|Year : 2019 | Volume
| Issue : 1 | Page : 89-91
Myxomatous form of lupus vulgaris in ear: A rare presentation
C Chandrakala, Gurusami Karuvelan Tharini
Department of Dermatology, Government Villupuram Medical College Hospital, Villupuram, Tamil Nadu, India
|Date of Web Publication||14-Feb-2019|
Department of Dermatology, Government Villupuram Medical College Hospital, Mundiyambakkam, Villupuram - 605 601, Tamil Nadu
Source of Support: None, Conflict of Interest: None
Lupus vulgaris is a postprimary, chronic form of cutaneous tuberculosis commonly seen over the extremities and gluteal region in Indian population. It is rarely seen over the ears. Lupus vulgaris occurring in the ear was described as “Turkey ear” in some articles. Lupus vulgaris occurring at the cartilaginous sites such as ears and nose lead to disfiguration. Early diagnosis of lupus vulgaris at these sites and initiation of antituberculous drugs will prevent the complications such as mutilation.
Keywords: Lupus vulgaris, myxomatous form, Turkey ear
|How to cite this article:|
Chandrakala C, Tharini GK. Myxomatous form of lupus vulgaris in ear: A rare presentation. Clin Dermatol Rev 2019;3:89-91
| Introduction|| |
Lupus vulgaris is the most common form of cutaneous tuberculosis (TB). It can follow a chronic course with the risk of cutaneous dissemination, scarring, and mutilation and the development of squamous cell carcinoma and basal cell carcinoma as a result of chronicity. Atypical forms can occur which needs to be identified and treated earlier to prevent complications.
| Case Report|| |
A 45-year-old female attended the outpatient department (OPD) with a history of asymptomatic swelling in the left ear lobule for 20 years which was gradually increasing in size and slowly involving the pinna for 3-year duration. She developed asymptomatic smaller lesions over the back and left cheek for 6 months. There was no history of fever, cough, dyspnea, loss of appetite, and weight. She has not suffered from any other form of TB in the past. There was no history of contact with TB patients. On examination, a soft, nontender, bulky, large, erythematous swelling was seen over the left ear lobule which was extending to the pinna [Figure 1]a. There was mild scaling over the lesion in the ear. The tumid swelling over the ear lobule showed erosion in the posterior aspect of the lesion with no scarring in the ear [Figure 1]b. A small plaque with minimal scaling and crusting was seen over left cheek [Figure 1]a. We also noticed two small shiny elevated nodules over the back [Figure 1]c. The systemic examination showed no abnormalities and no lymphadenopathy. Complete hemogram and other blood investigations were within normal limits. Chest X-ray was normal, and Mantoux test was positive with 17 mm in diameter [Figure 1]d. Skin biopsy of the ear lesion showed sheets of lymphocytes with multiple tubercles composed of lymphocytes, histiocytes, epithelioid cells, and Langhan's giant cells and some areas showing caseous necrosis [Figure 2]. Skin biopsy from the nodular lesion over the back also showed epithelioid cell granuloma composed of lymphocytes, histiocytes, and Langhan's giant cell in the dermis [Figure 3]. The patient was diagnosed as a case of lupus vulgaris, and she was started on antituberculous drugs composed of rifampicin, isoniazid, pyrazinamide, and ethambutol. She responded well to the antituberculous medication with reduction in size of the lesion [Figure 4].
