skin lesions.

Further, it has a sound-transmitting function and is located at a visible, esthetically obvious site, drawing considerable attention from the patient.

 

Depending on the localization, lesions on the external ear which lead the patient to seek professional help are noticed by the patient himself or by a relative or friend.

When hidden areas of the outer ear are affected, consultation may be delayed until very late in the disease process.

This is especially true for malignant tumors which may often present at an invasive stage, due to the minimal thickness of the skin compared to other parts of the body.

 

Described below are the diseases which are most frequent or call for special attention because of their prognosis.

 

Non-malignant tumors

 

Seborrhoic keratosis

(Syn.: seborrhoic wart, senile wart, and basal cell papilloma)

Diagnosis

  • Seborrhoic keratosis is one of the most common non-malignant tumor of the external ear.

  • It appears as a light brown, mostly flat, sometimes exophytic papular lesion which originates from proliferative epithelial cells 

  • Its spread increases with age and can potentially affect the whole ear, including the external auditory canal 

  • Ultraviolet light exposure, human papillomavirus infection, hereditary factors, action of oestrogen and other sex hormones are among those factors which have been suggested in the aetiology of this disease

  • Secondary malignant changes may occur but are extremely rare 

  • Since it may be confused with malignant melanoma or squamous cell carcinoma, obtaining a specimen for histology is essential.

 

Management

  • Varies from pure trichloroacetic acid, cryotherapy, electrodessication, to simple curettage or excisional surgery. 

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Seborrheic keratosis

Atheroma

(Syn.: sebaceous cyst, atheroma, steatoma, keratinous cyst)

 

Diagnosis

  • Benign tumor which is mostly located at the back of the earlobe.

  • 5 – 25 mm firm, displaceable nodule and may show signs of secondary infection.

  • Sometimes, a pinpoint depression at the surface of the cyst corresponds to the infundibulum of a pre-existing hair follicle.

  • The high density of sebaceous glands over the earlobe predisposes the ear for this lesion.

 

Management

  • Sindle-shaped excision to prevent recurrence.

  • Other techniques of removal include punch biopsy aspiration followed by curettage and avulsion of the cyst wall.

  • Cysts removed from the back of the ears have the highest recurrence rates (13% and 13.8%)

 
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Sebacious cyst

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Sebacious cyst, infected

Granuloma fissuratum

(Syn.: acanthoma fissuratum)

 

Diagnosis

  • reactive process of the skin usually caused by chronic trauma from ill-fitting eyeglass frames.

  • The constant pressure of an ill-fitting frame leads nearly always to an unilateral, skin colored to light red, tender mass of granulation tissue behind the auricle with an exophytic, elliptic growth pattern and a central notch 

  • Its macroscopic appearance has been compared to that of a coffee-bean.

  • It is a benign differential diagnosis of basal cell carcinoma or squamos-cell carcinoma

 

Management

  • correction of the ill-fitting eyeglass frame 

 
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Granuloma fissuratum

Pre-neoplasia

Actinic keratoses

(Syn.: solar keratoses or senile keratoses)

 

Diagnosis

  • UV light-induced lesion which is often located on the ear, especially on the helical rim

  • its frequency increases with age and can progress to invasive squamous cell carcinoma in 20%,

  • prevalence is higher in individuals with fair complexion

  • mostly, a well-defined patch with a rough texture, 3–8 mm in diameter, and typical erythematous base is visible, accompanied by occasional hyperkeratosis.

  • the lesion may grow to large hyperkeratotic plaques with several centimeters in diameter

  • signs of inflammation may occur

Management

  • in the case of a persistent, recurrent, or isolated lesion, a biopsy is recommended to confirm the diagnosis

  • effective treatment options are curettage, photodynamic therapy, laser therapy, topical 5-fluorouracil (5-FU), diclofenac and hyaluronic acid application, imiquimod application

 
 
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Actinic keratosis

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Actinic keratosis

Cutaneous Horn

(Syn.: cornu cutaneum)

Diagnosis

  • Cutaneous Horn is not a pathological diagnosis.

  • A variety of primary underlying processes, benign, premalignant or malignant, can cause this lesion.

  • It presents a mostly asymptomatic, variably sized, keratotic mass arising from the superficial layers of the skin or deeply from the cutis.

  • It generally occurs on sites which are subjected to actinic radiation, with the upper part of the face and the ears being the most common area.

