Ear signs in dermatology: At a glance

The ear with its unique anatomical structure and exposed location, often serves as a window into various underlying dermatological conditions. This review attempts to describe the classic “ear signs” relevant to dermatology and how recognising these can aid clinicians in making a prompt diagnosis.

• a.

  Infections

  • 1)

    Milian’s ear sign – It refers to the presence of tender, erythematous, oedematous pinna along with a well-demarcated facial erythematous plaque. It is a useful sign to differentiate erysipelas from cellulitis.¹ Erysipelas involves the upper dermis and lymphatics, while cellulitis affects the deeper dermis and fat. The pinna’s thin dermis, lack of fat, and rich lymphatics explains its involvement in erysipelas but sparing in cellulitis [Figure 1].

  • 2)

    The ear sign in Tinea capitis/faciei – In children with tinea capitis, erythematous papules/plaques with scaling are seen over the helix, antihelix, and retroauricular region (sparing the retroauricular fold). This is unlike seborrheic dermatitis, in which erythema, scaling, or fissuring is present in the retroauricular fold.² Ear sign has also been described in tinea faciei [Figure 2] and in solitary tinea auricularis.

  • 3)

    Turkey ear – In lupus vulgaris, the ear can develop massively enlarged, indurated earlobes with a bluish-red or violaceous hue, often studded with papulo-pustules and nodules with overlying ulceration [Figure 3]. Bilateral turkey ears have also been reported.³ The sign was originally demonstrated in lupus pernio and signifies granulomatous infiltration of the earlobe in sarcoidosis [Figure 4].⁴

  • 4)

    Buddha ear – It is the presence of thickened, nodular earlobes in lepromatous leprosy. The lobes become bulky, smooth, and sometimes shiny, often with a reddish discolouration. The bacteria multiply in cooler areas of the body and the dermal infiltration and accumulation of histiocytes causes the enlarged lobes.⁵ Buddha ear shows nodular thickening while turkey ear shows violaceous induration [Figure 5]. If the ear develops ulceration and notching at the margins, especially on the helix and antihelix, it is referred to as a “rat-bitten” ear.

  • 5)

    Red ear sign –Localised erythema and swelling of earlobes is seen in acute chikungunya infection. It is similar to Milian’s ear sign of erysipelas and can help in the early diagnosis of chikungunya, particularly during outbreaks.⁶

• b.

  Autoimmune/Inflammatory dermatoses

  • 6)

    Pigmented contact dermatitis – In Riehl’s melanosis, the ear sign refers to preferential pigmentation of the outer ear, helix, and earlobe. It may appear asymmetrically, as cosmetic or topical allergens applied with one hand often spare the ipsilateral earlobe while affecting the contralateral side. This pattern results from the hand’s anatomy and the sweeping movement during application.⁷

  • 7)

    Shuster sign – It refers to discoid lupus erythematosus (DLE) lesions in the conchal bowl—a somewhat concealed area of the ear—that may easily be missed on cursory examination. These plaques often show classic DLE features like follicular plugging, adherent scaling, and progression to atrophy with scarring and dyspigmentation [Figure 6].⁸

  • 8)

    Dermatomyositis – Helix/antihelix violaceous macules and erythematous auricular papules have been reported in dermatomyositis with anti-MDA5 (melanoma differentiation -associated gene 5) antibodies and may be predictors of poor prognosis.⁹

  • 9)

    Lambing ears – Sheep farmers during lambing season may develop blistering and crusting of the ear pinna called lambing ears. It is hypothesised to be a reaction to contact with bodily fluids or other products of conception and is more common in indoor lambing than outdoor lambing, where sunlight exposure may also be a risk factor. The histology and distribution of lambing ears are comparable with the juvenile spring eruption variant of polymorphic light eruption, but its demographics are unique, which are also presented as erythematous scaly itchy papules and vesicles on the superior helix of the ears.¹⁰ However, JSE typically affects boys and young men during springtime.¹⁰

  • 10)

    Blue ear sign – It is seen in relapsing polychondritis (RP), an episodic autoimmune condition of cartilaginous structures, particularly the ears, nose, larynx, and trachea. Recurrent inflammation thins the cartilage, making the underlying vasculature more visible resulting in a characteristic bluish hue. This sign is seen early in the disease course. Lepra reaction is a mimic of RP, but involves both pinna and lobule, as opposed to RP which spares the ear lobule.¹¹ Chronic inflammation and scarring can lead to permanent deformity in which the ear cartilage softens and collapses forward, described as a forward listing ear, floppy pinna, or cauliflower ear [Figure 7].¹²

  • 11)

    Chondrodermatitis nodularis helicis/Winkler’s disease – It is a benign inflammatory condition that presents as a small, tender nodule with central crusting over the helix [Figure 8]. It occurs predominantly in elderly males and is hypothesised to result from chronic pressure or repeated trauma to the ear.¹³ A close mimic is elastotic nodules of the ears, secondary to chronic actinic damage and located over the antihelix in elderly males, where it may simulate basal cell carcinoma.

