Lupus Vulgaris
Lupus vulgaris (LV) is a form of post-primary, chronic, and paucibacillary cutaneous tuberculosis in individuals with a high tuberculin sensitivity and moderate immunity. It is the most common type of cutaneous tuberculosis.
Presentation
Cutaneous tuberculosis (TB) can be caused by M. tuberculosis, M. bovis, and the Bacillus Calmette–Guérin (BCG) vaccine. The disease has diverse clinical features that can follow immune-mediated processes or endogenous and exogenous spread of the microorganism. The exogenous spread is less common. Even though cutaneous TB comprises less than 1% of all TB cases, it is important to consider it when a clinical picture suggestive of it is found [1]. Exogenous inoculation develops after M. tuberculosis is directly inoculated into the skin of an individual susceptible to the infection. This results in tuberculosis verrucosa cutis (TVC), TB chancre, and in some cases lupus vulgaris (LV). Endogenous spread occurs in previously infected cases by means of contiguous extension, hematogenous dissemination or through lymphatic circulation or in old scars of scrofuloderma [2]. Hematogenous spread is observed in LV, acute miliary TB, papulonecrotic tuberculoid (PNT), and metastatic TB abscess (gummatous TB). A contiguous extension can be seen in orificial TB and scrofuloderma.
LV is the most common type of cutaneous TB and affects females 2 to 3 times more often than males. It is chronic and progressive.The lesions of LV are commonly found in the region of the head and neck [3] and are generally solitary, small, sharply defined, nodular, reddish brown, with a gelatinous consistency (apple jelly nodules). In western countries, these lesions usually appear on the head and neck while in tropical or subtropical regions they develop on lower extremities and the buttocks.
The existing clinical variations are as follows:
- Classic plaque
- Keratotic
- Hypertrophic
- Ulcerative
- Vegetating
The classic plaques begin as discrete red-brown papules. They coalesce and form soft and gelatinous (apple-jelly) plaques with central atrophy and elevated verrucas border.
Persistent lesions become ulcerated and damage the underlying structures leading to disfigurement and have an increased risk of malignant transformation.
Immune System
Cervical Lymphadenopathy
- General and systemic examinations findings were insignificant, except for bilateral cervical lymphadenopathy. The body mass index was 16.6 Kg/m 2.[smjonline.org]
- RDD, originally known as sinus histiocytosis with massive lymphadenopathy, is a benign histiocytic proliferative disorder characterized clinically by a massive, painless, often bilateral cervical lymphadenopathy accompanied by fever, neutrophilia, anemia[scielo.br]
- Bilateral cervical lymphadenopathy was present. The rest of the systemic findings and vitals were normal. Considering the possibilities of sporotrichosis, chromoblastomycosis, cutaneous leishmaniasis and CTB, a skin biopsy was taken.[ijmm.org]
Entire Body System
Turkish
- A 46-year-old Turkish woman presented with a 42-year history of erythamatous plaque on her left arm. Ziehl-Neelsen and periodic acid-Schiff stains did not show any acid-fast bacilli.[ncbi.nlm.nih.gov]
- Case presentation A 46-year-old Turkish woman presented to our institute with a red, well-demarcated plaque on her left arm. She said the lesion had appeared when she was 4 years old.[jmedicalcasereports.biomedcentral.com]
Recurrent Infection
- After 6 months of ear piercing, she suffered from recurrent infections at the site of piercing in the left ear, while the other ear was normal.[ijorl.mums.ac.ir]
Relapsing Polychondritis
- Other diseases involving the ear such as pseudolymphoma, leprosy, perichondritis and relapsing polychondritis were clinically ruled out in our case.[scielo.br]
Respiratoric
Dry Cough
- There was now a history of malaise, fever, dry cough, and anorexia and weight loss for the past 2 months. The patient was fully vaccinated in childhood, including against varicella infection.[ncbi.nlm.nih.gov]
Skin
Cutaneous Manifestation
- Leprosy predominantly presents through skin manifestations whereas cutaneous manifestations of TB though not so frequent but are not rare.[ncbi.nlm.nih.gov]
- Its most common cutaneous manifestation is lupus vulgaris which is seldom diagnosed today.[ncbi.nlm.nih.gov]
