Sarcoidosis (synonyms: Benye-Beck-Schaumann disease, benign sarcoidosis, Beck's disease) is a systemic disease of unknown etiology, affecting a wide variety of organs and tissues, the pathomorphological basis of which is epithelial cell granuloma without signs of caseous necrosis.
The disease sarcoidosis was first described by the Norwegian dermatologist Beck (1899).
What causes sarcoidosis?
The causes and pathogenesis of sarcoidosis are not clear. For several decades, the theory of the tuberculous origin of sarcoidosis prevailed, i.e., it was believed that sarcoidosis was a special form of tuberculosis infection. The role of genetic factors in the development of the disease is important, as indicated by the greater concordance of monozygotic twins for this disease compared to dizygotic twins, the unequal association of sarcoidosis with certain histocompatibility antigens (for example, HLA-B8, DR3) in populations, and racial differences in incidence.
The presence of familial cases, damage to mono- and heterozygous twins confirms a genetic predisposition to granulomatous inflammation. Some authors believe that an imbalance in the immune system plays an important role in the development of sarcoidosis.
Summarizing all views on the etiology and pathogenesis of sarcoidosis, we can come to the conclusion that it is a polyetiological syndrome.
Histopathology of sarcoidosis
In all forms of sarcoidosis, the same type of changes are observed. In the middle and deep parts of the dermis, granulomas are found, consisting of epithelioid histiocytes with an admixture of lymphocytes, single giant cells of the Langhans type or foreign bodies. In contrast to tuberculosis, casual cheesy necrosis is usually absent. At the resolution stage, the granulomatous islands are replaced by connective tissue.
Pathomorphology of sarcoidosis
Typically, there are numerous similarly constructed, sharply circumscribed granulomas, consisting predominantly of histiocytic elements. Necrosis is not typical. In the center of individual granulomas one can see giant cells of the Pirogov-Langhans type, and cells of foreign bodies are also found. In the cytoplasm of these cells, crystalloid inclusions and Schaumann asteroid bodies are often found, which, however, are not specific for sarcoidosis. The peripheral rim of lymphoid elements at this stage is small or completely absent. These granulomas are characterized by the presence of concentrically located collagen fibers around them, stained red with picrofuchsin and giving a weak PAS-positive reaction. Impregnation with silver nitrate using the Fugue method reveals reticulin fibers both around the granuloma and inside it. At the stage of fibrotic changes, granuloma cells are mixed with fibroblastic elements, reticulin fibers turn into collagen.
In Beck-Schaumann sarcoid, epithedioid islands are localized in upper third dermis, closer to the epidermis, with Daria-Roussi sarcoid - mainly in the subcutaneous fat layer. Lupus pernio differs from Beck-Schaumann sarcoid only in the presence of sharply dilated capillaries in the upper part of the dermis. In the erythrodermic form, the infiltrate consists of small foci of epithelioid cells and a certain number of histiocytes and lymphocytes located around the surface capillaries.
Sarcoidosis must be differentiated from tuberculous lupus, in which tubercles of an epithelioid structure are also observed. In the presence of caseosis and a significant number of lymphoid elements in the granulomas, it is very difficult to distinguish sarcoidosis from tuberculosis. However, in tuberculosis, the granulomatous infiltrate is closely adjacent to the epidermis, often destroying it, while in sarcoidosis the infiltrate is separated from the epidermis by a strip of unchanged collagen. In sarcoidosis, granulomas, as a rule, have very few lymphoid cells, there is no or very mild necrosis, the epidermis is of normal thickness or atrophic. with tuberculous lupus, acanthosis and sometimes ulceration with symptoms of pseudoepitheliomatous hyperplasia are often observed. Bacteriological examination is important. It is difficult to differentiate sarcoidosis from the tuberculoid type of leprosy, since Mycobacterium leprosy is detected only in 7% of cases. However, granulomas in leprosy are located mainly around and along the skin nerves. As a result, they have an irregular shape and necrosis is often visible in their center.
Histogenesis is not clear. Currently, sarcoidosis is considered as a polyetiological disease, mainly of immunopathological origin. There is a decrease in the number of T-lymphocytes, an imbalance in their main populations; decreased T cell response to mitogens; weakened delayed hypersensitivity reactions; an increase in the number and hyperactivation of B lymphocytes with nonspecific polyclonal hypergammaglobulinemia, increased levels of circulating antibodies, especially in the presence of erythema nodosum. At the stage of granuloma formation, T-helper cells dominate in them with a relative increase in the number of circulating T-suppressor cells.
In the development of sarcoid granuloma of the skin K.A. Makarova and N.A. Shapiro (1973) distinguishes three stages: hyperplastic, granulomatous and the stage of fibrous-hyalinous changes. In the hyperplastic stage, proliferation of cells of the mononuclear-macrophage system is observed, among which epithelioid elements then appear. Forming granulomas, as a rule, are still unclearly defined. multinucleated cells are usually absent at this stage of the process. The hyperplastic and granulomatous stages are believed to be an expression of increasing tension cellular immunity, and fibrous-hyalinous changes - morphological feature the onset of the phase of immunological exhaustion. Electron microscopic examination showed that round cells located on the periphery of the granuloma, considered lymphocytes, have lysosomes containing acid phosphletase and other lysosomal enzymes. They are blood monocytes, from which epithelioid cells are then formed. There is no evidence for the presence of bacterial fragments in epithelioid cells, although they contain electron-dense and electron-light lysosomes, several autophagic vacuoles, and a complex of residual bodies. Giant cells are formed from epithelioid cells, Schaumann bodies are formed from residual gels of lysosomes. Asteroid bodies consist of collagen clumps with a typical (64 to 70 nm) periodicity. This is due to the fact that collagen ends up between epithelioid cells at the time of the formation of giant cells from them. Immunomorphological studies in some cases show the deposition of IgM in the area of the dermal-epidermal border and in the walls of blood vessels, as well as IgG in the granulomas themselves and in the surrounding dermis.
