Lupus Vulgaris: Causes, Symptoms & Treatment

Lupus vulgaris is one of about ten known types of so-called cutaneous tuberculosis, which, like pulmonary tuberculosis, is usually caused by Mycobacterium tuberculosis. As a rule, the infectious disease, which only rarely occurs in Central Europe, is a reinfection, because the skin normally represents an impenetrable barrier for the pathogen. Lupus vulgaris is usually manifested by persistent and chronic foci of inflammation in the nose, cheeks, and neck.

What is lupus vulgaris?

Lupus vulgaris is the name given to a particular form of skin tuberculosis. The causative agent is usually – as in other forms of tuberculosis – Mycobacterium tuberculosis. While the disease is rare in Central Europe, it is still relatively common in some developing countries due to less favorable hygienic conditions and less widespread medical care. Symptomatic of lupus vulgaris are skin lesions in the nose, cheeks and neck. Typically, small brownish papules form on the skin that are soft to the touch and sometimes develop into ulcers as the disease progresses. Unlike other forms of tuberculosis, lupus vulgaris is not notifiable due to its only mildly infectious course. As a rule, the disease is not caused by a primary infection with Mycobacterium tuberculosis, but it usually corresponds to a re- or secondary infection. In this case, the original infection with Mycobacterium tuberculosis may have occurred some time ago – up to several years – and may have triggered tuberculosis, or it may have taken an asymptomatic and thus unnoticed course.

Causes

Cutaneous tuberculosis lupus vulgaris is an infectious disease usually caused by the pathogenic germ Mycobacterium tuberculosis. However, the disease is rarely caused by a primary infection because the pathogenic bacteria cannot penetrate healthy skin and usually first enter the bronchi and lungs by droplet infection or may enter the digestive tract. The tubercle bacillus, as Mycobacterium tuberculosis is also called, can only cause lupus vulgaris if it is able to enter the connective tissue of the subcutis, for example, via the lymphatic or blood channels. Only in exceptional cases can the bacterium enter the skin directly via a weeping wound or similar ports of entry and trigger a primary infection there. The prerequisite here is that the potential skin entry ports come into direct contact with tubercle bacilli (smear infection).

Symptoms, complaints, and signs

Symptomatic of lupus vulgaris in the early stages are small, about pea-sized, brown blisters or nodules (papules) that initially feel soft and cause no pain or discomfort. Usually, the areas most affected are the nose, cheeks and neck. In some cases, the extremities, i.e. hands and feet, are also affected. Often, small scaly patches on the skin, which develop similar to warts, are symptomatic of the initial stage of the disease. Curiously, the general well-being of affected individuals is hardly impaired during the initial phase of lupus vulgaris, which can span a period of several years.

Diagnosis and course of the disease

In addition to the externally visible symptoms of the skin, the establishment of bacterial cultures plays an important role. The bacteria for culturing are isolated from biopsy material of the diseased tissue. In addition, differential diagnoses are recommended to clearly differentiate the suspected lupus vulgaris from other diseases with similar symptoms, especially tuberculosis cutis verrucosa and lupus erythematosus chronicus discoides. Attempts to detect the tubercle bacilli by light microscopy using common staining methods usually yield useless results. Histologically striking is the appearance of Langhans giant cells in the skin nodules. They result from the fusion of macrophages during the feeding process (phagocytosis). As the disease progresses, new nodules form at the edges of the inflammatory lesions, while those inside heal. Gradually, deep ulcers may develop, and in some cases where the nasal mucosa is affected, even the nasal cartilage is attacked, so that facial disfigurements must also be counted among the symptomatology of advanced lupus vulgaris.Deep tissue destruction of the hands and feet is also observed.

Complications

Usually, lupus vulgaris results in the formation of small blisters on the skin. The blisters themselves do not yet represent a complication or complaint and, in most cases, are not associated with pain. However, they can have a negative effect on the aesthetics of the patient, leading to reduced self-esteem or inferiority complexes. Furthermore, it is mainly the patient’s extremities that are affected by this complaint. The areas can also show warts, which can also affect the aesthetics. In many cases, no treatment is initiated by the patient if the symptoms do not lead to particular discomfort or do not hurt. However, if treatment is not initiated, this can lead to destruction of the tissue on the feet and hands. This may also result in pain. Usually, the treatment of this disease takes place with the help of medications and can limit the discomfort relatively well. However, the therapy can last for several months. Complications do not occur in most cases. Likewise, the life expectancy of the patient is also not reduced by this complaint.

When should you see a doctor?

