Elastosis perforating serpiginous elastosis: causes, symptoms, diagnosis, treatment

Elastosis perforans serpiginans (syn.: keratosis follicularis serpiginans of Lutz, elastoma intrapapillary perforans verruciformis Miescher) is a hereditary disease of connective tissue of unclear etiology, sometimes combined with Chernogubov-Ehlers-Danlos syndrome, elastic pseudoxanthoma, osteogenesis imperfecta, Marfan syndrome. Clinically manifested by the presence of figured foci, usually annular, consisting of horny papules of reddish-brown color with a depression in the center, after regression of which small atrophic scars remain. The rash is located mainly in the occipital region and neck, but can also be disseminated. An autosomal dominant type of inheritance is assumed. Sporadic cases occur. The disease may develop during long-term use of D-penicillamine.

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

The histogenesis of the process is associated with changes in elastic fibers, which acquire antigenic properties. D. Tsambaos and H. Berger (1980) draw attention to the increase in intraepidermal macrophages in the epidermis and dermis, often in contact with mononuclear elements. The deposition of IgM, C3 and C4, found in the dermis along the elastic fibers, can serve as evidence of activation of humoral immunity. At the same time, the altered elastic fibers can serve as foreign material, in response to the presence of which a corresponding reaction develops in the dermis with the release of modified elastic fibers onto the skin surface, like a foreign body. J. M. Hitch et al. (1959) believe that the changes consist of the proliferation of coarse fibers, which have the characteristics of elastic fibers, eliminated through the epidermis. It is also suggested that elastoid collagen dystrophy occurs, rather than hyperplasia of elastic fibers.