Symptoms of sarcoidosis
Skin lesions are observed in less than 50% of patients, it can be polymorphic (such as erythema nodosum, macular erythematous foci), but more often there are tubercular elements of various sizes, the originality of which forms the basis for the identification of such clinical variants as cutaneous Beck's sarcoid, Broca's angiolupoid - Potrie, lupus pernio of Besnier-Tennesson, subcutaneous sarcoids of Darier-Roussy. Beck's sarcoid can manifest itself in the form of small-tubercular, including lichenoid, large-nodular and diffuse plaque rashes. In rare cases, the process can occupy the entire skin (erythrodermic form of sarcoidosis). The color of the tubercles is characteristic: bluish, yellowish-brown, with diascopy yellowish-brown spots appear. In Besnier-Tennesson lupus pernio, changes are observed mainly on the skin of the nose and adjacent areas of the cheeks in the form of diffuse plaque-like foci of bluish-red color; with subcutaneous Darrieus-Ruesi sarcoid, hypodermal nodes are detected, the skin over which becomes pinkish-bluish. There are rare (atypical) variants of sarcoidosis: erythematous (spotty), erythrodermic, lichenoid (clinically similar to lichen planus), pruriginous, verrucous-papillomatous, ring-shaped, figured (circinate), erosive-ulcerative. ulcerative gangrenous, papulonecritical, scleroderma-like, post-traumatic (scar), elephantiatic, clinically similar to the tuberculoid form of leprosy, lupoid necrobiosis, erythematous-squamous (ichthyosis- and psoriasis-like), atrophic, angiomatous, etc., which may resemble a wide variety of dermatoses, in including such peculiar ones clinical manifestations like eczema.
Sarcoidosis is more common in women and is characterized by greater clinical polymorphism. Skin rashes may be nonspecific or specific. The latter are detected during histological examination of the affected skin.
Depending on the manifestation of dermatosis, there are typical (small nodular, large nodular, diffuse infiltrative, nodular sarcoid, Besnier-Thenesson lupus pernio) and atypical forms sarcoidosis.
The small-nodular form is the most common and is characterized by a rash of numerous pink-red spots, which over time turn into nodules of a dense elastic consistency of a brownish-bluish color, ranging in size from a pinhead to a pea, hemispherical in shape, with clear boundaries and a smooth surface, rising above the surrounding skin. Elements are often localized on the face and upper limbs. Diascopy reveals tiny yellowish-brownish specks (“specks of dust,” or the phenomenon of dustiness). With regression, hyperpigmentation or superficial atrophy and telangiectasia remain in place of the nodules.
The large-nodular form of sarcoidosis is manifested by single or multiple, sharply demarcated and clearly protruding hemispherical flat nodes of a violet-brown or bluish-brown color, the size of a 10- to 20-kopeck coin or more. The elements have a dense consistency, a smooth surface, and are sometimes covered with telangiectasia; large nodes can ulcerate. During diascopy, the phenomenon of dustiness is observed.
Diffuse-infiltrative sarcoid is most often localized on the face, rarely on the neck, scalp and is manifested by the development of plaques of dense consistency, brownish or brownish-blue in color, with a smooth surface, not sharply demarcated and slightly protruding above the skin level. The surface may be covered with a network of capillaries. Diascopy reveals a yellowish-brown color (the “speck of dust” symptom).
Broca-Pautrier angiolupoid usually develops on the nose, cheeks and, extremely rarely, on other parts of the body. At the beginning of the disease, round, painless spots the size of a bean or a coin, of medium size, red or violet-red, appear. Gradually they transform into slightly protruding round plaques with clear boundaries, acquiring a brown or rusty tint, with a smooth surface. Rarely, by palpation you can feel a slight thickening of the spot, and with diascopy you can see brownish dots (symptom of “apple jelly”). Sometimes telangiectasias are visible on the surface of the elements. Plaques persist for a long time.
Besnier-Tenneson's lupus pernio. Small erythematous infiltrated lesions appear on the skin of the cheeks, nose, chin, dorsum of fingers and forehead. As a result of the growth and fusion of elements, violet-red nodes or plaques are formed, which have fairly clear boundaries. Enlarged holes are observed sebaceous glands and hair follicles. The process worsens during the cold season. Some patients have lesions of the lungs, bones, joints, and enlarged lymph nodes. Diascopy reveals yellowish dots.
Nodular sarcoid (Derrieux-Roussy subcutaneous sarcoid) is clinically characterized by the formation of subcutaneous nodes ranging in size from 1 to 3 cm in diameter on the skin of the trunk, thighs and lower abdomen. They are usually few in number, painless, mobile on palpation and, when fused, form large plaque infiltrates, resembling the peel of an orange. The skin over the nodes has a normal or slightly bluish color, covering the lesions is dull pink.
Atypical forms of sarcoidosis. IN clinical practice There are several atypical forms: ulcerative, maculopapular, psoriasiform, ichthyosiform, scleroderma-like, scofulodermic, paillomatous, angiomatous, similar to basal cell carcinoma, lupus erythematosus, etc.
Patients with sarcoidosis experience damage to various organs and systems. In this regard, patients are recommended to undergo radiography and tomography of the organs. chest, bones and conduct an ophthalmological examination.
The skin is affected in 25% of patients with sarcoidosis. Typically, this symptom accompanies systemic manifestations of the pathology, but in some cases, skin signs are the only manifestation of the disease. With sarcoidosis, special non-disintegrating nodules are formed in the tissues - granulomas, which can occur in any area, including on the skin.
The first case of the disease was described back in 1869, but its causes are still unknown. Other signs of pathology are the formation of granulomas in 90-95% of patients, which determines the prognosis of the disease. Also affected are the lymph nodes (especially intrathoracic), eyes, liver, heart, nervous system, musculoskeletal system, nervous and endocrine systems. In some cases, sarcoidosis resolves spontaneously.
Causes of the disease
The exact cause of the disease remains unclear. However, there are some risk factors - female gender, Negroid race, as well as a significant influence of hereditary predisposition.
The pathology very rarely affects children, and the first symptoms usually appear between the ages of 20 and 40.
What is cutaneous sarcoidosis?
It's chronically current inflammatory disease, affecting primarily the skin and lungs, causing a variety of skin symptoms. It's not contagious. As a result of the disease, either scars form on the skin, or all manifestations spontaneously disappear, but it is impossible to predict the outcome in advance.
External manifestations
The classification of skin signs includes nonspecific and specific. Specific lesions are characterized by the formation of nodules - granulomas, while histological examination shows no nonspecific changes in granulomas.
The main nonspecific skin lesion in sarcoidosis is erythema nodosum. Specific forms include:
- lupus pernio;
- maculopapular;
- nodal;
- scar;
- flat;
- angiolupoid;
- ichthyazoform;
- lichenoid;
- warty;
- psoriasis-like;
- ulcerative;
- subcutaneous nodules.
Cutaneous sarcoidosis in children can be hereditary or occur sporadically. The disease is caused by a mutation in the NOD2 gene and is associated with autoimmune processes. This pathology appears in early age. Granulomas affect the skin tissue, joints and uveal tract of the eye.
Erythema nodosum
It is an increased skin reaction in response to the action of various infectious agents (especially streptococci), drugs (including oral contraceptives) and in systemic diseases, in particular sarcoidosis. It usually occurs suddenly and disappears spontaneously, but is prone to recurrence.
