Schamberg's disease: timely diagnosis will help to avoid complications

Chronic pigmentary purpura, or Schamberg's disease, usually does not cause pain or physical discomfort in the patient, so most often he comes to the dermatologist with an advanced form of the disease. Often, the first to pay attention to the characteristic rashes on the body becomes a cosmetologist during procedures that require undressing. It is very important to refer the patient to a dermatologist in time and make the correct diagnosis in order to avoid the development of complications.

Schamberg's disease belongs to vasculitis, a group of diseases characterized by inflammation of the walls of blood vessels not only in the dermis, but also in the subcutaneous tissue. A certain role in the development of vasculitis is played by chronic intoxication, endocrinopathies, metabolic disorders, hypothermia, general and local vascular diseases, and diffuse connective tissue diseases. The disease can last for years, accompanied by damage to blood vessels and internal organs. Timely diagnosis is important and often requires histopathological examination to confirm the diagnosis.

Patient P., aged 69, was admitted with complaints of spots, slight itching, burning of the skin of the trunk and extremities. From the anamnesis it is known that the disease arose 5 years ago, when for the first time on the right forearm, 2 years after the burn, a focus appeared 2x2 cm in size, rounded, red in color with clear boundaries, not causing any subjective sensations. After 6 months, she noted the appearance of similar rashes on the skin of the left forearm, then on the thighs, the rashes were accompanied by a slight burning sensation and itching. The process progressed, multiple spots appeared on the skin of the lower extremities, the dorsum of the feet, then on the buttocks, abdomen, back, chest and upper limbs, including the dorsum of the hands. Over the past 2 years, she was hospitalized twice in the regional dermatological and venereal department for dermatitis. She received calcium gluconate, suprastin, sodium thiosulfate, externally - ointments with corticosteroids.

In the anamnesis of life the patient notes frequent colds, she is seen by a cardiologist for hypertension, and by a mammologist for fibrocystic mastopathy.

Objectively: the general condition is satisfactory. The skin process is common, localized on the skin of the trunk, limbs; it is represented by multiple polymorphic multi-colored, crimson, red of various shades (dark red and brown on the shins), well demarcated, sometimes with uneven contours, rounded or polycyclic in shape, in places confluent extensive spots, slightly elevated plaques, pasty and more dense, with single purple papules along the edge or in the center; in some places - vasodilatation with darkening of the skin. The skin is dry, in places - peeling.

During the examination in the blood test, slightly elevated ESR and hemoglobin levels were noted, in the clinical analysis of urine - leukocyturia, cylindruria, oxalaturia. In the biochemical analysis of blood - bilirubinemia. Elevated levels of antibodies to denatured and formalized DNA were recorded. Coagulogram without features. Fluorography of the chest organs within the age norm.

Histological examination: the epidermis is practically unchanged, in the upper parts of the dermis there is diffuse infiltration, mainly by lymphocytes, histiocytes, fibroblasts with a small admixture of plasma cells; the walls of the vessels are thickened, the endothelium proliferates. When stained by Perls, hemosiderin is determined in the ground substance, histiocytes and endothelial cells. Kaposi's sarcoma, elevated erythema, Gougerot-Duperre disease and Bazin's erythema were considered in terms of differential diagnosis.

The clinical presentation, site (including lower extremities) and onset suggest Kaposi's sarcoma. In Kaposi's sarcoma, as in this case, the process may begin with the appearance of dark red spots, which turn into smooth, dense, purple-red merging plaques as they grow. However, with Kaposi's sarcoma, skin changes quite often begin with the appearance of red-bluish or reddish-brown small spots ranging in size from lentils to 1 cm. It is also important to note that in most patients with Kaposi's sarcoma, the color of spotted elements usually changes over time : reddish-cyanotic color turns into dark brown, reminiscent of the color of malignant melanoma. The spots are usually quite well demarcated, have a smooth surface,

Histologically, in contrast to Schamberg's disease, Kaposi's sarcoma shows dilation and proliferation of dermal vessels with an inflammatory response resembling granulation tissue. Characterized by hyperplasia with the penetration of endothelial cells into the walls of blood vessels. Tumor-like proliferation of both lymphatic and blood vessels is observed.

The obtained histological data differ from elevated erythema by the absence of hyaline degeneration of smooth-looking pericapillary reticulin fibers, and by the absence of polymorphonuclear leukocytes in the perivascular infiltrate.

Gougerot-Duperre disease excludes the absence of fibrinoid necrosis on histological examination; unchanged or pyknotic cell nuclei in the infiltrate, dust-like fragments of chromatin, a thrombus in unchanged capillaries, leukoclasia, nuclear necrosis and free chromatin fragments, as well as the absence of damage to small arteries and arterioles.

The pathohistological difference from Bazin's indurative erythema is the absence of caseous necrosis, atrophy of adipose tissue, sclerosis and fibrosis in the hypodermis.

The diagnosis was made: Schamberg's disease.

Treatment prescribed: vascular drugs, detoxification therapy, antihistamines, hepatoprotectors, cardioprotectors. The progression of the skin process as a result of the therapy was suspended. Significant blanching is observed in the foci. There are no subjective sensations. In the matter of differential diagnosis, pathohistological examination in this case was the decisive diagnostic criterion in establishing the diagnosis.

According to kiai.com.ua.