Management

  • Excision and histology to determine if pre-malignant or malignant at its base. ​

 
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Cutaneous horn

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Cutaneous horn

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Cutaneous horn

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Cutaneous horn

 

Lentigo maligna

(Syn.: Hutchinson's freckle)

Diagnosis

  • slow-growing, non-invasive melanoma in situ 

  • estimated lifetime risk of LM progressing to LM melanoma is 5%

  • lesion begins as an unevenly pigmented and irregularly bordered, brown to black macule which slowly extends 

  • lesions size can sometimes obtain several centimetres

Management

  • Non-surgical therapy such as cryosurgery, radiotherapy, electrodessication and curettage, laser surgery, and topical medications with a recurrence rate ranging from 20 to 100% at 5 years have been described in the literature.

  • Recurrence following standard therapies is common because histologic evaluation can be difficult due to the widespread atypical melanocytes that are present in the background of long-standing sun damage].

  • Whenever excision by means of micrographic-controlled or MOHS surgery is possible it should be the preferred method of treatment as it shows the lowest recurrence rate (4–5%) and the best form of margin control among all described forms of therapy.

  • As this lesion occurs more frequently in an elderly patient population, alternative forms of treatment, such as radiotherapy, have to be considered when patients present with very large lesions that are not subject to reconstructive surgery. ​

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Lentigo maligna

Malignant

 
 

Basal cell carcinoma

(Syn,: basalioma, basal cell epitelioma)

Diagnosis

  • 90% of all malignant cutaneous lesions in the head and neck region and is therefore the most common type of skin cancer on the ear.

  • 20% of neoplasms that involve the ear and the temporal bone.

  • The vast majority of BCC occurs on the auricular helix and periauricular area which are especially susceptible as they are exposed to the most UV light.

  • Nevertheless 15% arise in the external auditory canal.

 

  • Five different clinical forms : nodular-ulcerative, pigmented, cystic, superficial multicentric and morphealike.

  • The most common type is the nodular-ulcerative.

  • The lesion is a flesh-colored scaling papule, mostly erythematous to pink, sometimes pigmented, with a surrounding capillary network.

  • It has a pearly border and can show a central ulcer.

  • This most frequent form may infiltrate the cartilage.

  • Although metastases of BCC are extremely rare, the invasive character of the tumor can cause extensive local tissue destruction.

 

  • The second most common type is the morphealike or sclerosing subtype.

  • It is more troublesome as it has indistinct margins and infiltrates along deep tissue planes.

  • It spreads centrifugally with a finger-like growth pattern which complicates therapy.

  • The lesion can potentially extend to the temporal bone or parotid gland and remain undetected.

Management

  • The most successful therapy for basal cell carcinoma is micrographic-controlled surgery (two stage operation).

  • Five-year recurrence rates by micrographic-controlled surgery are reported to be between 1 and 5.6%.

  • Nevertheless, BCC found in the middle of the face (so-called H-zone), followed by those on the auricular and preauricular area have the highest rate of recurrence following treatment by excisional surgery, radiation, cryosurgery, curettage or electrodessication – all alternative forms of treatment.

  • Several theories attempt to explain the high rate of relapse.

  • The ear has a complex anatomy which can confuse the assessment of tumor boundaries.

  • Further an unusual horizontal growth phase makes this tumor prone to incomplete excision.

  • The skin on the concave aspect of the outer ear is very thin and close to the perichondrium. This encourages subclinical spread as skin cancers grow both radially and vertically. Additionally numerous embryonic fusion planes in the auricular skin have been suggested that may contribute to the spread of the tumor].

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Basal cell carcinoma, nodular ulcerative

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Basal cell carcinoma, sclerosing

Bowens disease

(Syn.: Morbus Bowen, carcinoma in situ, squamous intraepidermoid neoplasia)

Diagnosis

  • intraepidermal carcinoma in situ, presenting the preinvasive form of squamous cell carcinoma.

  • strongly associated with sun exposure and lesions are in up to 83% infected with human papillomavirus (HPV) type 16. 

  • erythematous, scaly patches or plaques with irregular borders which can occur anywhere on the skin.

  • can become hyperkeratotic, crusted, fissured, or ulcerated

  • on the ear, most frequently found on the helical rim or the external side of the auricle.