Reddened, painful nodes are most often located on the front surface of the lower leg. They may be accompanied by fever, joint pain and feeling unwell. Young women are usually affected.
Erythema nodosum often appears in cases of acute development of pathology. It is a marker of a favorable prognosis, that is, a high probability of spontaneous recovery. In this case, treatment with hormones is not prescribed.
It is contrasted with specific sarcoid lesions, since it can be observed in many other diseases, primarily tuberculosis, yersiniosis, Crohn's and Behçet's diseases, streptococcal infections, and pregnancy. It can be caused by taking sulfonamides and oral contraceptives. With sarcoidosis, this form is combined with damage to the intrathoracic lymph nodes, fever, pain in various joints, and the appearance of signs of inflammation in blood tests. In this case, they talk about Löfgren's syndrome, an acute variant of sarcoidosis.
Despite the nonspecific nature of the lesion, erythema nodosum is most often observed in sarcoidosis, and is its most common skin manifestation. If such a lesion appears, an X-ray or CT scan of the chest should be performed. A biopsy in this case is not prescribed.
Lupus pernio
The most indicative, but also one of the rarest, skin lesions in sarcoidosis. It is characterized by the appearance of red, purple or violet dense plaques or nodules. This is how sarcoidosis of the facial skin usually manifests itself, when the nose, cheeks, ears and lips. However, elements of the rash can appear on the arms, fingers and toes, less often on the forehead, and in rare cases on the walls of the nasopharynx and larynx, making breathing difficult.
Such a lesion is more often observed in people with long-standing pulmonary sarcoidosis, and is often accompanied by uveitis, enlargement of internal organs, sarcoidosis of the upper respiratory tract and the formation of bone cysts (cavities). The course is usually chronic and difficult to treat. As a result of the process, a permanent cosmetic defect may occur, including facial disfigurement.
Lupus pernio is a sign of an unfavorable course of the disease with rapid damage to internal organs.
A common form of sarcoidosis of the scalp. Defeat does not cause complaints. Outwardly, it resembles red-brown spots located on the face, around the eyes, and in the nasolabial folds. The extensor surfaces of the joints may also be affected.
After the spots disappear, scars often form. The lesion is most characteristic of acute sarcoidosis.
Symptoms of the flat form of cutaneous sarcoidosis include the following:
- red-brown round or oval plaques;
- elements of the rash penetrate the skin, their center may be atrophied;
- sometimes the plaques peel off, in which case they can be mistaken for manifestations of psoriasis or;
- limbs, face, scalp, back, buttocks are affected;
- symmetrical areas of the skin suffer;
- sometimes the plaques become ring-shaped.
The angiolupoid form manifests itself very similarly, but on the surface of the plaque there are additionally dilated vessels - telangiectasia. This is an extremely rare form.
The process of involvement of the skin is chronic, which can result in scarring. When the scalp is affected, areas of baldness occur. With this form of pathology, as a rule, there are systemic lesions, primarily of the lungs.
Papular and plaque variants - signs chronic course disease or its exacerbation, and they also occur when new organs are involved, for example, the heart or joints.
Subcutaneous nodules
Subcutaneous nodular sarcoidosis is also called Darnier-Roussy disease. The lesions are usually painless, oval, fleshy or purple. The nodules are 0.5-2 cm in diameter and are found on the limbs and trunk.
They usually occur at the onset of the disease, in the absence or mild severity of systemic lesions, as well as during exacerbation of the disease. In some patients, the nodules resolve spontaneously.
Other lesions
Cutaneous sarcoidosis is called the “great pretender” because it can manifest itself with almost any rash element. It may resemble lichenoid lesions, vasculitis, psoriasis, warts and skin ulcers.
Areas of the skin surface with scars and scars previously received as a result of injuries, operations or, for example, tattoos may be affected. Their infiltration and compaction occur, as well as color change. This sign is quite specific. For example, changing any previous tattoo color to red or purple is one of diagnostic signs. So-called revived scars are a sign of exacerbation of the disease.
Sometimes the tattoo itself provokes a granulomatous reaction and infiltration with sarcoid formations. In this case, systemic manifestations of the disease do not occur.
The ichthyasiform variant is often diagnosed accidentally. It is always combined with damage to the lungs, eyes and other organs.
In the ulcerative type, either the formation of a skin ulcer occurs as a primary sarcoid element on intact skin, or the formation of granulomas in existing skin ulcers of a different nature, for example, with varicose veins.
Sarcoidosis (Besnier-Beck-Schaumann disease)
Diagnosis and differential diagnosis
List of diseases that need to be distinguished from cutaneous sarcoidosis:
- addiction;
- granuloma annulare;
- ichthyosis;
- leprosy;
- lichen planus;
- discoid lupus erythematosus;
- lymphocytoma;
- necrobiosis lipoidica;
- syphilis;
- B-cell lymphoma (mycosis fungoides).
It is used for primary diagnosis. The final diagnosis is made using laboratory research and biopsies.
Diagnosis of cutaneous sarcoidosis involves two main laboratory tests.
The Kveim test is the most accurate diagnostic method. It is used quite rarely due to the difficulty of obtaining a reliable source of antigen, as well as the danger of transmitting various infections. The Kveim test involves intradermal injection of tissue from the spleen or lymph node of a patient with sarcoidosis. 4-6 weeks after this, a biopsy is taken from the injection area. If specific granulomas are found in the tissues, this confirms the diagnosis.
About 70% of patients with sarcoidosis have positive tuberculin tests. This is also taken into account when making a diagnosis.
For diagnosis, biopsy of lesions is most often used. Specimens should not be taken from lesions of erythema nodosum because it is a nonspecific lesion. The collected material is sent to histological examination. First of all, it is necessary to exclude infectious cause formation of granulomas (tuberculosis), as well as deep mycoses and tumors. If a fungal or tuberculous infection is suspected, a tissue culture can be obtained. This is a lengthy and expensive study, but it allows you to exclude other similar diseases.
Typical sarcoid lesions are characterized as noncaseating “naked” granulomas. There are no foci of necrosis around them, as with tuberculosis. There is only a thin strip of lymphocytic infiltrate. Granulomas are usually circumscribed and composed of epithelial cells, with minor necrosis possible. If there is scarring, then fibrous tissue spreads from the periphery to the center of the lesion. Granulomas are located in the superficial layer of the dermis, but they can penetrate into the subcutaneous tissue.
Islands of epithelial cells in a granuloma may not contain a large number of Langhans giant cells with inclusions of Schaumann bodies or star-shaped formations. These findings help differentiate sarcoidosis histologically from other skin diseases.
