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

Idiopathic atrophoderma of Pasini-Pierini (synonyms: superficial scleroderma, flat atrophic morphea) is a superficial large-spotted skin atrophy with hyperpigmentation.

The causes and pathogenesis of the disease have not been established. There are neurogenic (location of foci along the nerve trunk), immune (presence of antinuclear antibodies in the blood serum) and infectious (detection of Borrelia burgdorferi antibodies in the blood serum) genesis of the disease. Along with primary atrophoderma, there is probably a form close to or identical to superficial focal scleroderma. A combination of both is possible. Familial cases have been described.

Symptoms of idiopathic atrophoderma of Pasini-Pierini. Idiopathic atrophoderma of Pasini-Pierini most often affects young women.

Clinically, predominantly 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 sunken foci of atrophy with translucent vessels, round or oval outlines, brownish or livid-red color. Hyperpigmentation in the lesions is characteristic. There may be disseminated small-spotted foci on the trunk and proximal parts of the extremities. Subjective disorders are absent, the course is long, progressive, 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 round superficial, slightly sunken foci of atrophy with translucent vessels of a brownish or livid-reddish color are formed. The presence of hyperpigmentation is characteristic. The skin around the lesion is unchanged. The compaction at the base of the plaques is almost completely absent. In most patients, the purple halo along the periphery of the foci is absent. A combination with focal scleroderma and (or) scleroatrophic lichen is possible.

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

Pathomorphology. In fresh lesions, edema of the dermis, dilation of blood vessels (especially capillaries) and lymphatic vessels are observed. The walls of the lymphatic vessels are slightly thickened and edematous. In older lesions, atrophy of the epidermis is observed, and a significant amount of melanin is present in the basal cells. The dermis is edematous, its reticular layer is significantly thinned, the collagen fiber bundles of the upper dermis are even thicker compared to fresh lesions, sometimes compacted and homogenized. Similar changes in the collagen fibers can be observed in deeper parts of the dermis. Elastic fibers are mostly unchanged, but in places they are fragmented, especially in the deep parts of the dermis. The vessels are dilated, around them there are lymphocytic infiltrates, which can also be observed perifollicularly. The skin appendages are mostly without any particular changes; only in areas of collagen compaction and sclerosis can they be compressed by fibrous tissue, and sometimes completely replaced by it.

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

Differential diagnosis. This disease should be distinguished from focal scleroderma. In differential diagnosis, it is necessary to take into account that in Pasini-Pierini atrophoderma, there is swelling of collagen in the middle and lower parts of the dermis, absence of sclerosis, changes in elastic tissue due to swelling and congestion in deep vessels.

Treatment of idiopathic atrophoderma of Pasini-Pierini. At an early stage, penicillin is prescribed at 1 million units per day for 15-20 days. Externally, agents are used to improve blood circulation and tissue trophism.

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