Elastosis perforans serpiginosa EPS is a rare disorder classified as a primary perforating dermatosis. That group of diseases also includes reactive perforating collagenosis, 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 Fisher , and at that time, because of the clinical presentation, this condition was classified into a group of diseases with hyperkeratosis. In , Lutz described the morphology of EPS and named it serpiginosa follicular keratosis.
|Published (Last):||23 October 2015|
|PDF File Size:||3.73 Mb|
|ePub File Size:||17.68 Mb|
|Price:||Free* [*Free Regsitration Required]|
A year-old woman presented with a 2-year history of an eruption on the anterior aspect of her neck and right arm that was relatively asymptomatic. She had a history of cystinuria, which had been treated with D-penicillamine for several years, but the D-penicillamine therapy had been discontinued 2 years before the onset of the eruption.
A biopsy was performed approximately 1 year before her presentation to our institution. The biopsy specimen demonstrated clawlike downgrowths of epidermis surrounding collections of amorphous basophilic debris and hyperplastic elastic fibers.
Many elastic fibers were noted to be pushing through epidermal channels Figure 1 , a finding that was consistent with the clinical diagnosis of elastosis perforans serpiginosa EPS.
On physical examination, she was noted to have erythematous annular and arcuate keratotic plaques on the anterior aspect of her neck and right arm Figure 2.
Case 1. Biopsy specimen demonstrating transepidermal elimination of altered elastic fibers. Patient 1 on presentation to our institution with erythematous annular plaques on her neck. A year-old woman was referred to our institution for evaluation of a prutitic "nonhealing scar" that had been present on the posterolateral aspect of the left side of her neck for 1 year. The lesions developed 1 month after a revised facial rhytidectomy scar revision. The patient had been treated with oral cephalexin for 2 weeks and topical erythromycin solution for several weeks, without improvement.
Her medical history revealed that she had been treated with D-penicillamine for Wilson disease for more than 20 years. The D-penicillamine therapy had been discontinued 2 months before her presentation to our clinic.
Physical examination revealed multiple 2- to 7-mm crusted, erythematous, ulcerated papules and plaques with an arcuate configuration. These lesions were associated with a 6-cm-long scar on the posterolateral aspect of the left side of the neck.
A biopsy specimen demonstrated a central focus of pseudoepitheliomatous hyperplasia that appeared to be connected to the epidermis by a channel of epithelium.
Adjacent inflammation with dermal necrosis, both within and surrounding the epithelia, was evident. An elastic stain demonstrated an increased concentration of elastic fibers in the middermis extending to the epidermis.
Many of the elastic fibers appeared clumped and thickened. The patient was treated unsuccessfully with cryotherapy approximately 6 times; high-potency topical corticosteroids, including clobetasol and halobetasol, for 6 weeks; topical 0. Multiple therapies have been reported to be effective in the management of EPS.
However, none has been universally accepted as the treatment of choice. Reported effective treatments include liquid nitrogen cryotherapy 1 and oral isotretinoin therapy.
Because multiple therapies had failed in both cases, the patients were offered a trial of 0. Both patients agreed and began using 0. At the 1-month follow-up visits, their disease was somewhat improved. After 2 months of tazarotene therapy, the condition of patient 1 was greatly improved Figure 3 and that of patient 2 was moderately improved. After 4 more weeks, patient 2 was almost free of active disease.
Patient 2 then discontinued tazarotene therapy, and her disease flared. Other topical retinoid preparations were then tried, without improvement. Cryotherapy was tried a few more times, also without improvement, and the patient was finally re-treated with tazarotene, which flattened her lesions within 6 weeks. Patient 1 has tried to taper her usage of tazarotene but notices flares on discontinuation. The only adverse effect observed in both cases was mild irritation.
However, the irritation subsided after a few weeks of therapy. Both patients continue to use tazarotene daily for intermittent courses when their disease flares. Elastosis perforans serpiginosa is a disorder in which altered elastic fibers are recognized as foreign material and are extruded through the epidermis by transepidermal elimination. The result is a papular eruption that is usually arranged serpiginously, annularly, or arcuately.
Many conditions are associated with EPS, including Down syndrome, Rothmund-Thomson syndrome, Ehler-Danlos syndrome, Marfan syndrome, osteogenesis imperfecta, and pseudoxanthoma elasticum.
Also, patients treated with penicillamine are prone to develop EPS. Tazarotene is the first receptor-selective topical retinoid approved for the treatment of plaque psoriasis. Hofmann et al 4 reported tazarotene's effectiveness in the treatment of congenital ichthyoses in an open, intraindividually controlled, half-side investigation.
Burkhart and Burkhart 5 reported tazarotene's effectiveness in treating a patient with Darier disease who had responded poorly to other agents.
One mechanism of action of tazarotene in psoriasis is thought to be attributable to the down-regulation of keratins 6, 10, and To our knowledge, this is the first report of EPS being successfully treated with tazarotene. The mechanism of action of tazarotene in treating EPS is unknown. Tazarotene may have comedolytic properties that allow for the unplugging of transepidermal pores in this disease. Also, the blockage of retinoic acid receptors may play a role in decreasing the proliferation in EPS.
View Large Download. Case 2. Therapeutic challenge. Patient 1 after 2 months of treatment with 0. J Am Acad Dermatol. Br J Dermatol. J Invest Dermatol. See More About Dermatology. Sign in to access your subscriptions Sign in to your personal account.
Institutional sign in: OpenAthens Shibboleth. Create a free personal account to download free article PDFs, sign up for alerts, and more. Purchase access Subscribe to the journal. Sign in to download free article PDFs Sign in to access your subscriptions Sign in to your personal account. Get free access to newly published articles Create a personal account or sign in to: 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.
Get free access to newly published articles. Create a personal account to register for email alerts with links to free full-text articles. Sign in to save your search Sign in to your personal account. Create a free personal account to access your subscriptions, sign up for alerts, and more. Purchase access Subscribe now. Purchase access Subscribe to JN Learning for one year. Sign in to customize your interests Sign in to your personal account.
Elastosis perforans serpiginosa(Elastosis Perforans Serpiginosum, Lutz-Meischer Disease)
Elastosis perforans serpiginosa EPS represents a distinct entity within the spectrum of perforating dermatoses. It is a rare disorder characterized by transepidermal elimination of altered elastic fibers. About one-quarter of cases are associated with an underlying disorder, typically of connective tissue, or with D-penicillamine use. These include Marfan syndrome, osteogenesis imperfecta, pseudoxanthoma elasticum PXE , Ehlers-Danlos syndrome type IV , Down syndrome, cutis laxa, Rothmund-Thomson syndrome, acrogeria, morphea, D-Penicillamine therapy, and scleroderma. The remaining cases are idiopathic.
Tazarotene Is an Effective Therapy for Elastosis Perforans Serpiginosa
Elastosis perforans serpiginosa is a unique perforating disorder characterized by transepidermal elimination of elastic fibers and distinctive clinical lesions , which are serpiginous in distribution and can be associated with specific diseases. Histopathology of elastosis perforans serpiginosa: Degenerated elastic fibers and transepidermal perforating canals arrow points at one of them . This condition is inherited in an autosomal dominant manner. From Wikipedia, the free encyclopedia.