Polymorphic dermal angiitis (Gougereau-Ruiter’s disease) is a chronic recurrent dermatosis caused by a nonspecific inflammatory process in the walls of skin vessels. It is characterized by a pronounced variety of clinical manifestations: plaques, nodules, skin hemorrhages, blisters, blisters, areas of skin edema, pustules, superficial necrosis, ulcers, scars. The main method of diagnosis is dermatoscopy. Treatment may include the appointment of anti-inflammatory and antihistamines, antibiotics, glucocorticoids, vitamins, calcium preparations, chloroquine. Severe forms of the disease require the use of extracorporeal methods of blood purification.
General information
Currently, dermatology adheres to the immunocomplex theory of the development of polymorphic dermal angiitis, according to which vascular lesions are caused by the action of circulating immune complexes (CIC) on their wall. Local or general infections (staphylococcal, streptococcal, angina, influenza, ARI, rubella, measles, etc.) can act as antigens in this case. With this disease, thickening and bulging of the vascular wall occurs at the sites of the CIC lesion, nodules can form in its thickness. In the future, it is impregnated with fibrin, its permeability increases, which leads to the appearance of hemorrhages. There is necrosis of individual sections of the vessel wall and compensatory proliferation of others.
Symptoms
Polymorphic dermal angiitis, as a rule, occurs with a lesion of the skin of the shins. However, there may be another localization of rashes. In some cases, the appearance of skin elements is preceded by general symptoms: fatigue, fever, weakness, arthralgia, headache. Appearing on the skin, the rash lasts up to several months. After their resolution and healing, the impression of a complete recovery of the patient is created, but there is always a risk of relapse.
According to the predominance of certain elements of the rash, several varieties of polymorphic dermal angiitis are distinguished. The urticarian variant of polymorphic dermal angiitis is manifested by the appearance of blisters on any skin areas of different sizes, which resembles the clinical picture of urticaria. But, unlike the latter, rashes are accompanied not by itching, but by a kind of discomfort and soreness.
Hemorrhagic variant is the most common type of polymorphic dermal angiitis. It is characterized by the appearance of focal intradermal hemorrhages and may resemble other hemorrhagic purpura (for example, with systemic vasculitis or hemorrhagic fever). This also includes Schenlein-Henoch disease, a hemorrhagic variant of polymorphic dermal angiitis with general symptoms, abdominal pain and bloody stools.
The papulonodular variant is relatively rare. Its manifestation is flattened nodules and large (larger than a walnut) nodes, not accompanied by any discomfort, but painful when probing. The papulonecrotic variant of polymorphic dermal angiitis is represented by small inflammatory nodules, in the center of which necrotic changes occur over time. A black crust forms, under which bleeding ulcers are found. Healing occurs with the formation of scars. Localization of elements on the extensor surface in the area of limb joints is characteristic. This form of the disease must be differentiated from papulonecrotic tuberculosis of the skin.
The pustular-ulcerative variant begins with the appearance of pustules and vesicles on the skin of any part of the body, resembling manifestations of folliculitis or acne. Then deep ulcers form at the site of the rash, prone to increase in size and healing with the formation of rough scars.
Necrotic-ulcerative variant is characterized by the formation of blood clots in the affected vessels, which causes necrosis of entire skin areas with the formation of a black scab. It has a severe course with a lightning onset and rapid deterioration of the patient’s condition, often leading to a fatal outcome. If the patient does not die, then necrotic areas transform into long-term healing and poorly treatable ulcers, leaving large scars. The polymorphic variant is characterized by the combined manifestation of several variants of polymorphic dermal angiitis at once.
Diagnosis and treatment
The diagnosis is carried out by a dermatologist. The main method of investigation in this case is dermatoscopy, during which swelling and inflammation of the walls of vessels passing through the skin, their impregnation with fibrin, necrotic changes, thrombosis, the exit of blood cells outside the vessel and areas of endothelial proliferation are detected.
Anti-inflammatory drugs (phenylbutazone, indomethacin), antihistamines (desloratadine, loratadine, chloropyramine), calcium preparations, vitamins B15, P and C. are used in the treatment. If the association of polymorphic dermal angiitis with infection is detected, antibiotic therapy is performed. In severe cases, it is necessary to use glucocorticosteroid therapy and methods of extracorporeal hemocorrection: hemosorption, plasmapheresis, cryoaferesis. With a recurrent course, a long course of chloroquine is prescribed.
In the local treatment of polymorphic dermal angiitis, bandages and ointments with corticosteroids are used, for ulcers and necrotic lesions — methylurocyl ointment, lotions with chymotrypsin.
