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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Infobox medical condition new All stub articles. Published online Oct Isolated reports also indicate a possible association between EPS and Wilson’s disease and cystinuria, particularly in patients on long-term penicillamine therapy 510 – EPS occurs because the epidermis the outer layer of the skin perceives abnormal elastic tissue to be a foreign object, thus responding through an inflammatory attack.
Both patients agreed and began using 0. 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. Indian Dermatol Online J. Author information Article notes Copyright and License information Disclaimer. A punch biopsy specimen revealed focal epidermal hyperplasia with the formation of a narrow vertical transepidermal channel containing nuclear debris and eosinophilic degenerated elastic fibers Fig.
Report of a case and review of the literature. D-penicillamine-induced elastosis perforans serpiginosa in a child with juvenile rheumatoid arthritis. What is standard of care in the evaluation of elastosis perforans serpiginosa? Corresponding author and reprints: Copyright American Medical Association.
Treatment with topical 0. J Am Acad Dermatol ; The first one, known as reactive one, is associated elaastosis connective tissue diseases, such as Ehlers-Danlos syndrome type IVcutis laxa, Marfan syndrome, acrogeria, Rothmund-Thomson syndrome, osteogenesis imperfecta and pseudoxanthoma elasticum [ 3 — 6 ]. Treatment of elastosis perforans serpiginosa with the flashlamp pulsed dye laser.
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J Am Acad Dermatol. To confirm the clinical diagnosis, the biopsy was taken from the primary circinate lesion of the cheek. Elastic fibers perforate through the epidermis in patients with EPS. Get free access to newly published articles Create a personal account or sign in to: 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.
Increased elastic tissue and solar elastotic syndromes. Perforating diseases are histologically characterized by transepidermal elimination of dermal substances.
In these cases, elastin synthesis may be affected either directly by penicillamine or via a localized copper deficiency, ultimately inducing the production of abnormal elastic fibers that are transepidermally eliminated from the dermis.
Lichen sclerosus Anetoderma Schweninger—Buzzi anetoderma Jadassohn—Pellizzari anetoderma Atrophoderma of Pasini and Serpigonosa Acrodermatitis chronica atrophicans Semicircular lipoatrophy Follicular atrophoderma Linear atrophoderma of Moulin.
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 ]. In the transepidermal channel, and particularly in its lower part, basophilic degeneration mass consisting of keratinocytes, remainings of crumbled inflammatory cells and elastic fibers can be detected.
Elastosis perforans serpiginosa EPS is a rare skin condition characterized by hyperkeratotic papules, transepidermal elimination of abnormal elastic fibers, and focal dermal elastosis. Numerous treatment modalities have been described, including dry ice 8cellophane tape stripping 813electrodessication and curettage 14cryotherapy 13 – 15intralesional and topical corticosteroid therapy 1516topical calcipotriol 14topical tretinoin 141517oral isotretinoin 1415topical tazarotene 15topical imiquimod 18topical glycolic or salicylic acid therapy 1417narrow band ultraviolet B radiation 14pulsed dye laser, Er: In our case, we serpigijosa treatment with topical 0.
Purchase access Subscribe to the journal. 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. However, others, like Saxena did not show a perforns response to numerous sessions using these lasers, and in turn, CO 2 laser caused scarring [ 16 ]. These papules first appeared 2 years ago and leastosis increased in number.
The second form of the disease is a drug-induced one, which is caused by treatment with D-penicillamine. Usually it spontaneously resolves without complications after a few years.
In cases of EPS associated with penicillamine therapy, the characteristic skin lesions typically develop within the first year of treatment and can be histologically distinguished from the other forms of EPS by the presence of calcium deposits in elastlsis elastic fibers 7.