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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Though numerous treatment modalities have been described in the literature, most are not very effective.
Elastosis Perforans Serpiginosa
Sign in to customize your interests Sign in to your personal account. Expression of the kDa elastin receptor in perforating skin disorders. In addition, the patient was suffering from hypothyroidism, tetralogy of Fallot and bilateral congenital dislocation of the hip joints. How to cite perdorans article: Many of the elastic fibers appeared clumped and thickened.
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However, no ‘gold standard’ therapy exists among these treatment modalities. Etiology and pathogenesis of EPS has not yet been fully understood. These serpgiinosa first appeared 2 years ago and slowly increased in number. None, Conflict of Interest: One mechanism of action of tazarotene in psoriasis is thought to be attributable to the down-regulation of keratins 6, 10, and Topical imiquimod and topical tazarotene therapy is reported to improve EPS skin lesions 15 Although lesions serpigknosa spontaneously resolve, they often persist for longer periods.
Many conditions are associated with EPS, including Down syndrome, Rothmund-Thomson syndrome, Ehler-Danlos syndrome, Marfan syndrome, osteogenesis imperfecta, perforsns pseudoxanthoma elasticum. Usually there are no symptoms but sometimes EPS is itchy. J Laryngol Otol ; Lichen sclerosus Anetoderma Schweninger—Buzzi anetoderma Jadassohn—Pellizzari anetoderma Atrophoderma of Pasini and Pierini Acrodermatitis chronica atrophicans Semicircular lipoatrophy Follicular atrophoderma Linear atrophoderma of Moulin.
Journal List Postepy Dermatol Alergol v. This page was last edited on 2 Mayat Elastosis perforans serpiginosa with simultaneous onset in two sisters. Sign in to make a comment Sign in to your personal account. A survey of pediatric dermatologists. Indian J Dermatol ; Oxford Textbook of Medicine. You can help Wikipedia by expanding it.
Her medical history revealed that she had been treated with D-penicillamine for Wilson disease for more than 20 years.
Elastosis perforans serpiginosa
Elastosis perforans serpiginosa is a unique perforating disorder characterized by transepidermal elimination of elastic fibers and distinctive clinical lesionswhich are serpiginous in distribution and can be associated with specific diseases. 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. Am J Dermatopathol ;3: We can distinguish three subtypes of EPS. There is no effective standard treatment of EPS, and so far there have been only single reports of the use of different drugs for systemic and topical therapy [ 2 ].
D-penicillamine, elastosis perforans serpiginosa, lumpy-bumpy elastic fibers. The biopsy specimen demonstrated clawlike downgrowths of epidermis surrounding collections of amorphous basophilic debris and hyperplastic elastic fibers.
This article has been cited by. Gomori methenamine silver stain and D-PAS stain were both negative for fungal elements. Sign in to save your search Sign in to your personal account. J Am Acad Dermatol.
Burkhart and Burkhart 5 reported tazarotene’s effectiveness in treating a patient with Darier disease who had responded poorly to other agents. Support Center Support Center. The channel opens into a bowl-shaped cavity filled with horny plug. Familial elastosis perforans serpiginosa. Penicillamine-induced elastosis of the mucosal lip.