• c.

  Genetic syndromes

  • 12)

    Crumpled ear – It is characteristic of congenital contractural arachnodactyly (Beals syndrome) caused by fibrillin 2 gene mutation. The upper helix is folded, the crura are prominent and the concha is shallow. Other features of the syndrome include joint contractures, arachnodactyly, marfanoid habitus, and kyphoscoliosis.¹⁴

  • 13)

    Mickey mouse ears – They are a feature of Cockayne syndrome, caused due to DNA repair defect. Ears are prominent against a small face with a thin nose and marked loss of subcutaneous fat. Other features include photosensitivity, premature skin ageing, disproportionately large hands and feet, and mental retardation.¹⁵

• d.

  Miscellaneous

  • 14)

    Frank’s sign – It is a diagonal earlobe crease running at a 45° angle from the tragus outward towards the lower edge of the earlobe. It is independently associated with a higher risk of cardiovascular diseases, especially when the crease is complete, bilateral, deep, and has accessory creases.¹⁶ Possible pathogenesis is the loss of dermal elastic fibres due to microvascular disease in the ear (an end-artery organ). Another sign, called PECH (paired ear creases of the helix), is also reported to be associated with coronary artery disease.¹⁷

  • 15)

    Cauliflower ear – It is an auricular deformity from repeated blunt trauma, causing cartilage damage, typically from contact sports like boxing and wrestling. The ear appears thickened, bumpy, and irregular, and the normal contours of the pinna are lost. There is a sparing of the earlobe owing to its lack of cartilage. It may also occur in RP, leprosy, and pheohyphomycosis.¹⁸

  • 16)

    Swimmer’s ear (otitis externa) – It is an inflammation of the pinna and meatal skin caused by pseudomonas and Staphylococcus aureus. Common triggers include water exposure, trauma, and dermatologic conditions like eczema. It presents with otalgia, discharge, and hearing loss.

  • 17)

    Petrified ear – It is the development of stony-hard auricular cartilage secondary to ectopic calcification or, less commonly, ossification. Common causes include frostbite, mechanical trauma, and Addison’s disease; however, diabetes, hypothyroidism, and acromegaly have also been reported.¹⁹

  • 18)

    Acanthoma fissuratum – It is a reactive condition caused by chronic low-grade trauma from eyeglasses. It presents as a unilateral flesh-coloured plaque with a central groove (coffee bean appearance) in the post-auricular groove [Figure 9]. Underlying factors include ill-fitting spectacle frames, the weight of glasses, and pre-existing skin disease. It may sometimes be misdiagnosed as basal cell carcinoma.²⁰

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Figure 1: Erythematous oedematous pinna of erysipelas – Milian’s ear sign.

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Figure 2: Scaly erythematous plaque over the ear with concomitant tinea faciei.

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Figure 3: Enlarged indurated ear with overlying ulceration – lupus vulgaris.

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Figure 4: Turkey ear of lupus pernio. (Image courtesy: Dr. Vinay K, PGIMER, Chandigarh)

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Figure 5: Thickened nodular earlobes of leprosy, called Buddha ear.

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Figure 6: Shuster sign – DLE plaque with scarring and dyspigmentation over concha of ear.

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Figure 7: Cauliflower ear in relapsing polychondritis.

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Figure 8: Chondrodermatitis nodularis helicis – single nodule with central crust over the helix in an elderly male.

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Figure 9: Acanthoma fissuratum – plaque with central groove in the post-auricular groove (Image courtesy: Dr Manju Daroach, AIIMS, Bilaspur)

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Figure 10: Multiple pink-to-red papulonodular lesions of ALHE in the periauricular region. ALHE: Angiolymphoid hyperplasia with eosinophilia

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Other conditions where the ear gives diagnostic clues are summarised in Table 1.