Purpura pigmentosa progressive chronic: causes, symptoms, diagnosis, treatment

Purpura pigmentosa chronica (syn.: purpuric-pigmented dermatosis, hemosiderosis). Depending on the difference in the clinical picture or the mechanism of occurrence, purpuric-pigmented dermatoses in the literature have a large number of forms.

In the classification of M. Samilz (1981), the group of purpuric-pigmentary dermatoses includes pigmented progressive dermatosis of Schamberg, eczema-like purpura of Lucas-Kapitanakis, itchy purpura of Leventhal, transient pigmented-purpuric rashes of the lower extremities and, annular telangiectatic purpura of Majocchi, pigmented purpuric lichenoid dermatitis of Gougerot-Blum, serpiginizing angioma of Hutchinson, as well as monologically similar, but etiologically different hyperglobulinemic purpura of Waldenstrom and stasis dermatitis. The combination of many forms of purpuric-pigmentary dermatoses into one disease is poorly justified, in connection with which it is indicated the need for a more in-depth study of the etiology and pathogenesis of hemosiderosis. According to H. Zaun (1987), various forms of hemosiderosis are based on specific pathological mechanisms.

Symptoms of purpura pigmentosa chronic progressive

The clinical picture is polymorphic, but the leading ones are three clinical signs: erythema, hemorrhages, pigmentation. They occur in various combinations. Lichenoid rashes, eczema-like changes, telangiectasias are observed less often, but are considered signs inherent in one or another form of hemosiderosis. Thus, the most characteristic of Schamberg's disease are pigmentation and brightly colored point hemorrhages located in its zone or on the periphery, for Majocchi's purpura - telangiectasias, for pigmented purpuric dermatitis of Gougerot-Blum - lichenoid rash, etc. With prolonged existence, it is possible to develop mild superficial atrophy. Itching, as a rule, is slight, more intense in itchy Leventhal's purpura. The disease develops in attacks, spreading with each relapse to increasingly larger areas of the skin. It usually begins with pinpoint hemorrhages that first appear on the knees, in the armpits, on the back of the feet, often symmetrically, then on the thighs, torso, and arms. Men are more often affected. The disease can occur at any age, including in children. The course is chronic, but the general condition is not disturbed. The Rumpel-Leede symptom is positive only in the area of the lesions. In some patients, the process can regress spontaneously.

Pathomorphology of progressive chronic pigment purpura. Skin changes in all the above forms of hemosiderosis are similar with some minor features. In the main upper part of the dermis, a dense infiltrate is found, consisting of lymphocytes, histiocytes, tissue basophils and fibroblasts. Among the infiltrate cells, neutrophilic granulocytes and plasma cells can be found, sometimes in significant quantities. Changes in the vessel walls can be defined as productive-infiltrative capillaritis with thickening of the walls and dense infiltration by lymphoid elements. Hemosiderin is almost always found around the vessels, as well as among the infiltrate cells. As a rule, there are no changes in the epidermis, only in the acute period exocytosis is noted in it, and in some cases, for example, with eczematous purpura of Dukas-Kyapitanakis, unevenly expressed acanthosis and parakeratosis, focal intercellular edema, sometimes with the formation of bubbles.

Histogenesis of chronic progressive pigment purpura. Based on immunological analysis and the corresponding histological picture, it is believed that hemosiderosis is based on delayed-type hypersensitivity. Etiological agents can enter the body as a result of direct contact or by inhalation. Quite often, this group of diseases can be caused by drugs (bromurea, biocodyl, diazepam), which is proven by skin tests.

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