- Cutaneous manifestations of secondary syphilis are protean and skin tuberculosis may be considered in the differential diagnosis, especially in HIV-infected patients.[ncbi.nlm.nih.gov]
- Cutaneous manifestations of disseminated tuberculosis are unusual, being seen in less than 0.5% of cases. Scrofuloderma, tuberculosis verrucosa cutis and lupus vulgaris comprise most cutaneous tuberculosis cases.[ncbi.nlm.nih.gov]
- IMAGE SERIES HANDPICKED PROF.DR.G.SUNDARAMURTHY’S UNIT M7 DR.K.BHARGAVI 3. 1.LUPUS VULGARIS CUTANEOUS MANIFESTATION OF TB SMOLDERING DISEASE WITH NODULES,PLAQUES AND FISSURES STARTS AS AN ANNULAR INDURATING PLAQUE PROGRESSING IN SIZE OVER DECADES APPLE[slideshare.net]
Skin Ulcer
- If left untreated, the skin lesions can mature into skin ulcers. The term 'serpiginosus' means 'slow, snakelike' movement over the body, which leaves scarring.[wellcomecollection.org]
- There were multiple, nontender skin ulcers on bilateral lumbar areas, some oozing serosanguinous discharge and few others with scarring [Figure 1] .[lungindia.com]
- There were multiple, nontender skin ulcers on bilateral lumbar areas, some oozing serosanguinous discharge and few others with scarring [ Figure 1 ].[ncbi.nlm.nih.gov]
Facial Skin Lesion
- Int J Dermatol 40: 336-339. [ Crossref ] Werschler WP, Elgart ML, Williams CM (1990) Progressive asymptomatic annular facial skin lesions. Cutaneous tuberculosis (lupus vulgaris).[oatext.com]
Excoriation
- Figure 1 (a) Multiple erythematous excoriated papules over the left forearm. (b) Erythematous scaly plaques and mild atrophic changes over both legs and feet.[ejdv.eg.net]
- The great advantage, according to Hebra, of this arsenical treatment consists in its not injuring or even excoriating the healthy skin, while the morbid tissue is safely and thoroughly destroyed.[chestofbooks.com]
- […] and nodular vasculitides (eg, syphilitic gumma, nodular pernio) Differentiate papulonecrotic tuberculid from other papulonecrotic entities, such as leukocytoclastic vasculitis, lymphomatoid papulosis, papular eczema, and prurigo simplex with neurotic excoriation[emedicine.medscape.com]
Skin Plaque
- Histopathological examination of skin plaque showed typical tuberculoid granulomas in the dermis. However blood sugar, sputum for AFB, LFT including serum enzymes were within normal limits. Blood sent for HIV was found to be negative.[ijdvl.com]
Face, Head & Neck
Malar Rash
- Malar Rash About half of people with lupus experience a characteristic red “malar” rash or color change that may appear across the cheeks and bridge of the nose in the shape of a butterfly.[lupusresearch.org]
Facial Ulceration
- Original Article O Fatusi, O Onayemi, K Adebiyi, V Adetiloye, F Owotade, O Oninla Keywords facial ulcer, histopathological diagnosis, lupus vulgaris, tuberculosis cutis orificialis Citation O Fatusi, O Onayemi, K Adebiyi, V Adetiloye, F Owotade, O Oninla[ispub.com]
Neurologic
Neglect
- Here, we report a 56 year-old patient diagnosed with skin squamous cell carcinoma and with a 30 year course of neglected lupus vulgaris, which was very rare.[ncbi.nlm.nih.gov]
- The buttocks are common but often neglected sites for lupus vulgaris in children. We present a case report of lupus vulgaris occurring on the buttocks, and discuss our approach in diagnosis and treatment.[content.iospress.com]
- Facial lupus vulgaris neglected for 50 years.[jddsjournal.org]
Workup
The diagnosis of LV can be a challenging task as the skin changes are atypical and there is a paucity of TB bacilli in the lesions.
Skin biopsy and histology may show tubercules or tuberculoid granulomas without caseation in the papillary dermis with few or no bacilli, and variable epidermal hyperplasia [4] [5]. If a superficial biopsy is obtained then pseudo-epithelial hyperplasia with a non-specific inflammatory cell infiltrate may be observed, missing the features of LV [6].
Identification of Mycobacteria is essential for the definitive diagnosis of cutaneous TB but this is usually impossible since there are few bacilli. M. tuberculosis rarely grows on culture when the sample is taken from a patient with high levels of immunity or from chronic lesions.
Special tests like the polymerase chain reaction (PCR) can be performed to confirm the diagnosis or when the histopathological findings are not characteristic of the condition [7].
