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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Physical examination revealed multiple 2- to 7-mm crusted, erythematous, ulcerated papules and plaques with an arcuate configuration. Exfoliating erythematous papular lesions with a diameter of a few millimeters within the skin of the left cheek and upper and lower limbs were observed.
InMiescher showed that the extruded material contains elastin and suggested the term elastomer intrapapillare perforans veruciformewhich in pegforans, Dammert and Putkonem changed into EPS [ 12 ]. Other topical retinoid preparations were then tried, without improvement.
Such a case of EPS associated with penicillamine therapy was reported in the Korean literature 5. Disorders of connective tissue. The diagnosis of elastosis perforans serpiginosa is made by its clinical appearance and by the characteristic histology changes seen on skin biopsyshowing transepidermal elimination of elastic tissue. J Am Acad Dermatol. The last subtype, idiopathic one, may be associated with genetic basis of the autosomal dominant type of inheritance. Laser treatment, including carbon dioxide laser and Er: 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 Seborrheic keratosis Clonal seborrheic keratosis Common seborrheic keratosis Irritated seborrheic keratosis Seborrheic keratosis with squamous atypia Reticulated seborrheic keratosis Dermatosis papulosa nigra Keratosis punctata of the palmar creases other hyperkeratosis: However, to date, the candidate gene has not been identified [ percorans8 ].
Table 1 summarizes the clinical data from four cases of EPS in the Korean literature in addition to our case. Create a personal account to register for email alerts with links to free full-text articles. Tazarotene is an effective therapy for elastosis perforans serpiginosa.
Some researchers have demonstrated the effectiveness of the laser light, like Abdullah et al. Psoriasis and Down syndrome: Werpiginosa in to download free article PDFs Sign in to perforwns your subscriptions Sign in to your personal account. Furthermore, in this group of patients seborrhoeic dermatitis, skin dryness or the presence of hand and feet hyperkeratosis are the most common skin comorbidities.
Patient 1 after 2 months of treatment with 0. Click on image for details. D ICD – The pinhole method using a carbon dioxide laser showed complete clearing without post-treatment scarring D-penicillamine, elastosis perforans serpiginosa, lumpy-bumpy elastic fibers. pperforans
Elastosis Perforans Serpiginosa
DermNet NZ does not provide an online consultation service. The epidermis in the vicinity of the channel is thicker and covers a tunnel in the shape of pincers. An elastic stain demonstrated an increased concentration of elastic fibers in the middermis extending to the epidermis. Response of elastosis perforans serpiginosa to pulsed CO2, Er: We report petforans case of D-penicillamine induced widespread alteration in skin elastic tissue with distinct histopathologic features.
Find articles by Si-Hyung Lee. Moreover, even though transepidermal elimination can occur in any of the three other perforating diseases, the elastic tissue changes present in EPS are virtually pathognomonic. However, none has been universally accepted as the treatment of choice.
Elastosis perforans serpiginosa: a review of the literature and our own experience
In addition, discontinuing penicillamine therapy does not guarantee preventing further development of EPS lesions in patients undergoing penicillamine therapy 15 Cutaneous keratosis, ulcer, atrophy, and necrobiosis L82—L94— The fibers are compacted, twisted and fragmented, what can be clearly seen in the staining for elastic fibers such as in van Gieson staining.
Fitzpatrick’s Dermatology in General Medicine. 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. Support Center Support Center. 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 ]. Acquired disorders of elastic tissue: Acquired disorders of elastic tissue: A biopsy specimen demonstrated a central focus of pseudoepitheliomatous hyperplasia that appeared to be connected to the epidermis by a channel of epithelium.
Since it was first described by Lutz inEPS is one of four classic primary perforating disorders along with reactive perforating collagenosis, perforating folliculitis, and Kyrle disease 1. The first one, known as reactive one, is associated with connective tissue diseases, such as Ehlers-Danlos syndrome type IVcutis laxa, Marfan syndrome, acrogeria, Rothmund-Thomson syndrome, osteogenesis imperfecta and pseudoxanthoma elasticum [ 3 — 6 ].
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. In addition, they are mostly single, and the presence of numerous scattered plaques is atypical.
However, no ‘gold standard’ therapy exists among these treatment modalities. Sign in to customize your interests Sign in to your personal account.
Open in a separate window. Kyrle disease Reactive perforating collagenosis Elastosis perforans serpiginosa Perforating folliculitis Acquired perforating dermatosis. Pergorans side effects of penicillamine therapy in one patient with rheumatoid arthritis.
Biopsy specimen demonstrating transepidermal elimination of altered elastic fibers. A summary of the 5 cases of elastosis perforance serpiginosa reported in the Korean literature including our case.