Sarcoidosis or Besnier-Boeck disease-Schaumann (commonly referred to as BBS) or benign lymphogranulomatosis is a systemic inflammatory disease of unknown cause, which preferentially reaches the lungs, but can reach any other organs.
Usually mild, but it resolves spontaneously in 20.
Sarcoidosis affects men and women of all ages (usually between 30 and 50) and of all ethnic backgrounds.
In Caucasians, there is an equal frequency of occurrence in men and women. Black people (Africans and West Indians) is most commonly affected with a female predominance. Forms of sarcoidosis in these populations are also more serious.
Its incidence varies worldwide, with, for example, for Europe, a north-south gradient (640 to 100. In France, the prevalence is 10 to 100. A mutation in the gene encoding an immunoglobulin BTNL2 could intervene in the disease development.
The mechanism of the onset of the disease remains unclear. One hypothesis involves a cell-mediated immunity against an antigen, so far unknown, a genetic predisposition. One of the possible antigens is a mycobacterium, a mycobacterial catalase-peroxidase is found in half the cases.
The immunological mechanisms responsible for sarcoidosis are known. The chronology of sarcoid inflammation was particularly well studied in the lung. It is characterized by immunological disorders, explaining its impact on the body. There are three different phases that follow.
In 33. These signs correspond to uveitis, anterior and typically chronic. The examination at the slit lamp shows bilateral precipitates, adhesions (synechiae, iris nodules. Conjunctivitis is often associated.
Joint pain are common. They are due to arthritis (joint inflammation). An increase in blood calcium (hypercalcemia) is only present in 5.
Involvement of the heart is seen in 4 of 10 cases. It can be silent or present with malaise, signs of heart failure. Rarely, sudden death can occur. Echocardiography and cardiac MRI may show nonspecific abnormalities.
The liver damage is 20. This achievement is described in less than one in ten cases. A depressive syndrome may be a little less than half the cases.
One can observe renal, training of calculations in the urine or inflammation of the nose and sinuses.
In its typical form, Löfgren's syndrome involving erythema nodosum, lymphadenopathy and diffuse joint pain, enough to make a diagnosis without requiring other additional examinations.
The diagnosis is not obvious and may be delayed, especially in the lung only forms in which the delay may exceed six months.
The diagnosis requires the collection of elements by several orders. These tests can detect lesions early and thus ensure medical supervision. There are two main families of examinations. Löfgren's syndrome in particular, only heals in 90. For this, we must distinguish between two forms of the disease.
The most widely used treatment is corticosteroid therapy (corticosteroids), either locally (in the form inhaled corticosteroid, local anterior uveitis or posterior uveitis for injection) or systemically.
The absolute indications for oral corticosteroids are the locations causing life-threatening or major functional (heart, central nervous system, eye, muscle, persistent hypercalcemia) or significant skin sites (disfigurement). Forms only in lung, only symptomatic patients with significant functional and radiographic abnormalities should be treated immediately.
The corticoid treatment is extended, of at least 2. In some treatment, corticosteroids may be initiated simultaneously with methotrexate weekly by subcutaneous injection or tablet form.
The effectiveness of corticosteroid therapy is better if treatment is initiated before irreversible damage.
Other molecules have been tested.
Jonathan Hutchinson (1828-1913) is the first described sarcoidosis in 1877.
The French dermatologist Henri Ernest Besnier (1831-1909) in 1889 described a lesion of the skin of the extremities symmetrically.
The Swedish dermatologist Cæsar Peter Møller Boeck (1845-1917) in 1899 mentioned the histological lesions of the skin and then put already the suspicion of a systemic disease. That's why these lesions are called since then as sarcoidosis of Boeck.
The Danish ophthalmologist Heerfordt Frederick Christian (1871-1953) describes a feverish infection of the conjunctiva and because laboratory analyzes of the class as being due to mumps.
In 1924 Jörgen Nilsen Schaumann Swedish dermatologist (1879-1953) confirms the discovery of Boeck and that this is a systemic disease and multiple organ sarcoidosis dénomma ". They often work together and are given almost the same goals.
A primary goal is information of sarcoidosis patients and their families. They are usually very difficult to find information for the general public. Given the varied symptoms and the need for differential diagnosis and relatively minute knowledge of many doctors regarding this disease, they also must be informed in a more extensive. Another goal is the public awareness to the problems of patients, because of their diffuse symptoms, are placed in the category of ". Awareness of the political sphere, as well as health officials and social, also plays a role. Many aid organizations are active for sarcoidosis to promote research into this disease.
Porphyria Toxiderma