|Figure 1: (a) Myxomatous swelling in ear lobule with scaly, crusted plaque over the cheek. (b) Erosion over the ear lesion. (c) Papulonodular over the back. (d) Positive Mantoux test|
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|Figure 2: (a) Epithelioid cell granulomas with lymphohistiocytic infiltrate and Langhan's giant cell (black arrow) in the ear (H and E, ×100). (b) Histiocytic granuloma with epithelioid cells and plenty of lymphocytes in the ear (H and E, ×400). (c) Caseation necrosis within the granuloma (H and E, ×400). (d) Langhan's giant cell (black arrow) in granuloma of ear lesion (H and E, ×400)|
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|Figure 3: (a) Nodular skin lesion showing epithelioid cell granulomas with lymphocytes and histiocytes in the dermis (H and E, ×50). (b) Epithelioid cell granuloma with Langhan's giant cell (black arrow) in the dermis (H and E, ×100)|
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|Figure 4: (a-c) Resolving lesions after 1 month of antituberculosis drugs|
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| Discussion|| |
Cutaneous TB is a form of extrapulmonary TB with lesser prevalence when compared to other forms of TB. Females are commonly affected than males. The clinical spectrum of cutaneous TB varies depending upon the route of infection and the immunological status of the host. The most common type of cutaneous TB is lupus vulgaris with the prevalence of 0.37% among the skin OPD patients. Lupus vulgaris is a chronic, progressive, postprimary form of cutaneous TB, which occurs in individuals with moderate or high degree of immunity. Lupus vulgaris usually results from hematogenous spread from tuberculous focus elsewhere or lymphatic extension from tuberculous adenitis. It also occurs as a result of direct inoculation of the organism into the skin. It may occur following Bacillus Calmette–Guerin vaccination. The lesions are commonly seen over the thighs, buttocks, and extremities in Indian patients, whereas the lesions are predominantly seen over the head and neck region in the western population. Mucosal involvement also occurs either primarily in the form of papule, nodule, or ulcer or by spread from a contiguous skin lesion.
The most common and characteristic lesion of lupus vulgaris is a plaque with apple jelly nodules and shows scarring in some areas with progression of lesion in the other areas. The other clinical types of lupus vulgaris are ulcerative and mutilating type, vegetating form, papular and nodular type, and tumor-like forms. Vegetating form is characterized by marked infiltration, ulceration, and necrosis with minimal scarring. Mucous membrane is invaded, and cartilage is slowly destroyed. In ulcerative and mutilating form, scarring and ulceration predominate. The deeper tissues and cartilages are invaded, and contractures and deformities occur. In milder forms, keratinous plugs overlying pinpoint ulcers are associated with slow scar formation. Papular and nodular forms present as multiple lesions occur in disseminated lupus called as “miliary lupus.” The tumor-like form presents as hypertrophic form with soft tumor-like nodules or hyperkeratotic masses due to epithelial hyperplasia and myxomatous form. The myxomatous form is an unusual type which commonly occurs on the ear lobules. Lymphedema and vasodilatation are sometimes marked with the myxomatous lesions. The other unusual types are frambesiform, gangrenous form, lichen simplex chronicus-like forms, and sporotrichoid forms. Our patient had huge myxomatous lesion in the ear lobule for 20 years which later lead to cutaneous dissemination resulted in the formation of papulonodular form. This clinical presentation with ear lobule involvement was described as “Turkey ear” in few articles and could be used as a sign of lupus vulgaris in addition to sarcoidosis.,
The long-term complications of lupus vulgaris are scarring, mutilation, and cutaneous malignancies, such as squamous cell carcinoma, basal cell carcinoma, and sarcoma., The differential diagnosis considered for lupus vulgaris lesions are lupus pernio of sarcodosis, lupoid form of leishmaniasis, leprosy, fungal infections, psoriasis, Bowen's disease, Wegener's granulomatosis, lymphomas, and histiocytosis. These clinical conditions are differentiated from lupus vulgaris by histopathological examination of skin lesions, culture, special staining techniques, and marker study. The diagnosis of lupus vulgaris mainly depends upon the clinical features, skin biopsy, Mantoux test and the response to antituberculous drugs. Culture of Mycobacterium tuberculosis from the skin biopsy specimens is not successful in all cases because of the presence of few bacilli in lupus vulgaris lesions.
| Conclusion|| |
In all atypical presentations of skin lesions, high index of suspicion is necessary to diagnose lupus vulgaris. The risk of cutaneous dissemination is there in longstanding skin lesions of lupus vulgaris as seen in our case. The earlobe involvement (Turkey ear) can be considered as a sign of lupus vulgaris in Indian patients. Early diagnosis and initiation of anti-TB drugs prevent the late complications such as cutaneous dissemination, scarring, disfiguration, and cutaneous malignancies.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]