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A “Turkey Ear” Persisting for 5 Decades

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A woman in her 50s presented with an edematous, massively enlarged right auricle. The patient had immigrated to Israel 9 years before admission. She was adamant that the lesion had been present since early childhood but had gradually increased with a recent appearance of foul-smelling otorrhea. On examination, the auricle was markedly enlarged, edematous, and encircled by an erythematous, indurated plaque partially covered with a fine scale (Figure 1A). Diascopy revealed an “apple jelly” appearance. There was no local or distant lymphadenopathy; general physical examination findings were unremarkable.

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C. Lupus vulgaris

A tissue culture was positive for Mycobacterium tuberculosis. The clinicohistologic findings and isolation of M tuberculosis confirmed the diagnosis of lupus vulgaris (LV). A trial of quadruple antituberculous therapy with isoniazid, rifampicin, pyrazinamide, and ethambutol for 2 months followed by rifampicin and isoniazid for the next 7 months resulted in a rapid clinical response. The patient was then lost to follow-up for several years, until 2020, when current physical examination revealed complete clinical resolution (Figure 2).

Lupus vulgaris is the most common type of cutaneous tuberculosis. It is a paucibacillary disease characterized by a chronic and progressive course that develops in a previously sensitized host with strong delayed hypersensitivity to tuberculin. In Western countries, LV is more common in females and predominantly occurs in the head and neck region, while in tropical countries, it is more common in males and predominantly involves the lower limbs.1 Lupus vulgaris may occur through hematogenous, lymphatic, or contiguous spread from a remote focus of disease elsewhere in the body. It may rarely follow primary inoculation or vaccination with bacille Calmette-Guérin.2 Clinically, LV starts as an erythematous-brownish plaque that slowly expands over the years. A classic clinical sign is the apple jelly appearance on diascopy. Histologically, LV is characterized by a classic granulomatous tubercle with minimal caseation. The cellular inflammatory infiltrate includes epithelioid cells, Langhans giant cells, and mononuclear cells. Because bacilli are few and sparse in LV, acid-fast staining is rarely positive, and the yield of tissue cultures is also low.36 In such cases, polymerase chain reaction for tissue mycobacterial DNA may increase diagnostic accuracy.6 As patients with LV may have an active visceral focus of tuberculosis elsewhere, this should be sought out and excluded. To note, results of chest radiography are often normal, as the initial site of tuberculosis may be extrapulmonary.

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Article Information

Corresponding Author: Nadav Astman, MD, PhD, Department of Dermatology, Sheba Medical Center, Ramat Gan 5262000, Israel (nadavastman@gmail.com).

Published Online: March 3, 2021. doi:10.1001/jamadermatol.2020.5832

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient’s family for granting permission to publish this information. We thank Ohad Hilly, MD (Otolaryngology and Head and Neck Department at Rabin Medical Center); Anna Lyakhovitsky, MD, and Sharon Baum, MD (Department of Dermatology at Sheba Medical Center, Tel-Hashomer); and Dov Polisky, MD (consultant), for assisting in professional guidance and comments on this case. Compensation was not received.

References
1.
Barbagallo  J , Tager  P , Ingleton  R , Hirsch  RJ , Weinberg  JM .  Cutaneous tuberculosis.   Am J Clin Dermatol. 2002;3(5):319-328.PubMedGoogle ScholarCrossref
2.
Pace Spadaro  E , Xuereb Dingli  J , Doffinger  R , Dinakantha  K , Betts  A , Pace  D .  BCG induced lupus vulgaris.   Arch Dis Child. 2018;103(1):71-72.PubMedGoogle ScholarCrossref
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Williams  C , Mitra  A , Walton  S .  ‘Turkey ear’.   Br J Dermatol. 2007;157(4):816-818.PubMedGoogle ScholarCrossref
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Woo  PN , Batta  K , Tan  CY , Colloby  P .  Lupus vulgaris diagnosed after 87 years, presenting as an ulcerated ‘birthmark’.   Br J Dermatol. 2002;146(3):525-526.PubMedGoogle ScholarCrossref
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Abraham  Z , Feuerman  EJ .  Lupus vulgaris diagnosed after 71 years.  Article in Hebrew.  Harefuah. 1991;120(12):720-722.PubMedGoogle Scholar
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Lu  Y , Wang  H , Zheng  H , Li  X .  Bilateral “turkey ear” as a cutaneous manifestation of lupus vulgaris.   Indian J Dermatol Venereol Leprol. 2018;84(6):687-689.PubMedGoogle ScholarCrossref
7.
Gawkrodger  DG . Sarcoidosis. In: Burns  T , Breathnach  SM , Cox  NH , Griffiths  CEM , eds.  Rook’s Textbook of Dermatology. 7th ed. Blackwell Publishing; 2004:58.1-58.22.
8.
Kempf  W , Ostheeren-Michaelis  S , Paulli  M ,  et al; Cutaneous Lymphoma Histopathology Task Force Group of the European Organization for Research and Treatment of Cancer.  Granulomatous mycosis fungoides and granulomatous slack skin.   Arch Dermatol. 2008;144(12):1609-1617.PubMedGoogle ScholarCrossref
9.
Kogut  M , Hadaschik  E , Grabbe  S , Andrulis  M , Enk  A , Hartschuh  W .  Granulomatous mycosis fungoides, a rare subtype of cutaneous T-cell lymphoma.   JAAD Case Rep. 2015;1(5):298-302.PubMedGoogle ScholarCrossref
10.
Landau  M , Srebrnik  A , Brenner  S .  Leishmaniasis recidivans mimicking lupus vulgaris.   Int J Dermatol. 1996;35(8):572-573.PubMedGoogle ScholarCrossref
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