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Table of Contents
Year : 2022  |  Volume : 1  |  Issue : 1  |  Page : 63-66

The diagnostic conundrum of lupus vulgaris versus cutaneous sarcoidosis

1 Department of Dermatology, AIIMS Raebareli, Raebareli, Uttar Pradesh, India
2 Department of Pharmacology, AIIMS Raebareli, Raebareli, Uttar Pradesh, India
3 Department of Pathology and Lab Medicine, AIIMS Raebareli, Raebareli, Uttar Pradesh, India

Date of Submission14-Feb-2022
Date of Decision17-Feb-2022
Date of Acceptance19-Feb-2022
Date of Web Publication23-Mar-2022

Correspondence Address:
K Geetha
Department of Dermatology, AIIMS Raebareli, Raebareli, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpdtsm.jpdtsm_12_22

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Infection with Mycobacterium tuberculosis causes tuberculosis (TB). Lupus vulgaris is one of the most common forms of cutaneous TB, which affects 1%–2% of TB patients. Sarcoidosis is a multisystem granulomatous disease with an unknown origin, and skin involvement is the second most common symptom. Both TB and sarcoidosis are granulomatous diseases. Depending on investigations, it is often hard to distinguish sarcoidosis from TB, especially when serum angiotensin-converting enzyme levels are high in certain cases of TB with negative acid-fast staining in the biopsy specimen. This is a report of two patients where there was trouble distinguishing between sarcoidosis and TB based on laboratory reports but was eventually diagnosed with cutaneous TB based on the Mantoux, QuantiFERON-TB Gold test, and histopathological pattern. Anti-TB therapy was administered to those patients, and the skin lesions resolved completely.

Keywords: Cutaneous sarcoidosis, cutaneous tuberculosis, Mantoux

How to cite this article:
Geetha K, Puja, Kumari N, Gupta S. The diagnostic conundrum of lupus vulgaris versus cutaneous sarcoidosis. J Prev Diagn Treat Strategies Med 2022;1:63-6

How to cite this URL:
Geetha K, Puja, Kumari N, Gupta S. The diagnostic conundrum of lupus vulgaris versus cutaneous sarcoidosis. J Prev Diagn Treat Strategies Med [serial online] 2022 [cited 2022 May 24];1:63-6. Available from: http://www.jpdtsm.com/text.asp?2022/1/1/63/340550

  Introduction Top

Cutaneous tuberculosis (TB) is a rare manifestation of TB, accounting for only 8.4%–13.7% of all TB cases. It accounts for 1%–1.5% of all extrapulmonary TB presentations. Despite its rarity, given its global prevalence, physicians have to be able to discern between the numerous clinical variants of cutaneous TB and the pretender infections such as granulomatous syphilis, discoid lupus erythematosus, psoriasis, tuberculoid leprosy, sarcoidosis, actinomycosis, mycetoma, and bacterial abscesses. The sensitivity and specificity of most cutaneous TB diagnostic techniques are low. As a result, clinicians must use every available test in conjunction with comprehensive clinical assessment. Thus, the accumulation of affirmative fundamentals would be vital in evaluating specific diagnosis.

TB of the skin can be divided into two broad categories based on the pathogen load on the skin. Tuberculous chancre, scrofuloderma, orificial TB, acute miliary TB, and tuberculous gumma are all multibacillary forms (easily detectable in skin tissue). TB verrucosa cutis, tuberculoid, and lupus vulgaris are examples of paucibacillary forms (bacilli are rare).[1]

Lupus vulgaris is a form of cutaneous TB that progresses in people with a moderate to a high level of immunity. Direct inoculation, direct extension from an underlying organ, or lymphatic spread are all possible causes. It can also happen as a result of hematogenous spread from an infective focus on rare occasions. The head and neck are the most commonly involved areas, followed by the arms and legs. The lesion usually appears as a small reddish-brown nodule that becomes raised and infiltrated over time. Lupus vulgaris manifests itself clinically in five distinct patterns: plaque form, ulcerative and mutilating form, vegetating form, tumor-like form, and papular and nodular form. The border of the plaque is well defined, with little infiltration and scaling and minimal scarring at the center. Crusts form over areas of necrosis and scarring in the ulcerating form, and ulceration predominates. The vegetating form is distinguished by prominent ulceration and extensive infiltration.[2]