Skin changes and abnormalities of the skin appearance are signs of the organism of existing irregularities. A visit to the doctor should take place if the changes persist for several weeks or are increasing. In particular, the formation of poplars in the area of the face should be examined and treated. If vesicles or nodules develop, a doctor should be consulted. If the affected skin areas are painful or open wounds develop, the affected person needs help. Sterile wound care is necessary to prevent further pathogens from entering the body through the skin. If adequate wound care cannot be provided, a physician should be consulted for help and support to prevent sepsis from developing. In case of dry skin, scales on the skin or itching, a visit to the doctor should be made. If the skin tightens or warts develop, these are further indications of the body that should be clarified. Medical treatment is necessary to initiate a regression of the symptoms. If, in addition to the facial area, the extremities also show peculiarities of the skin appearance, a doctor should be visited in time. If emotional or mental problems arise due to the optical changes, medical support is required. In the case of behavioral abnormalities, a social withdrawal or mood swings, a visit to the doctor is therefore necessary.

Treatment and therapy

Because lupus vulgaris is usually a postprimary type of tuberculosis, it can be assumed that the pathogen, Mycobacterium tuberculosis, is already present elsewhere in the body and can potentially become pathogenic again, even in the absence of appropriate symptoms. In addition, the chronic and very persistent course of lupus vulgaris suggests that local control of the tubercle bacilli is not sufficient for a sustainable therapy. Treatment with systemically acting tuberculostatics is recommended, which in the most favorable case leads to complete eradication of the pathogenic germs. A number of tuberculostatics are available which intervene in the metabolism of the tubercle bacilli at various points. As a rule, drug therapy begins as combination therapy with the simultaneous use of several drugs with different active ingredients in order to achieve the broadest possible effect. The initial therapy, which by default lasts about two months, is followed by another several months of therapy with a modified composition of the drugs.

Outlook and prognosis

Cutaneous tuberculosis is rare in Western industrialized countries. It is usually related to poor hygienic conditions. By far the most common site of the disease is the cheek region. The prognosis for complete healing is good. Strikingly, the disease lupus vulgaris affects more women than men. The treatment period can last for months. An early start of therapy usually has a positive effect on the duration. After two months, doctors usually change the medication. The symptoms disappear only if the tuberculostatics are taken consistently and systematically.If treatment is discontinued prematurely, a relapse is very likely. This is because the foci of infection are still present in the body. Many patients demonstrably refrain from consulting a doctor because they do not perceive any limitations. In many cases, however, this results in destruction of the tissue. Permanent pain is thus possible. After healing, continuous monitoring becomes necessary. This is due to the fact that sometimes tumors form in the affected areas. Skin tuberculosis in itself is not fatal. However, it should not go unmentioned that the psychological well-being suffers due to a protracted skin lesion.

Prevention

Directly preventive measures that could prevent infection with tubercle bacilli do not exist. The best indirectly effective preventive measures are to observe certain hygiene standards and to stay away from potential sources of infection, as well as to ensure that the immune system is strengthened. Until the 1990s, vaccination with live attenuated vaccine (BCG vaccination) was available to prevent tuberculosis infection. However, vaccination is currently no longer recommended by the Standing Commission on Vaccination (STIKO) due to poor efficacy and because of significant side effects.

Follow-up

For patients who have developed lupus vulgaris, careful follow-up is essential. Even after successful therapy, affected individuals are at high risk of relapse. Since the majority of recurrences occur within the first five years, follow-up examinations take place particularly regularly during this period. The intervals and extent of these examinations depend on the type and severity of the disease. For this reason, close medical check-ups (every three to six months) are recommended during the first five years. Follow-up care should be maintained throughout life. Some forms have a relatively high tendency to recur. Affected individuals should have regular dermatological examinations of the entire skin following surgery or alternative therapy. Conscientious follow-up care can detect pathological changes in the skin or secondarily affected organs in good time. Regular self-examination is also an important part of aftercare. Affected individuals should avoid strong sun exposure in the future and ensure adequate UV protection.

What you can do yourself

Lupus vulgaris must be medically clarified and treated in any case. Medical treatment can be supported by some self-help measures and home remedies. First of all, bed rest and rest and recuperation are recommended. Sufferers should ensure that the affected skin areas are not exposed to irritating substances such as lint, dust or sweat. It is best to clean the blisters and nodules regularly with lukewarm water and then disinfect them. In consultation with the doctor, natural remedies can also be used under certain circumstances – ointments made from lady’s mantle or bloodroot are recommended, for example. Arnica can also be applied in the form of an infusion, provided the skin is not inflamed. In addition, general measures such as a healthy and balanced lifestyle are recommended. Exercise and a suitable diet reduce stress and contribute to a speedy recovery. In the case of prolonged illnesses, therapeutic counseling is advisable. In a discussion with a specialist, the aesthetic and thus also psychological problems that lupus vulgaris brings with it can be worked through. If the complaints have not decreased after a few days, it is necessary to talk again with the responsible doctor.