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Revista da Sociedade Portuguesa de Dermatologia e Venereologia

versão impressa ISSN 2182-2395versão On-line ISSN 2182-2409

Rev Soc Port Dermatol Venereol vol.79 no.1 Lisboa mar. 2021  Epub 15-Jun-2021

https://doi.org/10.29021/spdv.79.1.1284 

Case Reports

Lupus Vulgar: Um Diagnóstico Desafiador

Lupus Vulgaris: A Diagnostic Challenge

Hisabella Lorena Simões Porto¹ 
http://orcid.org/0000-0001-7746-6405

Isabela Alves Guerra¹ 
http://orcid.org/0000-0001-5578-3813

Bárbara Figueiredo Bastos¹ 
http://orcid.org/0000-0002-1682-2290

Marina Ferreira¹ 
http://orcid.org/0000-0001-75828732

Marcelino Pereira Martins Neto²  3 
http://orcid.org/0000-0003-4621-1092

Maria Christina Marques Nogueira Castañon4  5 
http://orcid.org/0000-0002-2995-1761

1Residente de Dermatologia / Dermatology Resident at Federal University of Juiz de Fora Teaching Hospital, Minas Gerais, Brazil

2Professor de Dermatologia / Professor of the Dermatology Department at Presidente Antonio Carlos University

3Doutorando em Ciências Biomédicas / Doctoral student in Biomedics Science at Rosário University (IUNIR), Rosário, Argentina

4Professor titular e pesquisador / Full professor and Reseacher at the Institute of Biological Sciences of the Federal University of Juiz de FFora, Minas Gerais, Brazil

5Assistente de Dermatologia e Dermatopatologia / Consultant of Dermatology and Dermatopathology / Federal University of Juiz de Fora, Minas Gerais, Brazil


Resumo:

Lúpus vulgar é uma variante clínica de tuberculose cutânea, subtipo raro de infecção extrapulmonar causada pelo complexo Mycobacterium tuberculosis. Forma paucibacilar associada a alto grau de imunidade, predominantemente na face, pode apresentar vários diagnósticos diferenciais clínicos e histopatológicos, o que torna seu diagnóstico desafiador.

Apresentamos um caso de lúpus vulgar em paciente imunocompetente, cuja apresentação clínica e histopatológica iniciais não sugeriam o diagnóstico. O objetivo é ressaltar a relevância da hipótese de tuberculose cutânea como importante diagnóstico diferencial, principalmente em áreas endémicas.

Palavras-chave: Lúpus Vulgar/diagnóstico; Teste tuberculínico; Tuberculose Cutânea

Abstract

Lupus vulgaris is a clinical variant of cutaneous tuberculosis, a rare subtype of extrapulmonary infection caused by the Mycobacterium tuberculosis complex. A paucibacillary form associated with high degrees of immunity, predominantly in the face, may present several clinical and histopathological differential diagnoses, which makes its diagnosis challenging.

We present a case of lupus vulgaris in an immunocompetent patient, whose initial clinical presentation and histopathology did not suggest the condition. The objective is to highlight the relevance of the hypothesis of cutaneous tuberculosis as an important differential diagnosis, especially in endemic areas.

Keywords: Lupus Vulgaris/diagnosis; Tuberculin Test; Tuberculosis, Cutaneous

Introduction

Cutaneous tuberculosis is a rare form of extrapulmonary infection caused by the Mycobacterium tuberculosis complex, Mycobacterium bovis and Bacillus Calmette-Guerin.1,2There are various clinical forms of this condition depending both on individual immunological factors, characteristics of the bacillus, environmental factors and the inoculation pathway.3 Although considered the most common subtype of cutaneous tuberculosis in studies conducted in Spain, China, Indian, Japan and Pakistan, it is known that lupus vulgaris is underestimated and its diagnosis can be neglected.4,5We present an immunocompetent patient with lupus vulgaris whose clinical and histopathological features were challenging in establishing the diagnosis.