Treatment options
Although cutaneous sarcoidosis is not life-threatening and causes physical limitations in only a small proportion of patients, its psychological and social consequences are severe. Therefore, treatment is indicated for those patients who have cosmetically disfiguring lesions, ulcers, and also with a progressively worsening course of the disease.
Choice therapeutic tactics complicated by three factors:
- the possibility of spontaneous healing, which occurs in 65% of patients;
- different individual response to therapy;
- serious side effects of the medications used.
Therefore, with a stable course of the disease without cosmetic defects, treatment is not carried out, limited only to observation.
Which doctor treats cutaneous sarcoidosis?
The patient should consult a dermatologist. If necessary, a consultation with a pulmonologist, ophthalmologist, rheumatologist, or infectious disease specialist is scheduled.
Are used different ways treatment - medications, minimally invasive and surgical techniques.
Drug therapy
Drug treatment of cutaneous sarcoidosis is determined by the severity of damage to other organs. In patients with lung damage or hypercalcemia, as well as pathologies of the eyes, nervous system or heart, glucocorticoids are used for oral administration (Prednisolone tablets). In case of acute damage to multiple organs, immunosuppressants may be required.
If only the skin is involved, anti-inflammatory drugs (nonsteroidal or low-dose Prednisolone) are usually prescribed to relieve symptoms.
Glucocorticoids can also be injected directly into the plaques themselves. Methotrexate and antimalarials (Chloroquine) are also used.
In patients with persistent disease and a tendency to disfiguring cosmetic defects It is proposed to use biological drugs, in particular, anti-tumor necrosis factor alpha therapy.
Drugs that have shown effectiveness in some studies:
- Cyclosporine;
- Isotretinoin;
- Allopurinol;
- Tetracycline and Doxycycline;
- Psoralen in combination with ultraviolet irradiation;
- Infliximab, Adalimumab and Etanercept;
- Leflunomide;
- Melatonin;
- Pentoxifylline.
For sarcoidosis, external therapy is also used - various anti-inflammatory creams and ointments containing glucocorticoids. These include: Comfoderm K, Hydrocortisone, Prednisolone, Laticort, Lokoid, Triacort, Fluorocort, Afloderm and others.
It must be remembered that the manifestations of sarcoidosis in many cases disappear spontaneously. The choice of medication must be entrusted to the doctor, since all of the listed drugs have different activities and side effects.
To speed up the healing of rash elements and prevent the formation of scars, regenerating agents are prescribed. For skin sarcoidosis, Stellanin ointment promotes healing and prevents secondary infection of rash elements.
Other methods
How to treat skin sarcoidosis with insufficient effectiveness medications? For this purpose they use surgical methods. For small or significantly disfiguring lesions, you can try to remove them using a scalpel or laser radiation. However, there is a risk of scar formation and relapse of the pathology.
Treatment of cutaneous sarcoidosis with folk remedies is ineffective. It can only be used in addition to drug therapy. Here are some traditional medicine recipes:
- compresses made from chopped onions mixed with vegetable oil;
- baths with infusion of string, sage, chamomile;
- ingestion of propolis tincture, rosea radiola and other immunostimulating agents.
Observation
Patients with mild skin involvement are seen twice a year for the first 2 years, with increased frequency of visits if the lungs are involved. All patients are on dispensary observation see a dermatologist for at least 3 years after completion of treatment.
When visiting a doctor, an X-ray of the lungs is performed, respiratory function is assessed and an ECG is performed. Additionally, a blood test for calcium and vitamin D levels may be prescribed.
If a patient receives Plaquenil, he needs regular examination by an ophthalmologist.
Patients with cutaneous sarcoidosis have an increased risk of non-Hodgkin's lymphoma and leukemia, so regular monitoring of blood tests and skin changes is necessary.
Cutaneous sarcoidosis is a systemic disease of unknown etiology, morphologically characterized by an epithelioid cell granulomatous structure without caseous necrosis. It is considered as a reactive pathology, the leading role in the development of which is played by the unusual response of the immune system to a number of different factors (infections, including tuberculosis, allergens, chemical irritants, endogenous antigens, etc.).
The presence of cases of sarcoidosis in twins indicates a genetic predisposition to this disease. In these patients, as a consequence of the disease, a decrease in the content of T-lymphocytes in the blood with a predominance of suppressors, and an increase in B-lymphocytes are observed.
In sarcoidosis, skin lesions characterized by clinical polymorphism are observed in 10-30% of cases. They can be specific and nonspecific, combined with damage to internal organs or be isolated.
A nonspecific rash (erythema nodosum) is often combined with damage to internal organs, while a specific rash is observed only in 6-10% of patients with sarcoidosis. There are typical (classical) and atypical forms of cutaneous sarcoidosis. Typical ones include:
- Beck's sarcoidosis,
- Besnier-Tenneson lupus,
- Broca-Pautrier angiolupoid,
- subcutaneous Darrieus-Roussy sarcoidosis.
There are three types sarcoidosisBeka: finely nodular, in the form of large nodes and diffuse-infiltrative.
Finely knottedsarcoid happens more often than other varieties. Multiple, symmetrical, pink-red spots, gradually transform into nodules, appear in groups, in separate outbreaks after a few days, clearly demarcated, ranging in size from 1-2 mm to 0.5 cm, hemispherical, with a smooth surface, pale pink or brick-colored. Red. On the surface of large elements there may be telangiectasia and peeling. Diascopy reveals yellowish spots (dusty phenomenon). The elements may be lichenoid and resemble lichen planus. Nodules are located isolated or in groups on any area of the skin, but more often perioral and periorbital. They may not change for months and years, but then an exacerbation occurs with the appearance of new nodules. In the regressing stage, the nodules become flat, sink, the number of telangiectasias and scales increases, the color changes to yellow-brown, brown.
Sarcoidin the form of large nodes localized mainly on the forehead, nose, cheeks, neck, and upper extremities. Nodes the size of a pea to walnut clearly demarcated, protruding above the skin level, hemispherical, less often flat, dense, with a smooth surface, telangiectasia and milia-like elements, rarely with peeling. They are red, dark cherry, subsequently yellow-brown, purple, brown, a positive phenomenon of dustiness, do not cause subjective sensations and very rarely disintegrate, are few in number, have a persistent course and are resistant to therapy. Regression begins from the center with the development of retraction; an infiltrated ridge is observed along the periphery. Subsequently, hyper- or depigmentation, telangiectasia, and cicatricial atrophy remain at the site of the node.
