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Lupus Vulgaris
Vulgaris Lupus

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.

Images

WIKIDATA, Public Domain
WIKIDATA, Public Domain
WIKIDATA, Public Domain
WIKIDATA, Public Domain

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.

Entire Body System

  • Rigors and Chills

    Fever was evening rise in pattern with no rigors or chills and subsided after the antipyretic and there was no fever after five such episodes. There was no remarkable medical or surgical history. [cureus.com]

Eyes

  • Lagophthalmos

    There was no lagophthalmos or fixation to the underlying bone. The globe was intact, with good mobility, but the conjunctiva was very congested (Figure 1). Visual acuity was finger play at 6 meters. [paojournal.com]

Skin

  • Skin Disease

    Skin and Connective Tissue Diseases Skin Diseases Skin Diseases, Infectious Skin Diseases, Bacterial Tuberculosis, Cutaneous Diseases Bacterial Infections and Mycoses Infection Skin Diseases, Infectious Skin Diseases, Bacterial Tuberculosis, Cutaneous [hon.ch]

    Etymology[edit] The term "lupus" (meaning "wolf" in Latin) to describe an ulcerative skin disease dates to the late thirteenth century, though it was not until the mid-nineteenth that two specific skin diseases were classified as lupus erythematosus and [en.wikipedia.org]

  • Skin Ulcer

    Skin biopsy from the edge of ulcer was also suggestive of tuberculosis. Patient is doing well on antituberculous drugs. [lungindia.com]

    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]

  • 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]

    Cutaneous manifestations of secondary syphilis are protean and skin tuberculosis may be considered in the differential diagnosis, especially in HIV-infected patients. [moh-it.pure.elsevier.com]

  • Facial Skin Lesion

    [Crossref] Werschler WP, Elgart ML, Williams CM (1990) Progressive asymptomatic annular facial skin lesions. Cutaneous tuberculosis (lupus vulgaris). Arch Dermatol 126: 1227-1230. [oatext.com]

  • Skin Plaque

    Generally, lupus vulgaris present as one or two plaques with or without involvement of systemic organs. Special feature in our case was alongwith multiple skin lesions, her joints, bones, lungs and lymph nodes were also affected. [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]

    Some people with lupus have a red rash over their cheeks and the bridge of their nose -- called a "butterfly" or malar rash. Hair loss and mouth sores are also common. [webmd.com]

    Rashes: butterfly-shaped rash over the cheeks - referred to as malar rash red rash with raised round or oval patches - known as discoid rash rash on skin exposed to the sun Mouth sores: sores in the mouth or nose lasting from a few days to more than a [rheumatology.org]

    Systemic lupus erythematosus Share on Pinterest A malar rash is a key symptom of lupus. Image credit: Doktorinternet, 2013. SLE is the most familiar type of lupus. It is a systemic condition. This means it has an impact throughout the body. [medicalnewstoday.com]

    rash (malar discoid rash not counted), bullous lupus, toxic epidermal necrolysis variant of SLE, maculopapular lupus rash, photosensitive lupus rash (in the absence of dermatomyositis) or Subacute cutaneous lupus (nonindurated psoriaform and/or annular [merckmanuals.com]

  • Facial Ulceration

    This delay could have been averted if there was a high index of clinical suspicion especially with her unexplained and progressive ulcerated and nodular facial lesions. [ispub.com]

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

    In addition, men are more likely than women to have a positive tuberculin skin test result. The reason for these differences may be social, rather than biologic, in nature. [emedicine.com]

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]

Treatment

Though streptomycin is known to be of considerable value in the treatment of tuberculosis of various organs of the body, a conscientious review of the literature revealed only one report of its use alone in the treatment of lupus vulgaris. [jamanetwork.com]

We report this case to raise diagnostic awareness and treatment of atypical CTB with negative T-SPOT.TB. [dovepress.com]

The lesion healed with hypopigmented, atrophic scarring at the end of the 6 months of treatment. Figure 2A and Figure 2B. [escholarship.org]

Treatment is with standard antitubercular treatment. Read the full text or download the PDF: Log in using your username and password [casereports.bmj.com]

Prognosis

Deep 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]

It is the most common type of cutaneous tuberculosis.Lupus Vulgaris: Read more about Symptoms, Diagnosis, Treatment, Complications, Causes and Prognosis. [nsxmj.co]

Etiology

The etiology and pathology of many diseases have been cleared up and the way leading to the elucidation of many mooted questions pointed out. [jamanetwork.com]

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]

Etiology Etiology is still elusive but it is thought to be multifactorial, involving a genetic predisposition. [orpha.net]

Epidemiology

BACKGROUND: The epidemiological aspects and diagnostic problems encountered in a rural set up are largely unknown. [ncbi.nlm.nih.gov]

Leprosy and tuberculosis: The epidemiological consequences of cross-immunity. Am J Public Health 1997;87:1923-7. [Google Scholar] [ijdvl.com]

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]

Clinical and epidemiological observations of cutaneous tuberculosis in Larkana, Pakistan Int J Dermatol. 2002;41:159–65 5. Tappeiner GWolff K, Goldsmith LA, Katz SI, Gilchrist BA, Paller AS, Leffell DJ. [journals.lww.com]

Pathophysiology

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 to [wikidoc.org]

[…] develops 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 [wikizero.com]

Pathophysiology The clinical manifestations of cutaneous tuberculosis primarily depend on the mode of infection, previous sensitization, and the immune status of the host. The mode of infection may be exogenous, autoinoculation, or endogenous. [clinicaladvisor.com]

Pathophysiology Infection with M tuberculosis results most commonly through exposure of the lungs or mucous membranes to infected aerosols. [emedicine.com]

Prevention

Treatment of specific manifestations of SLE is aimed at prevention of complications. [medical-dictionary.thefreedictionary.com]

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]

Carbomer eye gel was applied to the right eye 4 times a day as a preventive measure against exposure keratitis. [paojournal.com]

References

  1. Rai VM, Shenoi SD, Gowrinath K. Tuberculous gluteal abscess coexisting with scrofuloderma and tubercular lymphadenitis. Dermatol Online J. 2005;11(3):14.
  2. 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.
  3. Barbagallo J, Tager P, Ingleton R, Hirsch RJ, Weinberg JM. Cutaneous tuberculosis: diagnosis and treatment. Am J Clin Dermatol 2002;3(5):319-328.
  4. Farina MC, Gegundez MI, Pique E, et al. Cutaneous tuberculosis: a clinical, histopathologic, and bacteriologic study. J Am Acad Dermatol. 1995;33:433–440.
  5. 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.
  6. 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.
  7. Khandpur S, Reddy BS. Lupus vulgaris: Unusual presentations over the face. J Eur Acad Dermatol Venereol. 2003;17:706-710.
  8. Sehgal V. Cutaneous tuberculosis. Dermatol Clin. 1994;12:645–53.
  9. Aliağaoğlu C, Atasoy M, Güleç AI. Lupus vulgaris: 30 years of experience from eastern Turkey. Clin Exp Dermatol. 2009;34:e289.
  10. 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.
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