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   Amyloid Nodules of Ear    


 

                
These are small papules usually located on the concha of the ear. They may be present on both ears. 

The lesions are usually not pruritic but slightly friable. 

The patients do not have lesions of amyloidosis on other parts of the body and no known insult precedes the appearance of lesions. 

Histochemical and electron microscopic methods help in confirming the amyloid nature of the substance.

Immunohistochemically, the amyloid substance stained positively with cytokeratin 34betaE12 and EKH4 keratin.

Otologic involvement with amyloidosis is extremely rare. The pathophysiology may be related to the local factors in the external auditory canal.

Differential diagnosis: Collagenous Papules of Ear

                  

Primary cutaneous amyloidosis of the auricular concha: case report and review of published work. J Dermatol. 2006 Feb;33(2):128-31.

A 70-year-old Japanese female developed tiny papules on her bilateral ears 2 years previously. A histological study of a biopsy specimen revealed that amorphous materials were present in the widened dermal papillae. Because these materials were positive for both Congo red and Dylon, we diagnosed the lesion as primary cutaneous amyloidosis of the auricular concha. Immunohistochemically, the amyloid substance stained positively with 34betaE12 (cytokeratin 1/5/10/14), suggesting that it had an epidermal origin. Seven reported cases of this unique disorder were also reviewed.

Lichen amyloidosis of the auricular concha: report of two cases and review of the literature. Dermatol Online J. 2006 Sep 8;12(5):1.

Two cases of lichen amyloidosis of the auricular concha are presented here, supplementing the already approximately twenty cases of lichen amyloidosis of the auricular concha reported to date. The first patient is a 60-year-old woman who presented with a 4-year history of an itchy rash that started in the bowl of her left ear, and soon after appeared on the right side as well. The second patient is a 44-year-old woman who presented with a long-standing pruritic rash on her upper back. This had become noticeably darker over the past several years. In one of the two cases lichen amyloidosis of the auricular concha reported here, concomitant macular amyloidosis of the back was present. Combined cases of lichen and macular amyloidosis are termed biphasic amyloidosis, and provide support to the theory that these two variants of amyloidosis exist on the same disease spectrum.

Otologic manifestations of amyloidosis. Otol Neurotol.2002;23(2): 158-9.

OBJECTIVE: To describe otologic manifestations of amyloidosis. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Case report of a patient with bilateral external auditory meatal stenosis secondary to amyloidosis. RESULTS: The patient is a 60-year-old man who presented with several months' history of bilateral ear blockage and drainage. Physical examination revealed markedly thickened skin in each ear in the area of concha and meatus that was associated with fissuring. The meatus were markedly stenosed. The medial aspect of the external auditory canal and the tympanic membrane were normal. He had partial improvement on medical therapy with topical steroid-containing eardrops and ointment. Past medical history was positive for multiple myeloma. Meatal biopsy was performed. Grossly, the tissue was hypovascular and markedly thickened. Histopathologic examination was consistent with amyloidosis. The patient was continued on topical steroid ointments, frequent ear cleaning, and close follow-up. He was subsequently diagnosed with systemic amyloidosis. A review of the literature was done. Head and neck involvement with amyloidosis is reviewed, with emphasis on otologic manifestations. CONCLUSION: Otologic involvement with amyloidosis is extremely rare. The pathophysiology may be related to the local factors in the external auditory canal.

Papules in the auricular concha: lichen amyloidosus in a case of biphasic amyloidosis. Dermatologica.1990;181(2):149-51.

We present a patient with lichen amyloidosus on the ears and macular amyloidosis on the back. These diagnoses were supported by histological, histochemical and immunohistochemical studies. This is to the best of our knowledge the first reported case of a biphasic form of amyloidosis whose lichenoid counterpart consists of papules on the ears. This suggests that primary cutaneous localized amyloidosis may have peculiar clinical manifestations depending on the location of the lesion.

Primary cutaneous amyloidosis of the auricular concha. J Am Acad Dermatol.1988 Jan;18(1 Pt 1):19-25.

Several patterns of primary cutaneous amyloidosis are recognized; in this report of four cases, we define a new pattern in which the lesions are composed of small papules grouped on the concha of the ear. The lesions typically are not pruritic but slightly friable. They may be present on both ears. The patients do not have lesions of amyloidosis on other parts of the body and no known insult precedes the appearance of lesions. This entity appears to have been described previously as collagenous papules of the ear. Histochemical and electron microscopic methods allow us to confirm the amyloid nature of the substance. We believe this condition to be a variant of primary cutaneous amyloidosis, particularly because monoclonal antikeratin antibody EKH4, which has been positive in lichenoid, macular, and skin epithelial tumor-associated amyloidoses, was positive in our cases.


November 2007

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Case Index

Infectious Disease Online; INDEX: A-D ; INDEX: E-L ; INDEX: M-PINDEX: Q-Z ; FUNGAL DISEASE ; VIRAL DISEASE.

Accessory Tragus

First Branchial Cleft Anomalies

Salivary Gland Choristoma

Gout of Ear

Malakoplakia of Ear

Granuloma Inguinale of Ear

Idiopathic Auricular Ossificans

Idiopathic Cystic Chondromalacia of Auricular Cartilage

Inflammatory Aural Polyp

Angiolymphoid Hyperplasia with Eosinophilia of Ear

Kimura's Disease of Ear

Labyrinthitis

Meniere's Disease

Chondrodermatitis Nodularis Chronica Helicis