Diffuse- infiltrative sarcoid often combined with the forms described above. Large, ill-defined, violet-red, violet-pink, bluish-brown infiltrated plaques with a smooth surface appear, most often on the face, but can also appear on the scalp and neck. They increase, can merge until the development of erythroderma, do not entail subjective sensations, last for months and years, and in place of the reverse development of plaques, hyperpigmentation, telangiectasia, and cicatricial atrophy remain.
LupusBeignet-Tenneson develops more often on the face (cheeks, nose, “butterfly” localization), dorsal surfaces of the hands and fingers with the appearance of small erythemal infiltrated elements with a smooth surface, prone to peripheral growth and the formation of large foci of irregular outline with blurred contours, increased infiltration, discoloration bluish-purple, purple, brown. In developed cases, the lesions are violet-brown, in the form of plaques, nodes with a smooth surface, telangiectasia, and dilated follicles. Sometimes a positive phenomenon of dustiness is observed, less often - slight itching and soreness. The fingers can take on a spindle-shaped shape, and disintegration is possible in the center of the lesions with the formation of significant defects with granulations and purulent-bloody discharge. In half of the patients, synovial and bone cysts of the fingers are formed; when localized on the scalp, cicatricial atrophy and alopecia may develop. At the same time, patients may experience lesions of the nasopharynx, larynx, and nasal bones.
AngiolupoidBroca-Pautrier localized in the nasal area in the form of a single plaque. At first it is a round bluish-pink spot with telangiectasias on a smooth surface, which over the course of weeks turns into a round or oval plaque with clear contours, a soft consistency of violet-red color, which then becomes yellow-brown, brown, with many telangiectasias on the surface. The diameter of the plaque reaches 1-2 cm, the dustiness phenomenon is positive, and slight itching or soreness is subjectively noted. In this state, the plaque exists for months, less often - years.
Hypodermalsarcoid Daria-Rousset rarely occurs in the form of single or multiple subcutaneous dense mobile nodes measuring 1-3 cm, the skin over which is unchanged or bluish. Subsequently, it connects with the nodes and resembles the skin of an orange; the nodes can merge, but do not disintegrate. Typical localization is thighs, lower abdomen. On palpation, slight pain is sometimes observed. The course is long, perennial.
Atypicalforms of sarcoidosis:
- psoriasis-like,
- ichthyosis-like,
- scleroderma-like,
- warty,
- ulcerative,
- spotted,
- maculopapular sarcoid.
Differential diagnosis with sarcoma, basal cell carcinoma, warts, lupus erythematosus, seborrheic eczema is required; The issues are resolved by histological examination. Possible isolated or combined (with skin damage) damage to the mucous membranes.
Nonspecific skin lesions in sarcoidosis occur in 1/3 of patients in the form of erythema nodosum, more often in women, and indicate an acute variant of sarcoidosis, which is accompanied by:
- fever,
- malaise,
- arthralgia,
- uveitis,
- acceleration of ESR,
- bilateral hilar lymphadenopathy (Löfgren's syndrome).
It proceeds benignly, in 80% of cases recovery occurs without treatment.
How to treat cutaneous sarcoidosis?
Main in treatmentcutaneous sarcoidosis is long-term, up to six months or more, use of glucocorticosteroids, taking into account organ damage. The initial dose of prednisolone is usually 30-40 mg per day with a gradual reduction to a maintenance dose.
A combination of corticosteroids with cytostatics, including methotrexate, azathioprine, and prospidin, is advisable. Antimalarial drugs are used - delagil, plaquenil. In cases of limited lesions, intralesional administration of corticosteroids and the use of corticosteroid ointments at the site of occlusion are sufficient, which, however, lasts for several months.
It is noted that after a long course of the process, spontaneous recovery is observed in two thirds of patients with sarcoidosis, especially in cases of acute sarcoidosis in women. The chronic, protracted course of the process requires active therapy.
What diseases can it be associated with?
Sarcoidosis of the skin is primarily an indication for examining the patient for the presence of of this disease pulmonary or bone localization. lungs or sarcoidosis of bones in their etiology contain many of the same factors as sarcoidosis of the skin; they often turn out to be idiopathic, and sometimes hormonal or genetically determined.
A disease mistaken for cutaneous sarcoidosis may actually be lymphoma, mastocytosis, xanthomatosis, or granuloma annulare, since their symptoms are similar. The presence of corresponding manifestations requires laboratory diagnostics within a professional approach.
Treatment of skin sarcoidosis at home
Treatment of cutaneous sarcoidosis often resolves at home, but there is still the need to consult with your doctor. Often the disease is not local, but systemic in nature and affects, for example, the lungs as well. Treatment at home requires compliance with all medical instructions. It will not be superfluous to adhere to a certain regime, maintain healthy image life. For example, there are dietary recommendations:
- it is necessary to limit consumption
- refined sugar,
- flour,
- cheese,
- milk and dairy products,
- table salt,
- alcohol;
- include in the menu:
- honey,
- nuts,
- sea buckthorn and black currant,
- seaweed,
- grenades,
- beans,
- basil.
What drugs are used to treat cutaneous sarcoidosis?
Recovery is possible without using medicines, however, if the attending physician considers it necessary to prescribe therapy, then the choice falls on glucocorticosteroids, the determination of the names and dosages of which requires an individual approach in this case. They are sometimes combined with cytostatics, including methotrexate, azathioprine, and prospidine.
Treatment of skin sarcoidosis with traditional methods
Treatment of cutaneous sarcoidosis can occur through folk remedies, but this does not guarantee a sufficient effect. It is better to discuss the use of any of the following recipes with your doctor, and if there is no result or worsening symptoms, stop using it altogether:
- propolis tincture - combine 50 ml of 70-degree alcohol with 10 grams of crushed propolis, place in a glass container, seal and leave in a dark place for 2 weeks, shake periodically; Treat the resulting skin defects with the resulting tincture;
- lilac tincture - place 1/3 cup of freshly picked lilac flowers in glass jar, pour 200 ml of high-quality vodka, mix thoroughly, close, leave for 7-8 days, shake periodically; after the allotted period, strain the infusion and use it for rubbing into the affected areas of the skin, as well as into the lung area, if the appropriate diagnosis has been established;
- decoction of herbs - combine an equal amount of oregano, plantain, knotweed, sage, calendula flowers, marshmallow root; 1 tbsp. collection, place a thermos, pour a glass of boiling water, leave for an hour, strain; use for compresses and applications.
Treatment of cutaneous sarcoidosis during pregnancy
Sarcoidosis of the skin is not a contraindication to pregnancy; moreover, it is noted that due to hormonal changes in pregnant women, the disease can weaken and go into remission. By the way, pulmonary sarcoidosis is also not an obstacle to pregnancy and the birth of a healthy child. However, if the latter causes the risks of developing pulmonary fibrosis and hypoxemia with further occurrence pulmonary heart and hypertension, then the localization of sarcoidosis is exclusively on skin is not fraught with this, which means that skin sarcoidosis is not at all an indication for termination of pregnancy.