Case Report

A 35-year-old male patient, a former truck driver, immunocompetent and without relevant pathological antecedents, sought medical care in December 2015, with an asymptomatic erythematous papule presenting a bright surface, half a centimeter in diameter, in the left malar region, with 6 months of evolution. With the diagnosis of an acneiform lesion, topical erythromycin 2% was prescribed. The lesion progressed and within approximately six months it presented as an asymptomatic erythematous-infiltrated oval plate with well-defined limits, 1 cm in diameter. Histopathology of 2 incisional biopsies was consistent with granulomatous rosacea, and a topical immunomodulator was instituted (tacrolimus ointment). When the patient was observed again after four months there was persistence of the plaque, but with a few pustules (Fig. 1). On diascopy it presented the apple jelly sign (Fig. 2). New diagnostic hypotheses were suggested, including sarcoidosis. A new biopsy showed a granulomatous inflammatory process (tuberculoid granuloma), without necrosis (Fig. 3). Histochemical studies for fungi (GMS and PAS) and BAAR (FITE-FARACO) were negative as well as the immunohistochemical study with antibody anti BCG (Dako B 0124, Copenhagen). Culture and IGRA (interferon gama releasing assay) Quantiferon TB Gold were not performed. The close correlation of the histopathological findings with the clinical data directed the investigation towards lupus vulgaris. A PPD (tuberculin test) revealed an eleven millimeter papule and chest X-ray was normal.

Treatment was initiated with rifampicin 600 mg, isoniazid 300 mg, pirazinamide 1600 mg and etambutol 1100 mg daily (RHZE) for two months followed by rifampicin 600 mg and isoniazid 300 mg daily for the next 4 months. There was a significant improvement of the lesion in the first months with resolution by the end of the treatment with a slight atrophy. No recurrence of the condition was observed after three years (Fig. 4).

Figure 1: (A) Lesion before treatment (B) Details of plaque and appearance of micropustules on it. 

Figure 2: Diascopy Showing the apple jelly sign. 

Figure 3: A and B: Dermal inflammatory infiltrate consisting of lymphocytes and histiocytes forming epithelioid granulomas without central necrosis. H&E 100X and 200X respectively 

Figure 4: (A) Regression of the lesion after 6 months of RHZE. (B) Detail of the left malar region after treatment. 

Discussion

Current data from the literature indicate that cutaneous tuberculosis accounts for 1%-2% of all extrapulmonary forms of tuberculosis in the world, therefore a rare condition, especially in industrialized countries.1,6In Europe, the predominant form of cutaneous tuberculosis is lupus vulgaris, which may represent 61.1% of the cases,7 whereas scrofuloderma is the most common presentation in some developing countries such as Brazil.5 According to the WHO, Brazil is one of the twenty countries in the ranking with the highest incidence of tuberculosis,8 and 40% of patients over 45 years of age with typical cutaneous lesions may have coexistent pulmonary tuberculosis, 9 which was not apparently the case of this patient.

Cutaneous manifestations of tuberculosis are classified into true cutaneous tuberculosis or tuberculids.3 Cutaneous tuberculosis includes a continuous spectrum according to the degree of immunity and the route of infection. Some authors further define the infection according to the amount of bacilli found in skin lesions as paucibacillary or multibacillary, the latter being associated with more deficient degrees of immunity.2,4,9

In lupus vulgaris, it is known that individuals have moderate to high degrees of cell-mediated immunity, with adequate immune activation and normal serum immunoglobulin levels. The number of bacilli in the skin is small, and they are not detected in special staining in histopathology or PCR and most will not be able to grow in culture.2,4

Lupus vulgaris is secondary to a pre-existing focus, especially in the joints, bones or lymph nodes, and lesions depend mainly on the hematogenous and lymphatic spread of the microorganism.10 In rare cases, the disease may develop after inoculation or an exogenous source.11 When it is not possible to find the focus, it is postulated that the picture may have been triggered by reactivation of latent cutaneous focus secondary to silent bacteremia.10

