Atrophic malignant papulosis: causes, symptoms, diagnosis, treatment

Papulosis maligna atrophica (syn.: lethal cutaneous-intestinal syndrome, disseminated cutaneous-intestinal thromboangiitis of Kellmeyer, Degos disease) is a rare disease, the symptoms of which include lesions of the skin and internal organs (mainly the small intestine), caused by endothrombovasculitis, probably of autoimmune genesis. The role of viral infection in the development of the disease has not yet been proven.

Symptoms of malignant atrophic papulosis

Papulosis maligna atrophica usually develops in young people, less often in children. With the exception of isolated cases, the process begins with skin rashes, which for a long time, sometimes for several years, may remain the only clinical symptom of the disease. The rashes consist of isolated scattered pale pink papules with a diameter of 2 to 10 mm. Their central part sinks, gradually becoming porcelain-white, and the remaining peripheral ridge acquires a bluish tint, with telangiectasias visible on its surface. The rashes, few in number at first, can increase in number over time and cover the entire skin. Some of them regress, leaving "stamped" scars, but new ones appear to replace them. Most often, papules are located on the trunk and proximal parts of the extremities. After a relatively calm period, characterized only by skin symptoms, the second stage suddenly occurs - damage to the gastrointestinal tract with perforations of the small intestine, the development of peritonitis, which is the main cause of death.

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

Pathomorphology of malignant atrophic papulosis. Initial changes in the skin are ischemic infarction, facing the epidermis with a broad base, having the appearance of a structureless mass, palely stained with hematoxylin and eosin. Toluidine blue stains it metachromatically in a pinkish-lilac color due to the presence of 8 NMG. A weak inflammatory reaction is characteristic around the necrotic focus, and only along the periphery in places are small accumulations of mononuclear cells detected. The epidermis in the infarction area is atrophic, mainly with necrotic changes in epithelial cells, when the necrotic masses melt, it can separate from the dermis. Hair follicles and vessels in the necrotic focus are mostly absent.

In later stages, collagen fibers appear in the infarction zone, partially hyalinized, arranged in bundles in various directions. There are usually very few cellular elements. Between the collagen bundles, individual small necrotic areas can be seen.

Histogenesis of malignant atrophic papulosis. The cause of infarction is damage to small arteries and arterioles in the form of endovasculitis, characterized by proliferation of the intima and swelling of endothelial cells, often accompanied by thrombosis. In the central zone of infarctions, a fibrinolysis defect is detected. Factors damaging the endothelium are unknown, but it is assumed that these are mononuclear leukocytes. Direct immunofluorescence in the vessels of the deep dermis revealed deposits of IgM or IgG associated with the C3 component of complement, which may indicate immunological disorders leading to endovasculitis. Sometimes granular deposits of IgG, IgA and the C3 component of complement are detected along the dermoepidermal zone, as well as around small veins.

Electron microscopic examination revealed particles resembling paramyxoviruses in some endothelial cells. Although most authors attribute the glycosaminoglycan deposits detected in the lesions to secondary changes in the ischemic zone, there are supporters of the theory of disease development against the background of progression of cutaneous mucinosis. The role of genetic factors is possible.

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