It should be noted that pregnancy planning for a woman with skin sarcoidosis should include chest x-rays and laboratory tests ( general analysis blood, liver enzymes, creatinine, serum calcium, urine nitrogen), pulmonary function testing to exclude the possibility of localization of the disease in internal organs. If a pregnant woman is prescribed medications for treatment of skin sarcoidosis(as determined by the attending physician), then the choice will fall on those that do not provide side effect for the fruit.
Caution should be exercised when consuming vitamin D, which partially provokes the development of hypercalcemia in women with sarcoidosis.
Treatment and treatment monitoring is carried out by a dermatologist, obstetrician-gynecologist and, if necessary, a pulmonologist.
Which doctors should you contact if you have cutaneous sarcoidosis?
Diagnosis of cutaneous sarcoidosis is based on clinical and histological symptoms and biopsy of peripheral lymph nodes.
Laboratory indicators for cutaneous sarcoidosis are as follows:
- increase in ACE level,
- hypercalciuria,
- increased levels of alkaline phosphatase and hydroxyproline,
- leukopenia,
- hyperchromic microcytic anemia,
- eosinophilia,
- hypergammaglobulinemia,
- acceleration of ESR.
Granuloma without caseous decay, consists of epithelioid cells, among which Langhans giant cells and foreign body cells are observed; around the granuloma - lymphocytes, macrophages, fibroblasts, in its center - sometimes fibrinoid necrosis, and in the cytoplasm of giant cells - Schaumann bodies (inclusion), asteroid and residual bodies.
Differential diagnosis with lymphomas, Kaposi's sarcoma, mastocytosis, xanthomatosis, systemic lupus erythematosus, granuloma annulare is advisable.
Treatment of other diseases starting with the letter - s
| Treatment of salmonellosis |
| Treatment of pulmonary sarcoidosis |
| Treatment of Kaposi's sarcoma |
| Treatment of uterine sarcoma |
| Treatment of Ewing's sarcoma |
| Treatment of type 2 diabetes mellitus |
| Treatment of type 1 diabetes mellitus |
| Treatment of seborrhea |
| Treatment of sepsis |
Two clinical cases of cutaneous sarcoidosis in combination with intrathoracic lesions are presented. It has been shown that the use of antimalarial drugs, glucocorticosteroids and methotrexate is effective in such patients, which is reflected in the positive dynamics of changes in the skin, intrathoracic lymph nodes and lungs. It has been noted that the dynamics of skin lesions in sarcoidosis often corresponds to the dynamics of intrathoracic changes and serves as an indicator of the effectiveness of therapy.
Sarcoidosis, an epithelioid cell noncaseating granulomatosis of unknown origin, remains the subject of discussion among specialists in various fields of medicine. In Russia, where this disease has become the object of attention of pulmonologists, internists and general practitioners, the diagnosis and treatment of sarcoidosis is very relevant. In the Republic of Tatarstan, the provision of care to patients with sarcoidosis is regulated by an order of the republican ministry in 2010, according to which the diagnostic process is optimized with the participation of the republican clinical hospital, oncology and phthisiology services. The register of patients with sarcoidosis in Tatarstan at the time of writing this article includes 1655 patients. Work is being carried out to increase the level of knowledge of doctors about sarcoidosis, they are recommended to familiarize themselves with teaching aid, available on the website of the Russian Respiratory Society. Thanks to the increasing level of physician literacy, sarcoidosis began to be identified not only by the results of fluorographic studies (in phthisiology and pulmonology) or by the symptom complex of Löfgren's syndrome (mediastinal lymphadenopathy, erythema nodosum, arthritis and fever - in rheumatology), but also by the skin and visceral signs of this granulomatosis.
Skin lesions occur in almost every 3rd patient with systemic sarcoidosis. The prevalence of different types of skin lesions varies both between races and between individuals. In Russia, the frequency of skin lesions with sarcoidosis, according to clinics where patients with pulmonary and systemic sarcoidosis are observed, is 22.5% of cases, while according to dermatologists - 63.4%. Skin lesions may be the first noticeable manifestation of the disease. Nodules, plaques, maculopapular changes, lupus pernio, cicatricial sarcoidosis are specific for sarcoidosis. Rare manifestations include lichenoid, psoriasis-like changes, ulcers, angiolupoid, ichthyosis, alopecia, hypopigmented macules, nail lesions and subcutaneous sarcoidosis. Sarcoidosis can also present with granuloma annulare plaques. Skin lesions in sarcoidosis are considered specific if the biopsy reveals noncaseating granulomas, and nonspecific if erythema nodosum. Skin biopsy allows early diagnosis of sarcoidosis through a minimally invasive procedure. Skin lesions have prognostic significance. Löfgren's syndrome is usually accompanied by a good prognosis and spontaneous remission. Maculopapular changes and subcutaneous nodules are most often accompanied by spontaneous remission within two years, while plaques and especially “lupus pernio” are signs chronic illness. Most skin lesions have mild symptoms and do not require treatment. However, chronic skin lesions, especially lupus pernio, lead to human disfigurement and have a strong psychological and social impact. Treatment of such changes is a challenge because such patients do not always respond to conventional therapy. However, the description individual cases indicates the curability of sarcoidosis of cutaneous localization. Thus, in a 28-year-old woman whose sarcoidosis manifested itself as purple plaques on the face, therapy with hydroxychloroquine 200 mg 2 times a day for 3 months did not lead to complete resolution of the changes, only prednisolone at a dose of 1 mg/kg, followed by a dose reduction in within 5 weeks ensured remission and absence of relapse during 6 months of observation. In a 52-year-old patient with multiple skin lesions, three weeks of hydroxychloroquine with low-dose prednisone was sufficient to resolve the changes. In a 15-year-old boy with isolated facial skin sarcoidosis, a combination of hydroxychloroquine and local application fluticasone – complete resolution of skin manifestations was noted after 4 months.
We present two clinical cases of skin sarcoidosis proven at the tissue level in combination with intrathoracic lesions.