Sarcoidosis is a granulomatous disease with an unknown origin that impacts different parts of the body. Skin involvement has been noted in approximately 25% of cases and typically starts at disease onset. Macules and papules seem to be the most common clinical manifestations of specific lesions, but plaques, nodules, lupus pernio, subcutaneous infiltrates, and infiltration of preexisting scars are also common. It is an exclusionary diagnosis that is made when granulomatous inflammation is found in the absence of an infectious or other cause.[3]

  Case Reports Top

Case 1

A 48-year-old woman came to our skin outpatient department with complaints of asymptomatic reddish raised skin lesions over the left arm for the past 6 months. It started as a small lesion and gradually increased in size. Her blood tests showed a slightly elevated erythrocyte sedimentation rate (ESR) (32 mm/h) with leukocytosis. Her serum angiotensin-converting enzyme (ACE) level was 307 nmol/ml/min, and her chest X-ray was normal. The differential diagnosis of sarcoid and skin TB was made with blood reports. Mantoux and skin biopsies were later performed. Mantoux was positive with a skin induration of 24 mm, which is more likely to be due to tuberculous infection. Histopathology revealed a moderately spongiotic psoriasiform epidermis with a patchy nodular tuberculoid granulomatous infiltrate of lymphocytes, plasma cells, histiocytes, and epithelioid cells with occasional Langhans giant cells. The diagnosis of lupus vulgaris was made and anti-TB therapy (ATT) was initiated. The patient responded well with complete recovery of the skin lesion [Figure 1].
Figure 1: Lupus vulgaris before and after antituberculosis therapy

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Case 2

A 33-year-old female patient presented for 2 years with slightly itchy, hypopigmented plaques with few erythematous papules on the dorsal and lateral side of the bridge of the nose [Figure 2]. She was treated with various topical medications such as mometasone cream, tacrolimus cream, and sunscreen lotions along with systemic prednisolone, azathioprine, and antihistamines on various sites with minimal improvement. A differential diagnosis of sarcoid and skin TB was made. Blood test reports showed elevated ESR (55 mm/h) and serum ACE values (123 nmol/ml/min). Her serum calcium was normal. The Mantoux test was positive with a reading of 28 mm. Because the patient was reluctant to have a facial biopsy, a QuantiFERON-TB Gold test was performed to confirm TB. However, the report was negative. Therefore, after approval, a skin biopsy was performed which showed AFB-positive with an extensive collection of lymphomononuclear infiltrate with epithelioid histiocytes [Figure 3]. The diagnosis was confirmed as cutaneous TB (lupus vulgaris type), and the patient was initiated on ATT.
Figure 2: Lupus vulgaris over the nose

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Figure 3: Histopathological image

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  Discussion Top

Serum ACE levels were elevated in both of the above patients, which was confusing. Elevated serum ACE levels are not entirely specific to sarcoid, and elevated levels have also been noted in diseases more likely to be confused with sarcoid, particularly TB. The main source of SACE in healthy subjects is the endothelial cells of the pulmonary vascular bed. It is believed that increased enzyme levels in sarcoid disease are due to increased enzyme synthesis by the granuloma epithelial cells and those serum levels of this enzyme may reflect the granuloma load on the body. Based on our study, it is recommended that a SACE be a useful differential diagnostic marker at concentrations above 100 IU/L or <52 IU/L in the diagnosis of sarcoid, but it is not appropriate when viewed alone for differential diagnosis. In this case, the confirmatory diagnosis requires additional examinations.[4]