Management

  • Progression to invasive SCC is noted in approximately 10% of Bowen's lesions.

  • excision when possible by means of micrographic guided surgery.

  • Histologically the atypical and disordered keratinocytes in bowens disease extend down the follicular epithelium. Superficial, topical treatment is therefore associated with an increased probability of recurrence.

  • Topical imiquimod, 5-FU, cryotherapy, photodynamic therapy, x-ray and grenz-ray radiation, cauterization or diathermy coagulation therapy are described to be effective but lack mircrographic control.

 
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Bowen's disease

Squamous cell carcinoma

Diagnosis

  • can arise anywhere on the outer ear and potentially involves the middle ear and the lateral skull base.

  • mostly originates on the helix and anthelix margin where the skin receives the greatest actinic exposure.

  • Patients are in their 5th to 6th decade of life whereas lesions originating primarily from the external auditory canal generally present 10–15 years earlier.

  • Sun exposure, fair complexion, cold injury, radiation exposure and chronic infection as well as an association with HPV induced viral carcinogenesis are among the predisposing factors.

  • scaly, indurated, irregular maculopapular lesion which shows an exo- or endophytic growth pattern with a hyperkeratotic or ulcerating surface, sometimes accompanied by seroanguinous exudates.

  • When originating from the external auditory canal hemorrhagic otorrhea, falsely treated as otitis externa, is common. Suspicion should arise and biopsy is mandatory whenever otitis externa fails to respond to adequate conservative therapy.

Management

  • A complete excision by means of micrographic surgery with tumor free margins is necessary for a successful outcome and should be attempted whenever possible.

  • Although this tumor tends to grow in a vertical fashion it is less likely to respect the barriers of cartilage and bone than BCC. Consequently intratemporal spread with involvement of the external auditory canal is possible and can lead to conductive hearing loss.

  • With further deep extension facial nerve palsy due to destruction of the facial nerve along its vertical or tympanic segment may evolve, and finally a further advancement into the internal auditory canal and cerebellopontine angle may cause dizziness and/or sensorineural hearing loss.

  • It is important to investigate for possible regional lymph node metastases which portends poor prognosis. Locoregional metastases follow the lymphatic drainage patterns which include the parotid and upper cervical nodes. 

 
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Squamous cell carcinoma

Melanoma

(Syn.: malignant melanoma)

Diagnosis

  • approximately twenty percent of all primary melanomas are located at the head and neck, of which 7–14% are located at the ear's helix and antihelix.

  • peripheral parts of the ear are more frequently affected.

  • Interestingly the left ear is more often affected than the right ear. The most accepted theory for this phenomenon is the asymetric UV-dosage in anglo-saxon countries with left-hand driven cars.

  • predisposition for males of 61.5–90.5%, and  fair-skinned individuals, can be explained with different hair styles which correlate with UV exposition.

  • with the exception of young children this disease affects all age groups. The average age is 50 years.

  • asymmetric flat hyperpigmentation or a raised nodular lesion which has changed in color and size.

  • amelanotic (non-pigmented) variants exist as well.

  • the three most described subtypes are the superficial spreading melanoma, the nodular melanoma and the lentigno maligna melanoma. Each type has its characteristic growth pattern with a horizontal and a vertical growth phase. The superficial spreading melanoma is the most common. The most aggressive melanoma type is the nodular variant.

Management

  • surgical, and in some instances adjuvant therapy.

  • the World Health Association requires a safety margin of 5 mm for melanoma in situ and 20 mm for melanoma which are >2.1 mm in vertical thickness.

  • In recent years the more aggressive surgical approach has changed towards narrower excision margins as it has been shown to have only an effect on the incidence of local recurrence and only little impact on disease specific survival.

 
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Malignant melanoma

superficial spreading variant

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Malignant melanoma

lentigo maligna variant

Non-malignant tumors

 

Keloid

Diagnosis

  • Keloid is first described in the Smith Papyrus from ancient Egypt.

  • name composed of the Greek words chele (ҳηλη), meaning crab's claw, and the suffix -oid, meaning like. Keloids are dermal fibrotic lesions which are considered an aberration of the wound healing process.

  • included in the spectrum of fibroproliferative disorders and commonly affect the ears.

  • dense dermal scar tissue projects above the surrounding skin which is sometimes tender or pruritic.

  • Keloids on the ear can sometimes be pedunculated.