In western countries lupus vulgaris occurs most commonly in the head and cervical region. and presents in discrete and varied forms, having four predominant types: hypertrophic, ulcerative, vegetative and plaque-type, which is the form in this patient. In this clinical presentation, lesions consist mostly on a brownish red papule or plaque, with progressive infiltration, an “apple jelly” appearance on diascopy (Fig.s 1 and 2) and tendency to centrifugal growth with central atrophy.3,4

In general, cutaneous tuberculosis may have no systemic symptoms and the skin lesion is not specific, which can make the diagnosis a great challenge for dermatologists in daily practice.6,12Lesional characteristics in this patient associated with the high level of immunity precluded an early diagnosis. At this stage lupus vulgaris may mimic other dermatoses such as sporotrichosis, cutaneous leishmaniasis, discoid lupus erythematosus, sarcoidosis, acne rosacea, nummular dermatitis, psoriasis, lichen simplex chronicus, dermatophytosis and erythema annulare centrifugum.4,10,12

If untreated, lupus vulgaris lesions persist for years, gradually growing in size up to tens of centimeters, with ulcerations and tissue destruction, leading to significant aesthetic disfigurement. Malignant transformation into squamous cell carcinoma can occur in 0.5% to 10.5% usually after 25-30 years of untreated disease.13

The diagnosis is based on clinic and histopathological findings, bacterial cultures and PCR.6 As lupus tuberculosis tends to occur in previously sensitized patients, PPD is usually positive, as in this patient, but its diagnostic value is considered controversial. Culture of the collected material may or may not show Koch's bacillus.2,9

Common histopathological findings are pseudoepitheliomatous hyperplasia associated with multiple and well developed tuberculoid granulomas, with or without caseous necrosis in the upper dermis. Non-specific inflammatory infiltrate and absence of bacillus are other striking features.2,3

Negative culture implies searching for bacillus identification through PCR or other technique. Although these tests are the gold standard for the diagnosis, they have a low sensitivity: 50% a 72% for PCR and 80% for culture in samples with BAAR negatives.6 In the absence of such examination, a therapeutic test can be used, as corroborated by Pinho A et al and Thomas S et al.10,14,15In this patient after a two months treatment with rifampicin, isoniazid, pyrazinamide and etambutol followed by four months of rifampicin and isoniazid there was a complete regression of the lesion (Fig. 4).

We conclude from this case report the importance of thinking of lupus vulgaris in immunocompetent patients who present indolent cutaneous lesions with various clinical presentations, with or without an obvious primary focus of tuberculosis in a patient with a positive PPD or IGRA, particularly in endemic areas.

References

Zhang J, Fan YK, Wang P, Chen QQ, Wang G, Xu AE, et. al. Cutaneous tuberculosis in China. A multicentre retrospective study of cases diagnosed between 1957 and 2013. J Eur Acad Dermatol Venereol. 2018;32:632-8. doi: 10.1111/jdv.14851. [ Links ]

Dias MF, Filho FB, Quaresma MV, Nery JA, Azulay DR. Update on cutaneous tuberculosis. An Bras Dermatol. 2014;89:925-38. [ Links ]

Barbagallo J, Tager P, Ingleton R .Hirsch RJ, Weinberg JM. Cutaneous Tuberculosis. Am J Clin Dermatol. 2002;3:319-28. [ Links ]

Verma S, Verma G, Shanker V, Tegta GR, Sharma A, Pandey ML. Facial lupus vulgaris of bilateral periorbital skin and conjunctiva: A case report and brief review. Indian J Med Microbiol. 2015;33:168-71. doi: 10.4103/0255-0857.148438. [ Links ]

Mann D, Sant'Anna FM, Schmaltz CA, Rolla V, Freitas DF, Lyra MR, et al. Cutaneous tuberculosis in Rio de Janeiro, Brazil: description of a series of 75 cases. Int J Dermatol. 2019;58:1451-9. doi: 10.1111/ijd.14617. [ Links ]