Clinical case 1
In patient A., 49 years old, an employee, during the next fluorographic examination, bilateral dissemination was discovered. The patient had no complaints. During an objective examination, the supraclavicular lymph nodes were palpated. Breathing was vesicular, and no wheezing was heard. No skin changes, eye or joint lesions were found. The patient was sent to an anti-tuberculosis dispensary. The Mantoux test was positive - papule 16 mm (2 tuberculin units (TU) of purified tuberculin (PPD-L)), acid-fast mycobacteria (AFB) were not detected in the sputum. Disseminated pulmonary tuberculosis was suspected and the complex anti-tuberculosis drug Mairin-P (isoniazid + pyrazinamide + rifampicin + ethambutol) was prescribed. After 2 months, there was no clinical effect from the treatment, and after a phthisiatric commission, anti-tuberculosis therapy was canceled, a diagnosis of sarcoidosis was made, and the patient was referred to a pulmonologist. A repeated tuberculin test was negative, and no AFB were detected. External respiration function did not suffer (forced vital capacity (FVC) was 125%, forced expiratory volume in 1 second (FEV₁) - 127% of the expected values), diffusion capacity of the lungs for carbon monoxide (DLco) - 125% of the expected values. In a clinical blood test: ESR 8 mm/hour, leukocytes 7 × 10⁹/l, lymphocytes in the leukocyte formula – 31%. Ultrasonography(ultrasound) of organs abdominal cavity no pathology was detected. X-ray computed tomography (CT) of the chest showed dissemination in combination with interstitial changes (Fig. 1). Careful examination revealed limited changes in the scalp (Fig. 2) and body. A skin biopsy was performed and revealed chronic granulomatous inflammation of the sarcoid type. The final clinical diagnosis was made: “sarcoidosis of the intrathoracic lymph nodes and lungs, peripheral lymph nodes and skin.” Given the large volume of the lesion and multiple organs, systemic steroid therapy was planned, which the patient refused. As an alternative, pentoxifylline 0.2 g 3 times a day and vitamin E 200 mg 2 times a day were prescribed. After 8 months of this therapy, the patient felt satisfactory, but skin changes remained unchanged. When performing dynamic CT, a deterioration in the picture was noted - an increase in the number of lesions in the lungs, multiple lesions in the pleura (Fig. 3). Respiratory function remained within normal limits, but the dynamics were negative (FVC 103%, FEV₁ 105% of predicted, DLco 117% of predicted). Prednisolone was prescribed at a daily dose of 30 mg (morning - afternoon) and vitamin E. After 6 months of therapy, skin manifestations disappeared, according to the RCT picture, positive dynamics were achieved (Fig. 4). To this moment daily dose Prednisolone remained at 10 mg. However, steroid therapy was accompanied by such phenomena as an increase in body weight (from 79 kg to 94 kg), decreased visual acuity, and tooth decay. After 3 months, treatment with prednisolone (course of 9 months) was completed, but at this time weakness and changes in the scalp appeared, indicating the onset of an exacerbation after discontinuation of prednisolone. The patient was offered methotrexate therapy, but he refused and was started on repeat course steroid therapy.
Clinical case 2
Patient K., 41 years old, was identified when visiting a therapist due to weakness and progressive changes in the skin of the face. The diagnosis was made seborrheic dermatitis", but the skin changes became rough (Fig. 5); The patient was referred to an oncologist, who quickly performed a skin biopsy and revealed noncaseating epithelioid cell granulomas with multinucleated giant cells. Cutaneous sarcoidosis (“lupus pernio”) was diagnosed. There were no complaints from the lungs, breathing was hard, but no wheezing was heard. CT scan revealed enlarged intrathoracic lymph nodes, more on the left (Fig. 6). Spirometry parameters were within normal limits (FVC 87.5%, FEV₁ 90.9% of predicted). There were no changes in the hemogram; total blood Ca was 2.09 mmol/l. Functional tests livers were normal. The patient suffered from type 2 diabetes mellitus, obesity, hypertension, which made the use of glucocorticosteroids unsafe. The tactics of further therapy were discussed with the patient, she agreed to the use of cytostatic drugs, and she was prescribed methotrexate once a week orally: in the first week 5 mg, in the second - 10 mg and from the third week onwards - 15 mg for a period of 6 months with monthly monitoring of the number of leukocytes and lymphocytes in peripheral blood, as well as the activity of alanine and aspartate aminotransferase for early detection adverse effects of methotrexate use. The patient tolerated the treatment well. By the fifth month, the changes on the skin disappeared completely. Radiographs showed mild mediastinal lymphadenopathy.
Conclusion
Literature data and the results of our own observations indicated the great practical significance of recognizing skin manifestations of sarcoidosis and verifying multiple organ lesions using a minimally invasive procedure - skin biopsy. Antimalarial drugs, glucocorticoids and methotrexate remain promising methods of treating cutaneous forms of sarcoidosis. It is noted that the dynamics of skin lesions in sarcoidosis, as a rule, corresponds to the dynamics of intrathoracic changes and serves as an indicator of the effectiveness of therapy.
What are the symptoms of sarcoidosis and what are the causes of this disease? We will talk about how diagnosis occurs and what treatment methods are possible for use in the treatment of affected organs: lungs, skin, bones, heart.
What is sarcoidosis - definition of pathology
Sarcoidosis- This idiopathic disease (that is, the cause is unknown) and systemic, since can cover the entire body.
This inflammatory granulomatous benign pathology; its main characteristic is formation of granulomas in various organs and tissues.
Affects mainly females (although this has not been confirmed by a sufficient number of studies), aged 20 to 40 years; although rare cases have been reported in children, after 50 years of age and in the elderly.
Areas and organs that are affected by sarcoidosis
As we said, sarcoidosis is a multisystem disease that affects various organs and systems; it should be noted, however, that it can often begin in one organ and then spread to other anatomical structures. In addition, some disease sites are very common, while others are much rarer.
Based on the areas affected, the following can be distinguished: types of sarcoidosis:
- Lungs and bronchi. This is a rare form of sarcoidosis in which granulomas are localized at the level of the lungs and bronchial tree. Symptoms are variable: there may be asymptomatic forms and forms of respiratory failure.
- Upper respiratory tract. In this case (one of the rare forms of the disease), granulomas form at the level of the nose and larynx.
- Bones. All granulomas are localized mainly in small bones or at the level of the jaws. It does not occur very often - in 3-9% of patients.
- Skin. In 10-40% of patients suffering from sarcoidosis, skin lesions are noted as the only symptom, where they can sometimes coincide with bone injuries.
- The lymph nodes. It is a very common form of sarcoidosis, occurring in 70-90% of patients.
- Heart beats. Tumor-like formations at the level of the heart, localized predominantly in the left ventricle, are rare (only 5-10% of patients), but always have a poor prognosis and death; destruction of the heart parenchyma leads to heart failure.
- Liver. The liver is affected in two thirds of people suffering from sarcoidosis, and sometimes (in 20% of cases) it is accompanied by sorcaidosis of the spleen.