  • common after small skin excisions, ear piercing, drainage of auricular hematomas, repair of other auricular traumas, viral infection (smallpox, and herpes varicella-zoster) or as secondary keloid formation after prior keloid excision.

Management

  • Several procedures have been described for effective treatment of post-surgical keloid scars. They include silicon occlusive dressings, mechanical compression, radiation, cryosurgery, topical Imiquimod application, bleomycin tattooing, intralesional injections of steroids, 5-floururacil, as well as interferon-alpha, -beta or -gamma in combination with excisional surgery.

  • Although optimal conditions for the prevention of keloid formation are still unknown the combination of excisional surgery and the placement of a silicone gel sheet over the wound surface with the application of light pressure are known to be advantageous

 
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Keloid

 

Inflammatory lesions

 

Winkler Disease

(Syn.: Chondrodermatitis Nodularis Chronica Helicis)

Diagnosis

  • chronic perichondritis which is thought to be related to limited vascularity at the lateral and anterior aspect of the auricle.

  • the skin is tightly stretched over the underlying cartilage with minimal subcutaneous tissue which results in limited vascularity and ischaemia which is thought to promote the development of this lesion.

  • mostly located on the helix this disease is characterized by a hard nodule which involves the skin and the cartilage of the ear.  presents with severe pain in the affected ear especially when slept on it at night.

Management

  • although conservative treatment (radiation, topical antibiotics, intralesional steroids) has been described surgical excision should be preferred as lesions show a tendency to recur. A minimal skin excision should be combined with a more extensive cartilage resection to avoid recurrence.

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Winkler disease

Lymphocytoma

(Syn.: Lymphadenosis cutis benigna)

Diagnosis

  • can be an early manifestation of an infection with Borellia burgdorferi causing Lyme disease.

  • initially it causes a characteristic rash, erythema chronicum migrans, which is located at the tic bite area. During the second stage an intensely red-violet swelling of the earlobe is characteristic.

  • an infection with Borellia burgdorferi is the case in one third of all earlobe lymphocytomas wherefore is has to be ruled out serologically when suspected.

  • the majority (two third of all cases) are idiopathic. Antibiotic therapy consists of doxycyclin p.o. for 2–3 weeks. When the lesions do not improve under antibiotic treatment pseudolymphoma is one possible differential diagnosis. Small lesions can be excised.

Management

  • antibiotic therapy consists of doxycyclin p.o. for 2–3 weeks.

  • when the lesions do not improve under antibiotic treatment pseudolymphoma is one possible differential diagnosis. 

 
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Lymphocytoma

Infectious lesions

 

Auricular chondritis and perichondritis

Diagnosis

  • infection of the auricle.

  • the ear appears erythematous, tender and a fluctuant swelling is mostly present.

  • typically an inciting event (piercing, surgery, trauma, wrestling, acupuncture) is followed by an infection resulting from the collection of blood or serum in the subperichondrial space.

  • the most common organisms which have been tested as causative are S. aureus, P. aeruginosa and Proteus species

Management

  • the subperichondral space must be surgically evacuated and antibiotic therapy consisting of an antipseudomonal aminopenicillin or a flourquinolone for a period of 2–4 weeks, applied [76].

  • as a result of recurring chondritis persisting deformities of the ears' cartilage can remain

 
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Auricular chondritis and perichondritis

Lupus vulgaris

Diagnosis

a persistent form of cutaneous tuberculosis which potentially involves the ear.

Lesions are sharply defined and brown, with a gelatinous consistency on erythematous base

Management

  • combination of antibiotics (isoniazid, rifampicin, pyrazinamide and ethambutol) given over a period of several months, and surgical excision of necrotic tissue.

  • as tuberculosis is enjoying a renaissance in western countries the incidence of cutaneous tuberculosis will increase in the future.

 
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Lupus vulgaris

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Lupus vulgaris

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Lupus vulgaris

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Lupus vulgaris

 
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Cutaneous lesions of the external ear

M Sand, D Sand, D Brors, P Altmeyer, B Mann, F G Bechara

Head & Face Medicine 2008 4:2

08 February 2008

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Ireland notes.png

topical 5-fluorouracil (5-FU) is available in Ireland as Efudix

diclofenac and hyaluronic acid application is available in Ireland as Solaraze

imiquimod application is available in Ireland as Aldara

 

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