Ziehl-Neelsen staining and PCR may also give negative results in LV due to the paucity of TB bacilli [8] [9].
Diagnosis relies heavily on clinical features, histopathological findings, a positive purified protein derivative test (PPD) and response to anti-TB medication [2] [10].
The differential diagnosis of cutaneous TB includes leprosy, leishmaniasis, syphilis, actinomycosis, and deep mycosis.
Skin Test
Skin Test Positive
- Verhagen et al demonstrated that large clusters of TB are associated with an increased number of tuberculin skin test–positive contacts, even after adjusting for other risk factors for transmission. [24] The number of positive contacts was significantly[emedicine.com]
Microbiology
Mycobacterium Fortuitum
- We report a woman with a lupus vulgaris-like skin eruption caused by Mycobacterium fortuitum.[ncbi.nlm.nih.gov]
- Mycobacterium fortuitum infection arising in a new tattoo. Dermatol Online J. 2014;15:20.[ijord.com]
Treponema Pallidum
- Serology for syphilis was positive, and immunohistochemistry confirmed the presence of Treponema pallidum in lesional skin. A diagnosis of secondary syphilis was made, and the patient was successfully treated with benzathine penicillin G.[ncbi.nlm.nih.gov]
- External Links Miliary tuberculosis Tuberculosis v t e Infectious diseases · Bacterial diseases : G- (primarily A00-A79, 001-041,080-109 ) Spirochaete Treponema Treponema pallidum ( Syphilis / Bejel, Yaws ) · Treponema carateum ( Pinta ) Borrelia Borrelia[wikidoc.org]
Other Pathologies
Granulomatous Tissue
- CLINICAL FEATURES The skin covering the inflamed lymph nodes becomes indurated , purplish in color followed by ulceration with crusty ,irregular pale granulomatous tissue ending in scarring.[drmhijazy.com]
Biopsy
Liver Biopsy
- Liver biopsy was not carried out in this patient. She was diagnosed and treated with ATT as a case of disseminated tuberculosis.[ijdvl.com]
Treatment
- Strong clinical suspicion, histopathology, and response to antituberculous treatment led to the diagnosis and all three had excellent response to treatment.[ncbi.nlm.nih.gov]
- A diagnosis of lupus vulgaris was made based on histopathology, culture and polymerase chain reaction, and isoniazid, rifampicin and ethambutol were administered as antituberculosis treatment.[ncbi.nlm.nih.gov]
- We report our experience and emphasize on recent advances in the diagnosis and treatment of paediatric skin TB.[ncbi.nlm.nih.gov]
- Prompt evaluation and treatment is necessary to prevent disfigurement and systemic involvement associated with DLE. The following case presented a diagnostic dilemma as the lesion mimicked lupus vulgaris.[ncbi.nlm.nih.gov]
- The eruption resolved after treatment with amikacin and clarithromycin. Lupus vulgaris-like lesions might be included in the clinical spectrum of infections caused by rapidly growing mycobacteria.[ncbi.nlm.nih.gov]
Prognosis
- New search Scientific articles from MEDLINE for "Lupus Vulgaris" - English - French - German - Spanish - Portuguese - Italian All recent articles Therapy: by recall / by precision Diagnosis: by recall / by precision Etiology: by recall / by precision Prognosis[hon.ch]
- […] fungal infection [4] Management [ edit ] A dermatologist or general physician usually administers combination therapy of drugs used for tuberculosis, such as Rifampicin, Isoniazid and Pyrazinamide (possibly with either streptomycin or ethambutol ). [3] Prognosis[en.wikipedia.org]
Etiology
- Consult HON's world-wide database of medical meetings Browse - New search Refine the search for "Lupus Vulgaris" Subheadings: complications / diagnosis / embryology / epidemiology / etiology / metabolism / microbiology / surgery / therapy Broader term[hon.ch]
- Lupus vulgaris is also known as tuberculosis luposa cutis or tuberculosis cutis luposa because of its etiologic agent.[wisegeek.com]
- The etiology of RDD remains unknown. It is believed that RDD represents an exaggerated immune response to an infectious agent due to the polyclonal nature of infiltrating cells and the clinical course of the disease.[scielo.br]