- Nervous system . In this case, they talk about neurosarcoidosis and it manifests itself only in 5% of cases. Primarily the cranial nerves are damaged, but the brain, brain stem and spinal cord can also be affected.
- Eyes. This form affects one percent of patients. Granulomas can be localized both in eyeball, and at the level of the eyelids and lacrimal glands, less often - at the level of the retina and conjunctiva.
- Kidneys. This form is found in approximately 25% of cases, involves the entire renal parenchyma and leads to progressive loss of renal function.
- Other forms. Sarcoidosis can also affect the salivary glands, mammary glands, pancreas, pituitary gland and hypothalamus, gastrointestinal tract and reproductive system.
Causes of sarcoidosis
The causes of sarcoidosis are still unknown today. It is assumed that autoimmune processes play a major role, but a genetic component cannot be excluded.
General and local symptoms of sarcoidosis
Sarcoidosis produces general and local symptoms related to the affected anatomical structure. Sometimes asymptomatic development of the disease can be observed.
Common symptoms include:
- Fever, general malaise, fatigue, joint pain, lack of appetite and weight loss.
- Changes in calcium metabolism caused by macrophages that form granulomas and produce large amounts hydroxycholecalciferol (active form vitamin D). This causes hypercalcemia and hypercalciuria. This general symptoms, but extremely important.
Specific sarcoidosis symptoms:
- Skin. Sarcoidosis of the skin is characterized primarily by the appearance of very painful purple nodules on the skin, which are localized mainly at the level of the lower extremities. Other symptoms of skin lesions: maculopapillary rash, ulcers, wounds, scar injuries. You may also experience hair loss.
- Heartfelt. Symptoms of heart damage include heart failure, cardiomyopathy, and heartbeat abnormalities (bradycardia or tachycardia). Sudden cardiac arrest is even possible, leading to the death of the patient.
- Bone. In this case, the typical symptom is pain in the bones and joints. Manifestations of rheumatic diseases such as arthritis and acute polyarthritis may also appear.
- Nervous system. Depending on the affected area, hematochemical changes may occur, such as leukopenia, thrombocytopenia and anemia, neuropathies, hearing and vision disorders (decreased vision and hearing, headaches, meningitis, paralysis or weakness in the face), seizures, dizziness, loss of smell, psychiatric disorders, epilepsy.
- Pulmonary. Pulmonary sarcoidosis may be completely asymptomatic or cause difficulty breathing, coughing with or without blood, shortness of breath, chest pain, and inability to bear weight.
- Liver and spleen. In this case, enlargement of the liver (hepatomegaly) and spleen (splenomegaly), as well as changes in hematochemical values (most often increased alkaline phosphatase levels) can be observed.
- Ophthalmic. Most common symptom– uveitis, but burning and itching in the eyes, visual disturbances, damage to the conjunctiva, glaucoma and cataracts may also occur.
- Kidneys. The most common symptom is renal failure; glomerulonephritis, interstitial nephritis, glomerular damage, kidney stones and nephrocalcinosis also appear.
- The lymph nodes. There is an increase in lymph nodes, a change in elasticity (they become hard to the touch).
- Upper Airways . Symptoms include nasal polyps and granulomatosis, hoarseness and dysphonia.
- Salivary glands. In this case, xerostomia, also known as dry mouth, is found.
- Hypothalamus and pituitary gland. Metabolic disorders occur.
Tests to diagnose sarcoidosis
Diagnosis of sarcoidosis carried out through various types of examinations, during which the general state patient, paying special attention to skin lesions, regularity of heartbeat and breathing, the presence of pain points and the shape of the lymph nodes.
Only after this can additional diagnostic studies be carried out:
- Blood analysis. Allows you to evaluate the levels of blood cells, thus identifying possible leukopenia and thrombocytopenia, and assessing organ function indicators. For example, in cases of liver damage, alkaline phosphatase levels increase, while elevated creatinine indicates kidney damage.
- Radiography. Particularly suitable for detecting granulomas in the lungs.
- Magnetic resonance imaging. Particularly useful in the diagnosis of sarcoidosis of the nerves and possible damage to the muscles and heart. It is also useful in identifying granulomas in the abdominal cavity: liver, spleen and pancreas.
- Six Minute Test. This test is done to test the patient's ability to exert effort while observing changes in the heartbeat.
- Breath test. Performed to assess the subject's breathing capacity.
- Hemogas analysis. Serves to assess the ability of the lungs to regulate the level of carbon dioxide and oxygen in the blood. It is performed using arterial blood sampling.
- Biopsy. It consists of obtaining a piece of tissue for subsequent examination under a microscope. It can be performed in cases of sarcoidosis of the lungs, bronchi, skin, conjunctiva and lymph nodes.
Therapy, risk factors and chances of cure
Sarcoidosis is a disease that can regress spontaneously, but in some cases (about 10-15% of individuals) it can become chronic.
Drug therapy for sarcoidosis
In case of diagnosed sarcoidosis, you should contact a specialized center that is able to provide treatment in accordance with the form and aggressiveness of the disease. Therapy is usually based on corticosteroids and lasts several months. If corticosteroids do not have an effect, then switch to more aggressive drugs:
- Cytotoxic drugs, that is, capable of killing cells. Chemotherapy drugs are typically used to treat autoimmune diseases such as rheumatoid arthritis. In the case of sarcoidosis, methotrexate is used, azathioprine And cyclophosphamide.
- Immunomodulators. Has a direct effect on the body's immune response. Such drugs are usually used after transplantation or to treat chronic autoimmune diseases. In case of sarcoidosis use pentoxifylline And infliximab.
- Antimalarial. Corticosteroids (such as chloroquine And hydroxychloroquine), which are used, in particular, in patients with high calcium levels, with cutaneous sarcoidosis.
The dosage and regimen are determined by the doctor depending on the case.
Sarcoidosis Risk Factors and Prognosis: What You Need to Know
Risk factors for developing sarcoidosis include:
- Some categories of persons, such as firefighters, foresters and workers who work in contact with silicon, aluminum, beryllium and zirconium powder are at risk due to dust inhalation. In such cases they talk about an occupational disease.
- Living in Scandinavian countries, where, due to the climate and high humidity, many cases of sarcoidosis appear to be reported.
- Cases in the family, especially if one of the parents suffers from the disease.
Sarcoidosis may develop individually, but in general we can say that:
- in 1/3 of patients, after three years from the moment of diagnosis, the disease goes into pseudo-remission, that is, it does not disappear completely, but is asymptomatic.
- in 2/3 of patients it goes into remission 10 years after the diagnosis of the first symptoms.
- In approximately 5% of cases, after a year of remission, the disease becomes active again.
- In 10-15% of patients suffering from sarcoidosis, the disease can become chronic.
