Shamberg’s disease (hemosiderosis of the skin, chronic pigmented purpura, progressive pigmented dermatosis) is a chronic disease that has arisen as a result of damage to the vascular wall. Schamberg’s disease is characterized by spot hemorrhages (petechiae), eventually turning into brown-brown spots. The diagnosis is carried out using dermatoscopy of the patient’s skin. In the treatment of Shamberg’s disease, glucocorticoids, anti-inflammatory drugs are used, and if necessary, plasmapheresis and hemosorption are performed.
The content of the article:
Symptoms of Shamberg’s disease
Diagnosis of Shamberg’s disease
Treatment of Shamberg’s disease
Shamberg ‘s disease
Shamberg’s disease is a rare skin disease that is not life—threatening, but it needs to be treated immediately. To date, Shamberg’s disease has not been fully studied, especially with regard to the mechanism of its development. There is a statement according to which the damage to the capillaries is autoimmune in nature, since circulating immune complexes are deposited on their walls. Inflammation provokes an increase in the permeability of the capillary wall, so intradermal petechial hemorrhage may occur.
The hemoglobin released from the vascular bed contains iron, which is converted into the pigment hemosiderin after a while. It is worth noting that it is hemosiderin that has a decisive influence on the color of spots characteristic of Shamberg’s disease. More often, young men suffer from this disease.
Symptoms of Shamberg’s disease
In Schamberg’s disease, rashes are characterized by a variety of appearance and symmetry. The diversity lies in the presence of both old and new petechiae on the skin. Shamberg’s disease will not cause changes in a person’s well-being, he may not even suspect anything. Shamberg’s disease is characterized by a lesion of only the skin capillary. The disease is benign in nature. Hemorrhages in internal organs are not observed.
Shamberg ‘s disease is divided into four types:
petechial;
lichenoid;
telangiectatic;
eczema.
The petechial type of Shamberg’s disease is characterized by the presence of spots of various sizes on the skin with uneven outlines. These spots are small petechial hemorrhages. After a while, they may change color to brown-yellow or brown-brown. Such changes are not accompanied by discomfort or swelling. The skin on the thighs and shins is affected.
With the lichenoid type, spots of brown-brown hemosiderosis, petechial-type rashes and small shiny nodules appear. It is worth noting that the skin color remains unchanged. There are cases when the lichenoid type of the disease is characterized by the presence of only nodules. At the same time, the rash has a multiple character, spots appear on any part of the patient’s body.
Schamberg’s telangiectatic type disease can be characterized by medallion-like spots on the skin. In the center of these formations there is a cystic-expanded section of capillaries (vascular type), and point hemorrhages are located on the periphery.
The eczematoid type is a combination of rashes resembling the petechial type and rashes characteristic of eczema. In the area of foci of brownish-brown spots and spot hemorrhages, puffiness occurs, crusts, nodules and bubbles may appear. Rashes are accompanied by severe itching. This symptom is also observed in eczema.
Diagnosis of Shamberg’s disease
Only a dermatologist can make a diagnosis. The exact diagnosis of Shamberg’s disease is usually made on the basis of dermatoscopy of the rash area and the general clinical picture. The papillary part of the dermis is examined using a microscope, which allows you to identify some changes in the capillaries. These may be clusters of immune cells, bulbous vasodilation or swelling of the capillary wall.
The detection of erythrocytes indicates that there is an increased permeability of the capillary wall. In addition, necrosis and ulceration of the capillary wall may occur. Shamberg’s disease causes the appearance of one nodule or a pair of similar formations on the walls of capillaries.
Treatment of Shamberg’s disease
In Shamberg’s disease, treatment is complex. First, diet therapy is prescribed with the exception of irritating and potentially allergenic products. Excluded: chocolate, coffee, tea, citrus fruits, fried dishes, alcohol and smoked meats. Hypothermia, bruises, and physical overloads should also be avoided.
In parallel with the treatment of Shamberg’s disease, it is necessary to carry out therapy of concomitant diseases. This also applies to endocrine disorders. Also, close attention is paid to the rehabilitation of foci of chronic infection. If we talk about endocrine disorders, they can provoke the development of the disease, contribute to its degeneration into a chronic type.
The autoimmune mechanism of the development of Shamberg’s disease requires the use of corticosteroids during treatment: methylprednisolone, prednisolone, dexamethasone, etc. Potassium and calcium preparations, vitamins will help strengthen the walls of blood vessels. To reduce inflammation and pain, nonsteroidal anti-inflammatory drugs are used.
Ointments are also widely used in the treatment. The walls of the vessels will help strengthen rubbing with heparin. Drugs with corticosteroids will reduce the symptoms of itching, swelling and inflammation.
Severe cases of Shamberg’s disease require the use of specific techniques to remove immune complexes from the blood: plasmapheresis, hemosorption and cryoprecipitation.
