What is it?
Also known as Besnier-Boeck-Schaumann disease, it is an inflammatory disease caused by the formation of granulomas in various organs and tissues (especially the lung, lymph nodes and skin).
The granulomatous reaction is similar to that against a foreign body, with cell accumulations around an area of necrosis. To date, however, no foreign or infectious agent has been identified within the granuloma.
Causes and risk factors
It usually affects young adults between the ages of 20 and 40, with a slight preference in women.
The cause of the disease is unknown, although the most authoritative hypothesis is an overreaction of the immune system.
Which are the symptoms?
The lung is the most frequently affected organ. Hence, the first symptoms may be: a cough, shortness of breath and sometimes pain.
The disease can also affect:
• skin, with pimple-like lesions (lupus pernio) or painful subcutaneous nodules (erythema nodosum);
• eyes, mainly with iridocyclitis and uveitis;
• joints with frank arthritis.
In addition to erythema nodosum and arthritis, the onset of the disease is often accompanied by a modest fever, asthenia and swelling of the lymph nodes.
Less frequently, almost all other internal organs are involved.
How is it diagnosed?
Diagnosis is based on medical examination, chest X-ray and CT scan, which may show different degrees of involvement of the lung parenchyma and / or the lymph nodes of the pulmonary ili.
Respiratory function tests and blood tests are also useful, which can show an increase in inflammatory indices, calcium levels and ACE (an enzyme that converts a circulating protein substance, called angiotensin I).
Biopsy of a skin, lymph node, or lung lesion is often necessary to be certain of the diagnosis.
The evolution of the disease is very variable: from spontaneous recovery in the most benign forms, to chronic and progressive involvement of various organs.
How is it treated?
Since the disease heals spontaneously in some patients, the onset is often limited to salicylates and other non-steroidal anti-inflammatory drugs.
In chronic forms, cortisone is used, possibly associated with immunosuppressants, such as methotrexate, azathioprine and cyclosporine. Also useful are the hydroxychloroquine and colchicine for skin lesions.
Where do we treat it?
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