Tuberculosis cutis luposa A18.4

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Eating lichen; Mucous membrane tuberculosis ulcerous; Pineal lichen; Tuberculosis cutis luposa et mucosae; Tuberculosis luposa cutis et mucosae; vulgar lupus

History
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Robert Willan 1757-1812

Definition
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Altogether rare, but most frequent (endogenous, more rarely exogenous) post-primary form of skin tuberculosis with normal reaction of the organism, usually not very contagious.

There is an obligation to register by name!

In 50% of the cases an association with another, active organ tuberculosis is detectable (in most cases pulmonary tuberculosis).

Pathogen
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Mostly human, rarely bovine types of Mycobacterium tuberculosis.

Occurrence/Epidemiology
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Tuberculosis cutis luposa is (in contrast to atypical mycobacterioses) only very rarely observed in large Central European dermatology centres (1:100,000 dermatological patients). More frequent occurrence in Brazil (Mann D et al. 2019). w:m=2:1.

Etiopathogenesis
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The pathogens are eliminated haematogenically, lymphogenically or per continuitatem from an organ tuberculosis or also by exogenous inoculation. S.a. stage tuberculosis.

Manifestation
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mostly older people; w:m=2:1

Localization
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Mainly acral parts of the face (nose, cheeks, ears, lips), rarely extremities and mucous membranes

Clinical features
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  • Initially there is a reddish-brownish, 0.2-0.4 cm large, somewhat raised, diascopically apple-jelly-coloured n odule (lupus nodule), which is usually ignored. If not treated, peripheral spread results in a 1.0-5.0 cm large (or even larger) brown-red, symptom-free, only slightly consistency-propagated plaque with atrophic, parchment-like surface in the absence of follicle pattern (follicle destroyed by granuloma formation). Smaller satellite nodules are usually found in the marginal area, so that usually a broken marginal contour is formed instead of a smooth one
  • Positive probe phenomenon or mandrin phenomenon can be triggered (only in the case of cheese making!). The clinical course depends on the immunity status of the organism. Cave! Revival of tuberculosis in immunocompromised persons.
    Depending on the secondary change:vulgaris.
  • Mucosal changes: Grey-white or glassy-transparent, mostly raised, bumpy nodules. In case of ulceration formation of soft, slightly bleeding, serous-purulent covered ulcers, corking.

Histology
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  • The epidermis is atrophic, more rarely acanthotic. Erosions or ulcerations are possible. Nodular infiltrates of epithelial cells, giant cells of the Langhans type mixed with a large number of lymphocytes, which can condense into a mantle around the edges of the granulomas, are present, preferably localised in the upper dermis, but also extending to the deep dermis. A central necrosis (tendency to caseate) is possible, but is often not present in young granulomas. The skin appendages in the granulomas are destroyed.
  • Microscopic detection of tubercle bacilli with the common staining methods(Ziehl-Neelsen staining, Fite-staining) or by means of immunohistological methods is possible only very rarely.

Differential diagnosis
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Complication(s)
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General therapy
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The therapy of tuberculosis cutis luposa is based on the current respective recommendations of the CDC. The antituberculosis therapy has two goals:
  • Recurrence-free healing due to safe sterilisation
  • Prevention against selection of resistant strains.

External therapy
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In small lupus herds excision in healthy persons, followed by 6 months of tuberculostatic treatment. Plastic-surgical measures in case of mutilation after healing.

Internal therapy
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  • Since the bacterial populations react with different sensitivities to tuberculostatics, the therapy should be designed as a triple or quadruple therapy (see Table 1). Highly effective drugs such as isoniazid or rifampicin should be included in each combination.

Notice! The monotherapy of cutaneous tuberculosis with isoniazid is obsolete!

