Elastosis perforans serpiginosa: a review of the literature and our own experience

Elastosis perforans serpiginosa (EPS) is a rare disorder classified as a primary perforating dermatosis. That group of diseases also includes reactive perforating col-lagenosis, perforating folliculitis or Kyrle disease. In EPS, protrusions of altered elastic fibers and other connective tissue material through the papillary layer and epidermis (called transepithelial elimination) clinically manifest as small papules arranged in an annular pattern [1, 2]. The first description of EPS was most likely published in 1927 (Fisher), and at that time, because of the clinical presentation, this condition was classified into a group of diseases with hyperkeratosis. In 1953, Lutz described the morphology of EPS and named it serpiginosa follicular keratosis. In 1955, Miescher showed that the extruded material contains elastin and suggested the term elasto-mer intrapapillare perforans veruciforme, which in 1958, Dammert and Putkonem changed into EPS [1, 2]. Elastosis perforans serpiginosa is considered as mild dermatosis, which is not accompanied by involvement of other organs [2]. The disease prevalence has not been clearly specified so far but this is not a condition commonly found in routine clinical practice. It is believed that 90% of EPS cases are patients before the age of 30, although 5-year-old and 89-year-old patients have been reported in the literature. Elastosis perforans serpiginosa more often affects men and only 25% of cases were detected among women [2, 3]. In the course of EPS, the presence of erythematous as well as skin-colored papules or plaques with keratotic surface which are grouped together and form serpigi-nous or annular lesions is usually observed. The lesions tend to subside in the center and often solitary satellite lesions in the close vicinity are detected. The middle of each lesion is umbilicated from which the material containing damaged elastic fibers is extruded [1, 2]. With the exception of EPS occurring in the course of Down's syndrome and penicillamine treatment, skin lesions are usually symmetrical. In addition, they are mostly single, and the presence of numerous scattered plaques is atypi-cal. The lesions are mostly located on the arms, face and neck. There is a slow course of the disease and in some cases a spontaneous regression of lesions in the period from 6 months to 5 years with a possibility of scarring may appear [1–4]. Figures 1 A–C present typical clinical presentation of EPS. We can distinguish three subtypes of EPS. The first one, known as reactive one, is associated with connective tissue diseases, such …