- In this case, chronic non-healing ulceration may have been the etiological factor for the development of basal cell carcinoma. [5] To conclude, this case emphasizes the rare possibility of malignancies in longstanding cases of lupus vulgaris.[ijpmonline.org]
Epidemiology
- BACKGROUND: The epidemiological aspects and diagnostic problems encountered in a rural set up are largely unknown.[ncbi.nlm.nih.gov]
- Consult HON's world-wide database of medical meetings Browse - New search Refine the search for "Lupus Vulgaris" Subheadings: complications / diagnosis / embryology / epidemiology / etiology / metabolism / microbiology / surgery / therapy Broader term[hon.ch]
- History and epidemiology of tattoos and piercings. Legislation in the United States. In: Dermatologic Complications with Body Art. 2009: 1–11. Goldstein N. Tattoos defined. Clin Dermatol. 2007;25(4):417–20. Sanghavi SA, Dongre AM, Khopkar US.[ijord.com]
- An appraisal of epidemiologic, clinical, bacteriologic, histopathologic, and immunologic parameters in cutaneous tuberculosis. Int J Dermatol. 1987; 26 :521–6. [ PubMed ] [ Google Scholar ] 15. Ramam M, Mittal R, Ramesh V.[ncbi.nlm.nih.gov]
Pathophysiology
- […] due to inadequately treated pre-existing tuberculosis. [3] It may also develop at site of BCG vaccination. [4] Rarely, it has been shown to be associated with tattoo mark and also with long term bindi use, the so-called "bindi tuberculosis". [5] [6] Pathophysiology[en.wikipedia.org]
- The term "lupus" may derive from the rapacity and virulence of the disease; a 1590 work described it as "a malignant ulcer quickly consuming the neather parts; ... very hungry like unto a woolfe". [3] Pathophysiology Lupus vulgaris often develops due[wikidoc.org]
- Pathophysiology Infection with M tuberculosis results most commonly through exposure of the lungs or mucous membranes to infected aerosols.[emedicine.com]
Prevention
- Prompt evaluation and treatment is necessary to prevent disfigurement and systemic involvement associated with DLE. The following case presented a diagnostic dilemma as the lesion mimicked lupus vulgaris.[ncbi.nlm.nih.gov]
- Today, when tuberculosis threatens to burst into pandemics again, early diagnosis and treatment are more important than ever for control and prevention of morbidity.[ncbi.nlm.nih.gov]
- Early diagnosis and treatment of lupus vulgaris might have prevented the development of carcinoma in lupo and ensuing metastatic death of the patient.[ncbi.nlm.nih.gov]
- Treatment of specific manifestations of SLE is aimed at prevention of complications.[medical-dictionary.thefreedictionary.com]
References
Article
- Rai VM, Shenoi SD, Gowrinath K. Tuberculous gluteal abscess coexisting with scrofuloderma and tubercular lymphadenitis. Dermatol Online J. 2005;11(3):14.
- Marcoval J, Servitje O, Moreno A, et al. Lupus vulgaris. Clinical, histopathologic, and bacteriologic study of 10 cases. J Am Acad Dermatol. 1992;26(3 Pt 2):404–407.
- Barbagallo J, Tager P, Ingleton R, Hirsch RJ, Weinberg JM. Cutaneous tuberculosis: diagnosis and treatment. Am J Clin Dermatol 2002;3(5):319-328.
- Farina MC, Gegundez MI, Pique E, et al. Cutaneous tuberculosis: a clinical, histopathologic, and bacteriologic study. J Am Acad Dermatol. 1995;33:433–440.
- Tan SH, Tan BH, Goh CL, et al. Detection of Mycobacterium tuberculosis DNA using polymerase chain reaction in cutaneous tuberculosis and tuberculids. Int J Dermatol. 1999;38:122–127.
- Andrew PW, Edward WJ. Cutaneous tuberculosis of the nose with unusual clinical and histological features leading to a delay in the diagnosis. Clin exp Dermatol. 1977;2:235-242.
- Khandpur S, Reddy BS. Lupus vulgaris: Unusual presentations over the face. J Eur Acad Dermatol Venereol. 2003;17:706-710.
- Sehgal V. Cutaneous tuberculosis. Dermatol Clin. 1994;12:645–53.
- Aliağaoğlu C, Atasoy M, Güleç AI. Lupus vulgaris: 30 years of experience from eastern Turkey. Clin Exp Dermatol. 2009;34:e289.
- İnönü H, Sezer E, Doruk S, Koseoglu D. Cutaneous tuberculosis: report of three lupus vulgaris cases: original image. Turkiye Klinikleri J Med Sci. 2009;29:788–91.