Elastosis perforans serpiginosa eps represents a distinct entity within the spectrum of perforating dermatoses. Broad areas of epidermal erosion with adjacent epidermal hyperplasia. 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, described as transepithelial elimination. Treatment has included various modalities and cryotherapy is one of the effective options. Elastosis perforans serpiginosa eps is a rare dermatologic condition where connective and elastic tissues are viewed as foreign objects and subsequently. What is the prognosis of elastosis perforans serpiginosa. In idiopathic elastosis perforans serpiginosa, the cause and risk factors for the condition remain unknown. The list of treatments mentioned in various sources for elastosis perforans serpiginosa includes the following list. It is a rare disorder characterized by transepidermal elimination of altered elastic fibers. Elastosis definition of elastosis by the free dictionary. Feb 17, 2014 elastosis perforans serpiginosa is a rare skin disease characterized by transepidermal elimination of abnormal elastic fibers. These syndromes are differentiated into four subtypes. It has been reported to occur in wilsons disease and cystinuria, usually after many years of highdose therapy.
Elastosis perforans serpiginosum accessed 27 august 2018 back of neck in teenage boys. Eps is defined as elastosis perforans serpiginosa somewhat frequently. Elastosis perforans serpiginosa symptoms, diagnosis. Elastosis perforans serpiginosa with a history of familial involvement. Secondary lesions are vague scars or areas of roughness and hypopigmentation or hyperpigmentation that result after the primary lesions have subsided and disappeared. Elastosis perforans serpiginosaelastosis perforans. Elastosis perforans serpiginosa genetic and rare diseases.
This process is known as transepithelial elimination, an unusual method for removing foreign debris from the skin when the body. Elastosis perforans serpiginosa eps is a rare skin disease in which abnormal elastic tissue fibers, other connective tissue elements, and cellular debris are expelled from the papillary dermis via. Elastosis perforans serpiginosa usually is asymptomatic but can be pruritic. Linear focal elastosis is an uncommon dermal elastosis that usually affects the back or trunk but can involve the thighs, arms and breast. Retrogressive change in cutaneous connective tissue resulting in excessive amounts of material which give the. Elastosis perforans serpiginosa, transepidermal elimination, co2 laser. Elastosis medical definition merriamwebster medical dictionary. How is elastosis perforans serpiginosa abbreviated.
A rare acquired dermis elastic tissue disorder with increased elastic tissue characterized by focal dermal elastosis and transepidermal elimination of abnormal elastic fibers, presenting as small keratotic papules or plaques arranged in groups in serpiginous or annular patterns on the neck, face, and arms, while other areas are less frequently affected. Elastosis perforans serpiginosa eps is a form of perforating dermatoses, a group of cutaneous syndromes that are characterized by transepidermal elimination of connective tissue components. This condition classically presents as small papules arranged in serpiginous or annular patterns on the neck, face, arms, or other flexural areas. Elastosis perforans serpiginosa in an unusual location in a patient with downas syndrome abstract. A rare skin condition characterized by the development of small, usually red, bumps on the skin. Elastosis perforans serpiginosa is an uncommon and chronic dermatosis characterized by transepidermal elimination of abnormal elastic fibers originating in the dermis. Elastosis perforans serpiginosa has also been thought to be resultant of an injured epidermal barrier. Eps is characterized by the presence of multiple tiny papules on the neck or face in a winding pattern. Elastosis perforans serpiginosa disease definition a rare acquired dermis elastic tissue disorder with increased elastic tissue characterized by focal dermal elastosis and transepidermal elimination of abnormal elastic fibers, presenting as small keratotic papules or plaques arranged in groups in serpiginous or annular patterns on the neck. If you would like a large, unwatermarked image for your web page or.
Elastosis perforans serpiginosa also known as perforating elastosis omim 100 presents as small papules, either grouped or in a circinate or serpiginous arrangement, on the neck, upper extremities, upper trunk, or face. Elastosis perforans serpiginosa 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. It is a very rare skin condition and this is a community were people can share their experience with it, ask questions and offer advice. Pathology outlines elastosis perforans serpiginosum. Elastosis definition of elastosis by medical dictionary. Mar 06, 2019 elastosis perforans serpiginosa eps is a rare skin disease in which abnormal elastic tissue fibers, other connective tissue elements, and cellular debris are expelled from the papillary dermis through the epidermis transepithelial elimination. Elastosis is a condition that increases with age and is most commonly associated with fairskinned women who have had sunburn on a regular basis. This is a secondary phenomenon not uncommonly encountered in lesions of prurigo nodularis. Always seek professional medical advice about any treatment or change in treatment plans. A compromised epidermal barrier or a dermal response to chronic trauma may contribute to the development of eps. It presents as asymptomatic, palpable or atrophic, yellow lines or striae stretch marks elastosis perforans serpiginosa. If you would like a large, unwatermarked image for your web page or blog, please purchase the appropriate license. Nodular elastosis article about nodular elastosis by the.