The Mantoux test is usually not part of diagnosing active TB infection. However, the cutaneous TB group agreed that it could be used as an additional test in select cases where the diagnosis was inconclusive. However, only a strongly positive result (with a diameter of 22 mm or more when reading) supports the diagnosis of cutaneous TB.[5] Serum markers related to sarcoidosis, including soluble interleukin-2 receptor, ACE, and Krebs van den Lungen-6, are not sufficiently sensitive or specific to separate these diagnoses. It has recently been observed that the combination of leptin and intercellular adhesion molecule-1 has a sensitivity of 86.5% and a specificity of 73.1% as a marker of sarcoidosis, and this combination could therefore be employed to differentiate the two conditions.[6]

The distinction between sarcoidosis and TB is particularly important in South East Asia, where TB is common and may have a similar clinical presentation as that of sarcoidosis, but requires specific and more prolonged therapy. Some points for differentiating both lupus vulgaris and cutaneous sarcoidosis are given in [Table 1].[7],[3] [Table 1] Real-time quantitative polymerase chain reaction analysis of specific messenger RNA and microRNA is likely required as a sensitive, accurate, and rapid diagnostic test for the detection of traces of TB in sarcoid. It is a significant challenge for diagnosing sarcoidosis in regions with a high burden of TB. Bettered individual tests including genetic tests can ameliorate our knowledge and help in distinguishing these two conditions.[8],[9]
Table 1: Diagnostic differences between lupus vulgaris and cutaneous sarcoidosis

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In our case reports, initially, there was confusion in diagnosis based on blood reports alone without Mantoux test and histopathology. In any case with a suspected chronic granulomatous disorder, the Mantoux test is mandatory and histopathology is confirmatory to make the correct diagnosis and also to rule out TB.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patients have given her consent for her images and other clinical information to be reported in the journal. The patients understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Khadka P, Koirala S, Thapaliya J. Cutaneous tuberculosis: Clinicopathologic arrays and diagnostic challenges. Dermatol Res Pract 2018;2018:7201973. [doi: 10.1155/2018/7201973].  Back to cited text no. 1
Hassan I, Ahmad M, Masood Q. Lupus vulgaris: An atypical presentation. Indian J Dermatol Venereol Leprol 2010;76:180-1.  Back to cited text no. 2
[PUBMED]  [Full text]  
Singh P, Jain E, Dhingra H, Mohan H, Thami GP. Clinico-pathological spectrum of cutaneous sarcoidosis: An experience from a government institute in North India. Med Pharm Rep 2020;93:241-5.  Back to cited text no. 3
Ramam M, Malhotra A, Tejasvi T, Manchanda Y, Sharma S, Mittal R, et al. How useful is the Mantoux test in the diagnosis of doubtful cases of cutaneous tuberculosis? Int J Dermatol 2011;50:1379-82.  Back to cited text no. 4
Vyas S, Thangakunam B, Gupta R, Michael JS, Christopher DJ. Interferon gamma release assay and tuberculin skin test positivity in sarcoidosis. Lung India 2015;32:91-2.  Back to cited text no. 5
  [Full text]  
de la Fuente-Meira S, Gracia-Cazaña T, Pastushenko I, Ara M. Sarcoidosis and tuberculosis: A diagnostic challenge. Actas Dermosifiliogr 2016;107:605-7.  Back to cited text no. 6
Santos JB, Figueiredo AR, Ferraz CE, Oliveira MH, Silva PG, Medeiros VL. Cutaneous tuberculosis: Diagnosis, histopathology and treatment – Part II. An Bras Dermatol 2014;89:545-55.  Back to cited text no. 7
Mortaz E, Masjedi MR, Abedini A, Matroodi S, Kiani A, Soroush D, et al. Common features of tuberculosis and sarcoidosis. Int J Mycobacteriol 2016;5 Suppl 1:S240-1.  Back to cited text no. 8
Hayati F, Zainudin SP, Abidin ZA. Cutaneous sarcoidosis: A case of the great imitator. Oman Med J 2020;35:e184.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]


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