Atrophoscleroderma limited Pasini

Atrophosclerosis Pasini O. Sclerosis can be limited, with multiple deep incisions (atopic sclerosis) or extensive with atrophic scars and telangiectasia (scleroderma). Pasini's sclerosis is based on albinism, manifested in congenital melanopenic pigmentation. With a limited form of sclerosis, dystrophic skin damage is expressed, combined with thinning of the epidermis, hypopigmentation, and superficial peeling. The skin becomes dull, easily injured, atrophic areas appear in the depths of the dermis and subcutaneous tissue, the skin is bluish-white and has a “button” consistency. The spread of the process can be limited to the face, limbs, and less often the torso. The disease always begins at a young age (the average age of onset is about 20 years). Along with the classical form, a ring-shaped form is distinguished, characterized by the formation of an area of ​​hyperemia around the areas of atrophy with lenticular, scaly elevations raised above it by 2–3 mm. In addition, there is a juvenile form of sclerosis, which occurs in middle childhood or adolescence. It is characterized by severe moderate infiltration of the skin of the face, trunk and limbs of a purple color with a bluish tint, damage to muscles and joints, the formation of telangiectasia, and slight sweating at low ambient temperatures.

Pazuk O.K. calls this type sclerosis - Papini Pirini (Italy, 1876), and other authors note the possibility of the transition of a limited form to a widespread (endemic). The clinical picture persists for many years, and isolated rashes are observed only extremely rarely. A type of sclerosis spreading to the face and proximal limbs in the form of a mask or face with unilateral trapezoidal scars has been described; There is also a long-term course without skin lesions. Atrophonecrosis of the skin manifests itself in the form of death of the skin in areas of the skin affected by atrophosclerosis (mainly on the face). In addition, atrophonecrtosis also appears in sclerosing atrophoproliferative diseases (keloids, desquamative or bullous pemphygoid degeneration, pigmented xanthomas, painful ulcers). The most difficult differentiation of diseases of this group is scleroderma pityriasis parakeratotic aging of the skin in old age. In contrast to atrophonecrosis, it develops against the background of dyschromia of the scalp. The course of sclerosis is protracted and progradient. Scars, starting from the early period, are persistent, depigmented, stagnantly bluish in color and consist of chaotically intertwined leathery and normal elements (D. A. Molo