Elastosis perforans serpiginosa eps is a rare dermatologic condition where connective and elastic tissues are viewed as foreign objects and subsequently expelled from the papillary dermis through the epidermis. The three general categories of elastosis perforans serpiginosa are idiopathic, reactive, and drug. Elastosis perforante serpiginosa en pacientes con sindrome. Elastosis perforans serpiginosa eps is a rare skin disorder in which abnormal elastic tissue passes from. It is also dependent upon the underlying health condition, if any, associated with it. Outcomesresolutions the prognosis of elastosis perforans serpiginosa depends upon the severity of the condition. Sep 05, 2018 what is the prognosis of elastosis perforans serpiginosa. Diagnosis is based on clinical and histopathologic aspects. Elastosis perforans serpiginosa eps is a rare skin disorder in which abnormal elastic tissue passes from the papillary dermis to the epidermis, appearing as small groups of red bumps often in a snakelike pattern. This code description may also have includes, excludes, notes, guidelines, examples and other information. Apr 29, 2016 elastosis perforans serpiginosa is an uncommon and chronic dermatosis characterized by transepidermal elimination of abnormal elastic fibers originating in the dermis. Summary elastosis perforans serpiginosa is a rare chronic dermatosis characterized by extrusion of altered elastic fibers through the e pidermis.
Dermis elastosis perforante serpiginosa information on the. 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. Elastosis perforans serpiginosa eps is a rare disorder classified as a primary perforating dermatosis. Jul 01, 2011 rare skin disease in which abnormal elastic tissue fibers, other connective tissue elements and cellular debris are expelled from papillary dermis through epidermis emedicine. He named the lesion keratosis follieularis serpiginosa.
The cause of abnormal elastic tissue is unknown but may be from a genetic mutation or external factors such as penicillamine. Pdf elastosis perforans serpiginosa is a rare skin disease characterized by transepidermal elimination of abnormal elastic fibers. Elastosis perforans serpiginosa in association with. Dermis elastosis perforante serpiginosa information on.
You may not embed one of our images on your web page without a link back to our site. Rare skin disease in which abnormal elastic tissue fibers, other connective tissue elements and cellular debris are expelled from papillary dermis through epidermis emedicine. Since it was first described by lutz in 1953, eps is. Treatment list for elastosis perforans serpiginosa. Elastosis perforans serpiginosa eps is a rare skin disease in which abnormal elastic tissue fibers, other connective tissue elements, and cellular debris are expelled from the papillary dermis via transepithelial elimination. Elastosis perforans serpiginosa, therapeutics introduction elastosis perforans serpiginosa eps is a rare skin condition characterized by hyperkeratotic papules, transepidermal elimination of abnormal elastic fibers, and focal dermal elastosis. Elastosis perforans serpiginosa is a rare skin disease characterized by transepidermal elimination of abnormal elastic fibers. Elastosis perforans serpiginosa is a rare complication of dpenicillamine therapy.
Idiopathic elastosis perforans serpiginosa dovemed. Abnormal skin fibre tissue forms from the inner skin layer to the outer skin layer which in turn causes the top skin layer to respond to it as if it were a foreign particle i. Elastosis medical definition merriamwebster medical. About onequarter of cases are associated with an underlying disorder, typically of connective tissue, or with dpenicillamine use.
Elastosis perforans serpiginosum accessed 27 august 2018. Pero tambien sufren dermatosis peculiares como siringomas, calcicosis idiopatica tipo milia y elastosis perforante serpiginosa. Elastosis perforans serpiginosa pathology dermnet nz. Elastosis perforans serpiginosa synonyms elastoma intrapapillare perforans verruciforme keratosis follicularis serpiginosa serpiginous perforating elastosis perforating elastoma elastoma intrapapillare perforans presentation stereotypical age and sex most common during the second decade of life and in males mostly distribution of lesions symmetric, neck and upper extremities chiefly, but face. Always seek professional medical advice about any treatment or change in treatment. Elastosis perforans serpiginosa american osteopathic college of. Before any professional treatment, the patient must adhere to preventive measures against sun damage. Eps is characterized by the presence of multiple tiny. Elastosis perforans serpiginosa in association with scabies mite. Differential diagnosis of elastosis perforans serpiginosa. Report of a family with a chromosomal investigation.