Perforating serpiginating elastosis: causes, symptoms, diagnosis, treatment

Elastoza perforating serpiginating (syn: keratosis follicular serpiginating Lutza, intrapapillary perforating veraciforme elastomeric Michera) is a hereditary disease of connective tissue of unknown etiology, sometimes combined with Chernogubov-Ehlers-Danlos syndrome, elastic pseudocanthoma, imperfect osteogenesis, Marfan syndrome. Clinically manifested by the presence of figured foci, the ring is ring-shaped, consisting of horny papules of reddish-brown color with a depression in the center, after the regression of which remain small atrophic scars. Eruptions are located mainly in the neck and neck, but may also be disseminated. An autosomal dominant type of inheritance is assumed. There are sporadic cases. Possible development of the disease in the long-term use of D-penicillamine.

Pathomorphology. There is an increase in the number and thickness of elastic fibers, especially in the papillary layer of the dermis. In the epidermis - acanthosis, focal hyper- and parakeratosis. In the center of the papule is a deep invagination of the epidermis, which closes with the transepidermal canal, which is filled with elastic fibers in the lower segment, which can be found on the surface of the epidermis. In addition to elastic fibers, dystrophically altered cells with pycnotic nuclei can be seen in the canal. In the derma around the homogeneous masses of the modified elastic fibers inflammatory infiltrates from lymphocytes, histiocytes with an admixture of giant cells of foreign bodies, as well as extravasates from erythrocytes are seen.

The histogenesis of the process is associated with changes in elastic fibers that acquire antigenic properties. D. Tsambaos and N. Berger (1980) draw attention to an increase in the epidermis and dermis of intraepithelial macrophages often in contact with mononuclear cells. Evidence of activation of humoral immunity can be the deposition of IgM, C3 and C4, found in the dermis along the elastic fibers. At the same time, the modified elastic fibers can serve as a foreign material, in response to the presence of which a corresponding reaction develops in the dermis, with reshaped elastic fibers being exposed to the surface of the skin, like a foreign body. JM Hitch et al. (1959) believe that the changes consist in the proliferation of coarse fibers that have a characteristic of elastic, eliminating through the epidermis. It is also suggested that elastoid collagen degeneration occurs, rather than hyperplasia of elastic fibers.