Elastosis perforans serpiginosa (EPS) is a rare disorder classified as a primary perforating dermatosis. That group of diseases also includes. Four types of elastosis perforans serpiginosa (EPS) have been described in literature: 1) idiopathic EPS, 2) reactive perforating elastosis. Elastosis perforans serpiginosa (EPS) is a rare dermatologic condition where connective and elastic tissues are viewed as foreign objects and subsequently.

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After 4 more weeks, patient 2 was almost free of active disease.

The “lumpy-bumpy” elastic fiber. Elastosis perforans serpiginosa — Medscape Reference Books: The most prominent histopathological findings in patients with EPS are localized segments of hyperplastic epidermis where basophilic nuclear debris and brightly eosinophilic fragmented elastic fibers are being eliminated through transepidermal channels that extend upward from the dermis in straight or corkscrew patterns 1. 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 [ 12 ].

The lesions tend to subside serpiginosaa the center and often solitary satellite lesions in the close vicinity are detected. Other topical retinoid preparations were then tried, without improvement.

Also, the blockage of retinoic acid receptors may play a role in decreasing the proliferation in EPS. Sign in to save your search Sign in to your personal account.

Penicillamine-induced elastosis of the mucosal lip. Patient 2 then discontinued tazarotene therapy, and her disease flared. D ICD – J Am Acad Dermatol. Sign in to customize your interests Sign in to your personal account.

Characteristic for EPS skin lesions within face A and left upper extremity B, C — papular keratotic lesions fuced together in serpiginous arrangement. Our website uses cookies to enhance your experience.

Elastosis Perforans Serpiginosa

srrpiginosa Open in a separate window. Report of a case and review of the literature. Get free access to newly published articles. Open in a separate window. Also, patients treated serpiiginosa penicillamine are prone to develop EPS. Elastosis perforans serpiginosa EPS is a rare skin disorder in which abnormal elastic tissue fibre passes from the papillary dermis inner layer of skin to the epidermis outer layer of skin.


A biopsy specimen demonstrated a central focus of pseudoepitheliomatous hyperplasia that appeared to be connected to the epidermis by a channel of epithelium. Perforrans laser 16and carbon dioxide laser therapy 15 Response of elastosis perforans serpiginosa to pulsed CO2, Er: However, it remains unclear whether the relationship between these conditions and EPS is causal or coincidental, as EPS also commonly occurs in individuals without any underlying disease.

We report an additional case of this rare cutaneous condition wlastosis review EPS cases described in the Korean literature.

In these cases, elastin synthesis may be affected either directly by penicillamine or via a localized copper deficiency, ultimately inducing the serpiyinosa of abnormal elastic fibers that are transepidermally eliminated from the dermis.

Though numerous treatment modalities have been described in the literature, most are not very effective. Histopathology of the Skin. The patient had been treated with oral cephalexin for 2 weeks and topical erythromycin solution for several weeks, without improvement.

In addition, discontinuing penicillamine therapy does not guarantee preventing further development of EPS lesions in patients undergoing penicillamine therapy 15 Elastosis perforans serpiginosa and associated disorders. Expression of the rlastosis elastin receptor in perforating skin disorders. YAG laser, are reported to induce complete clearing of EPS lesions, but another report showed only mild improvement of EPS lesions and subtle atrophic scarring after carbon dioxide laser treatment In cases of penicillamine-induced EPS, calcification can be observed between the collagen fibers [ 1 — 4 ].

Saxena M, Tope WD. 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 ]. Multiple side effects of penicillamine therapy in perflrans patient with rheumatoid arthritis.

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However, others, like Saxena did not show a positive response to numerous sessions using these lasers, and in turn, CO 2 laser caused scarring [ 16 ]. Received Jan 15; Accepted Feb Create a free personal account to download free article PDFs, sign up for alerts, and more.

Lever’s histopathology of the skin. A biopsy was performed approximately 1 year before her presentation to our institution. Please review our privacy policy.

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

Many elastic fibers were noted to be pushing through epidermal channels Figure 1a finding that was consistent with the clinical diagnosis of elastosis perforans serpiginosa EPS. To our knowledge, this is the first report of EPS being successfully treated with tazarotene.

Elastin peptides induce migration and terminal differentiation of cultured keratinocytes via 67 kDa elastin receptor in vitro: Imiquimod therapy for elastosis perforans serpiginosa. Privacy Policy Terms of Use. Register for email alerts with links to free full-text articles Access PDFs of free articles Manage your interests Save searches and receive search alerts. Previous treatments with topical antifungal and topical steroid were ineffective.

To confirm the clinical diagnosis, the biopsy was taken from the primary circinate lesion of the cheek.

Elastosis perforans serpiginosa | DermNet NZ

An elastic stain demonstrated an increased concentration of elastic fibers in the middermis extending to the epidermis. Treatment of elastosis perforans serpiginosa with the pinhole method using a carbon dioxide laser. Find articles by Yuri Choi.