Idiopathic atrophoderma of Pasini-Pierini: causes, symptoms, diagnosis, treatment

Idiopathic atrophodermia Pasini-Pierini (synonyms: superficial scleroderma, flat atrophic morphae) is a superficial large-sparse atrophy of the skin with hyperpigmentation.

Causes and pathogenesis of the disease are not established. There is a neurogenic (location of foci along the nerve trunk), immune (presence of antinuclear antibodies in the serum) and infectious (detection of Borrelia burgdorferi antibodies in the serum of the blood). Along with the primary atrophodermia, there is probably a form close or identical to superficial focal scleroderma. Perhaps their combination. Family cases are described.

Symptoms of idiopathic atrophodermia Pazini-Pierini. Idiopathic atrophodermia Pazini-Pierini more often sick young women.

Clinically, mainly on the skin of the trunk, especially along the spine, on the back and other parts of the trunk, there are a few large superficial, slightly fading foci atrophy with translucent vessels, round or oval outlines, brownish or reddish-red. Characterized by hyperpigmentation in the lesions, There may be disseminated small-spotted foci on the trunk and proximal limbs. Subjective disorders are absent, the course is long, progressing, along with the increase in old foci, new ones may appear. Spontaneous stabilization of the process is possible. The number of lesions can be very diverse - from one to several. 

Large oval or rounded surface, slightly sinking foci of atrophy with translucent vessels of a brownish or reddish-red color are formed. Characteristic of the presence of hyperpigmentation. The skin around the lesion is not changed. The compaction in the base of the plaques is almost completely absent. In most patients, the purple corolla is absent along the periphery of the foci. Perhaps a combination with focal scleroderma and (or) sclerotrophic lichen.

Based on clinical observation, some dermatologists consider the idiopathic atrophodermia of Pasini-Pierini a transitional form between plaque scleroderma and skin atrophy.

Pathomorphology. In fresh lesions, edema of the dermis, dilatation of the blood (especially capillaries), and also lymphatic vessels are observed. The walls of the lymph vessels are slightly thickened and swollen. In older foci, there is atrophy of the epidermis, in the basal cells - a significant amount of melanin. The dermis is edematous, its mesh layer is much thinner, the bundles of collagen fibers of the upper dermis are even thicker than fresh foci, sometimes compacted and homogenized. Similar changes in collagen fibers can be observed in deeper parts of the dermis. Elastic fibers are largely unchanged, but in some places they are fragmented, especially in the deep sections of the dermis. The vessels are dilated, around them there are lymphocytic infiltrates, which can be observed and perifollicular. Appendages of the skin mostly without any special changes, only in places of compaction and sclerosis of collagen they can be squeezed by fibrous tissue, and sometimes completely replaced by it.

Histogenesis is poorly understood. There are assumptions about the possible neurogenic or the immune genesis of the process. There is also a hypothesis about the infectious origin of the disease, based on the detection in some patients of elevated titer of antibodies to the causative agent of borreliosis - Borrelia burgdorferi.

Differential diagnosis. This disease should be distinguished from focal scleroderma. In differential diagnosis, it should be taken into account that at Pasini-Pierini's atrophodermia collagen edema is observed in the middle and lower parts of the dermis, absence of sclerosis, changes in elastic tissue due to edema and stagnation in deep vessels.

Treatment of idiopathic atrophodermia Pazini-Pierini. At an early stage, penicillin is prescribed for 1 million units. Per day for 15-20 days. Outwardly, agents that improve blood circulation and tissue trophism are used.

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