Malignant atrophic papulosis: causes, symptoms, diagnosis, treatment

Papulosis malignant atrophy (syn: lethal skin-intestinal syndrome, disseminated skin-intestinal thromboangiitis of Kelmeyer, Degos disease) is a rare disease, the symptoms of which include skin and internal organs (mainly small intestine), caused by endotrombovasculitis, probably of autoimmune origin . The role of viral infection in the development of the disease has not yet been proven.

Symptoms of Papulosis of Malignant Atrophy

Papulosis malignant atrophy usually develops at a young age, less often in children. Except for single cases, the process begins with skin rashes, which for a long time, sometimes for several years, can remain the only clinical symptom of the disease. The rash consists of isolated scattered pale pink papules with a diameter of 2 to 10 mm. The central part of them sinks, gradually becomes a faience-white, and the remaining peripheral cushion acquires a cyanotic shade, on the surface of it are visible telangiectasias. Rashes, a few at the beginning, may increase in number over time and cover the entire skin. Some of them regress, leaving "stamped" scars, but they are replaced by new ones. Most often, the papules are located on the trunk and proximal parts of the limbs. After a relatively quiet period, characterized only by skin symptoms, the second stage suddenly begins - a lesion of the gastrointestinal tract with perforations of the small intestine, the development of peritonitis, which is the main cause of death.

Cases of cerebral infarction, as well as significant changes in the central and peripheral nervous system, eyes, mucous membrane of the oral cavity without signs of gastrointestinal tract damage are described, despite the long-term course of the disease.

Pathomorphology of papulosis malignant atrophy. Initial changes in the skin are ischemic infarction, which is turned with a broad base to the epidermis, having the form of an unstructured mass, pale with hematoxylin and eosin. Toluidin blue it is stained metachromatically in a pinkish-lilac color as a result of the presence of eight glycosaminoglycans. A weak inflammatory reaction around the necrotic focus is typical, and only small concentrations of mononuclear cells are detected at the periphery. The epidermis in the infarction area is atrophic. Mainly with necrotic changes in epithelial cells, when melting necrotic masses can be separated from the dermis. Hair follicles and vessels in the focus of necrosis are mostly absent.

In later stages, collagen fibers appear in the infarction zone, partially hyalineized, arranged in the form of bundles in various directions. Cellular elements are usually very small. Between the bundles of collagen, you can see individual small necrotic areas.

Histogenesis of papulosis malignant atrophy. The cause of myocardial infarction is the destruction of small arteries and arterioles in the form of endovascular disease. Characterized by the proliferation of intima and the swelling of endotheliocytes, often accompanied by thrombosis. In the central zone of infarcts, a fibrinolysis defect is detected. The factors damaging the endothelium are not known, but it is assumed that these are mononuclear leukocytes. Direct immunofluorescence in the vessels of the deep sections of the dermis revealed deposits of IgM or IgG, associated with the C3-component of complement, which may indicate immunological disorders leading to endovascular infection. Sometimes granular deposits of the IgG, IgA and C3 complement components along the dermoepidermal zone are identified, as well as around small veins.

In electron microscope studies, particles resembling paramyxoviruses were found in some endothelial cells. Although the majority of authors detected in the lesion lesions of glycosaminoglycans are attributed to secondary changes in the ischemia zone, there are supporters of the theory of the development of the disease on the background of the progression of cutaneous mucinosis. The role of genetic factors is possible.

[1], [2], [3], [4], [5], [6], [7], [8]