  • After the initial phase of 2 months with a quadruple therapy, treatment with two effective preparations is recommended for the subsequent stabilization phase. The detailed information on dosage, drug side effects and necessary control examinations must be observed (see in each case under the generic name). Important: The regular intake of the drugs should take place before breakfast. Rifampicin impairs the effect of anticonceptives through faster degradation.
  • Immunocompromised patients with tuberculosis cutis luposa are always treated with a quadruple therapy over 12 months.
  • Resistance situation: Several studies in Germany have revealed a resistance to isoniazid of 6-7% (USA 9.1%), RMP 1.5-2.4 (USA 3.9%) and EMB 1.0-1.9% (USA 2.4%). Resistance thus primarily concerns isoniazid. In these cases, antituberculostatically effective drugs that are less effective must be used.
  • The risk factors for the occurrence of resistant tuberculosis pathogens are:
    • Previous tuberculostatic treatment
    • Insufficient tablet intake
    • Stay in areas with high tuberculosis prevalence
    • Stay in areas with high resistance (over 4% compared to INH)
    • Contact with persons with resistant pathogens.
  • The following preparations are available on the market:
    • Isoniazid (e.g. Isozid Tbl., Tebesium-s-100/250 solution, Isozid Comp.)
    • Rifampicin (e.g. Eremfat Filmtbl., Rifa Drg.)
    • Pyrazinamide (e.g. Pyrafat Filmbl., PZA-Hefa Tbl.)
    • Streptomycin (e.g. Strepto-Fatol or Strepto Hefa injection solution)
    • Ethambutol (not for children under 10 years) (e.g. Myambutol Tbl., EMB-Fatol Filmtbl.).

Remember! The large number of Tbl. significantly reduces patient compliance!

The use of combination preparations simplifies the administration for the physician and the intake for the patient for the long period of the therapy, see Table 3.

Progression/forecast
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Chronic course; healing with atrophy and scars over the course of years/decades. Recurrences in lupus scars. Soft tissue destruction with subsequent mutilation. Restriction of joint movement. With appropriate chemotherapy, 95% of cases are curable within months.

Tables
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Therapeutic strategies for skin tuberculosis (as for other extrapulmonary organ tuberculoses)

Active substance

Dose per kg bw/day

Month 1

2

3

4

5

6

Additionally

Quadruple therapy

Isoniazid

5 mg

x

x

x

x

x

x

30 mg Vit. B6

Rifampicin

10 mg

x

x

x

x

x

x

Ethambutol

15-20 mg

x

x

Pyrazinamide

25 mg

x

x

Combination preparations for skin tuberculosis

Generic drugs

Example preparations

Dose in the sample preparation

Rifampicin + Isozianide

Rifinah

300/150 mg

Rifampicin + Isozianide

Iso-Eremfat 150

150/100 mg

Iso-Eremfat 300

300/150 mg

Rifampicin + Isozianide + Pyrazinamide

Rifater

120/50/300 mg

Ethambutol + Isoniazid

Myambutol-INH-I/-II

500/100 mg

300/100 mg

Ethambutol + Isoniazid

EMB-INH

300/60 mg

Note(s)
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The Quantiferon-TB-Gold test has established itself as the detection method. Its valence in skin tuberculosis has not yet been proven.

The detection of mycobacteria from lesional biopsy material is important. To cultivate the mycobacteria, laboratories should always use the faster, radiometric method in addition to conventional culture.

The PCR (polymerase chain reaction) is in no case a substitute for the cultural methods.

In industrialized countries, large-area, mutating forms with broad tissue loss (mutilations of nose, ears, septum perforations, wolf's tongue, etc.) are no longer observed. Such images still adorn older textbooks, but no longer correspond to current clinical reality (cave migrant situation!).

Literature
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  1. Diallo R et al (1997) Lupus vulgaris as the etiology of tuberculous mastitis. Pathologist 18: 67-70
  2. Ghorpade A (2003) Lupus vulgaris over a tattoo mark-inoculation tuberculosis. J Eur Acad Dermatol Venereol 17: 569-571
  3. Mann D et al (2019) Cutaneous tuberculosis in Rio de Janeiro, Brazil: description of a series of 75cases
    . Int J Dermatol doi: 10.1111/ijd.14617.
  4. Meyer FJ et al (1995) Renaissance of tuberculosis? Internist 36: 1162-1173
  5. Motta A et al (2003) Lupus vulgaris developing at the site of misdiagnosed scrofuloderma. J Eur Acad Dermatol Venereol 17: 313-315
  6. Pandhi D et al (2001) Lupus vulgaris mimicking lichen simplex chronicus. J Dermatol 28: 369-372
  7. Plum G et al (1995) The Epidemiology of Multidrug Resistant Tuberculosis in the United States of America. 36: 980–986
  8. Senol M et al (2003) A case of lupus vulgaris with unusual location. J Dermatol 30: 566-569

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020