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

Purple pigmentary progressive chronic (syn: purpuro-pigmented dermatosis, hemosiderosis). Depending on the difference in the clinical picture or mechanism of the occurrence, purpuro-pigmentary dermatoses in the literature have a large number of forms.

In the classification of M. Samilz (1981), the group of purpuro-pigmented dermatoses includes pigmentary progressive dermatosis of Schamberg, an eczema similar to the purple of Lucas-Captainakis. Pruritic purpura of Leventhal, transient pigment purpuric eruptions of the lower limbs and, ring-shaped telangiectatic purpura of Mayokki, pigment purpurea lichenoid dermatitis of Guzero-Blum, serpiginating Hutchinson's angioma, as well as monologically similar but etiologically different Waldenstrom hyperglobulinemic purpura and congestive dermatitis. The combination of many forms of purpuric-pigmented dermatoses into one disease is poorly grounded, which points to the need for a deeper study of the etiology and pathogenesis of hemosiderosis. According to N. Zaun (1987), various forms of hemosiderosis are based on specific pathological mechanisms.

Symptoms of purpura pigmentary progressive chronic

The clinical picture is polymorphic, but the leading ones are three clinical signs: erythema. Hemorrhage, pigmentation. They occur in various combinations. Lichenoid rashes, eczematoid changes, and telangiectasias are less common, but are seen as signs of some form of hemosiderosis. Thus, the most characteristic for the disease of Schamberg are pigmentation and brightly colored point hemorrhages located in its area or along the periphery, for purpura of Majokka-telangiectasia, for pigment purpurea dermatitis of Guzero-Blum-lichenoid rash, etc. With prolonged existence, it is possible to develop a mildly pronounced superficial atrophy. Itching, as a rule, is small, more intense with the itchy purple of Leventhal. The disease develops paroxysmally, spreading with every relapse to all large areas of the skin. It usually starts with pinpoint hemorrhages that appear first on the knees, in the armpits, on the rear of the feet, often symmetrically, then on the hips, trunk, hands. Suffer a cup of a man. The disease can occur at any age, including in children. The course is chronic, but the general condition is not violated. The Rumpel-Leide symptom is positive only in the area of lesions. In some patients, the process can regress spontaneously.

Pathomorphology of purpura pigmentary progressive chronic. Changes in the skin with all the forms of hemosiderosis are similar with some small features. In the main part of the upper part of the dermis, a dense infiltrate is found, consisting of lymphocytes, histiocytes, tissue basophils and fibroblasts. Among the cells of the infiltrate, neutrophilic granulocytes and plasmocytes can be detected, sometimes in a significant amount. Changes in the walls of the vessels can be defined as a productive-infiltrative capillary with thickening of the walls and dense infiltration of lymphoid elements. Around the vessels, as well as among the cells of the infiltrate, hemosiderin is almost always found. In the epidermis there are usually no changes, only exocytosis is observed in the acute period, and in some cases, for example, in the case of Dukas-Kiapitanakis purulent purple, uneven acanthosis and parakeratosis, focal intercellular edema, sometimes with the formation of vesicles.

Histogenesis of purpura pigmentary progressive chronic. Based on the 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 inhalation. Quite often this group of diseases can cause medicines (bromocarbamide, biocodil, diazepam), which is proved by skin tests.

[1], [2], [3], [4], [5]