Francisco T, Cunha D, Vieira R, Afonso A, Brito JM. Infecção cutânea e pulmonar por Mycobacterium Africanum. Reva Soc Port Dermatol Venereol. 2013;71:131-5. [ Links ]

Granado J, Catarino A. Cutaneous tuberculosis presenting as lupus vulgaris. Int J Infect Dis. 2020;96:139-40. doi: 10.1016/j.ijid.2020.03.069. [ Links ]

World Health Organization. Global tuberculosis report 2019. [access in 2020 Aug 20]. Available at: Available at: https://apps.who.int/iris/bitstream/handle/10665/329368/9789241565714-eng.pdf?ua=1Links ]

Hay RJ. Cutaneous tuberculosis and the control of infection in resource-poor environments. Dermatology. 2008;217:94-6. [ Links ]

Pai VV, Naveen KN, Athanikar SB, Dinesh US, Divyashree A, Gupta G. A clinico-histopathological study of lupus vulgaris: A 3-year experience at a tertiary care centre. Indian Dermatol Online J. 2014;5:461-5. [ Links ]

Mataix J, Botella R, Herrero A, Lucas A. Tuberculous primary complex of the skin. Int J Dermatol. 2008,47: 479-81. [ Links ]

Chen Q, Chen W, Hao F. Cutaneous tuberculosis: A great imitator. Clin Dermatol. 2019;37:192-9. doi: 10.1016/j.clindermatol.2019.01.008. [ Links ]

Santos JB, Figueiredo AR, Ferraz CE, Oliveira MH, Silva PG, Medeiros VL. Cutaneous tuberculosis: epidemiologic, etiopathogenic and clinical aspects - part I. An Bras Dermatol. 2014;89:219-28. doi: 10.1590/abd1806-4841.20142334 [ Links ]

Pinho A, Ferreira B, JCardoso JC, Moreno A, Reis JP. Lesões puched-out da glande. Reva Soc Port Dermatol Venereol. 2017;75:201-3. [ Links ]

Thomas S, Suhas S, Pai KM, Raghu AR. Lupus vulgaris - Report of a case with facial involvement. Br Dent Jl. 2005; 198: 135-7. doi: 10.1038/sj.bdj.4812038. [ Links ]

Responsabilidades Éticas Conflitos de Interesse: Os autores declaram a inexistência de conflitos de interesse na realização do presente trabalho. Suporte Financeiro: Não existiram fontes externas de financiamento para a realização deste artigo. Confidencialidade dos Dados: Os autores declaram ter seguido os protocolos da sua instituição acerca da publicação dos dados de doentes. Consentimento: Consentimento do doente para publicação obtido. Proveniência e Revisão por Pares: Não comissionado; revisão externa por pares

Ethical Disclosures Conflicts of Interest: The authors have no conflicts of interest to declare. Financing Support: This work has not received any contribution, grant or scholarship. Confidentiality of Data: The authors declare that they have followed the protocols of their work center on the publication of data from patients. Patient Consent: Consent for publication was obtained. Provenance and Peer Review: Not commissioned; externally peer reviewed

3© Author(s) (or their employer(s)) and SPDV Journal 2020. Re-use permitted under CC BY-NC. No commercial re-use. © Autor (es) (ou seu (s) empregador (es)) e SPDV Revista 2020. Reutilização permitida de acordo com CC BY-NC. Nenhuma reutilização comercial

Recebido: 11 de Outubro de 2020; Aceito: 29 de Novembro de 2020

Autor Correspondente/ Corresponding Author: Hisabella Lorena Simões Porto Medical Clinical (Dermatology) Department of the Federal University of Juiz de Fora Teaching Hospital (HU-UFJF) Address: Eugênio do Nascimento Street, Juiz de Fora - Minas Gerais - Zip Code 36038-330 - Brazil

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