Elastosis perforans serpiginosa an overview sciencedirect. Elastosis perforans serpiginosa is a disease that belongs to the group of perforating dermatoses, in which dermal elastic tissue extrusion occurs through epidermis. Elastosis perforans serpiginosa, down syndrome, and. Elastosis perforans serpiginosa eps is a rare disorder classified as a primary perforating dermatosis. 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. Closer inspection identifies material undergoing transepidermal elimination figure 2. Jan 31, 2019 dpenicillamine dpainduced pseudopseudoxanthoma elasticum pxe and elastosis perforans serpiginosa eps has been reported in the past, but most of the treatment modalities used before have a suboptimal response. About onequarter of cases are associated with an underlying disorder, typically of connective tissue. She reported a 5month history of asymptomatic cutaneous lesions on her groin and a slow progressive thickening involving the neck and axillae which started 10 years prior. Age and sex distribution druginduced elastosis perforans serpiginosa is a skin condition caused by certain medications that are administered for other underlying conditions. Cutis laxa, elastosis perforans serpiginosa, penicillamine, wilsons disease introduction elastosis perforans serpiginosa eps is a rare reactive perforating dermatosis that is characterized by the transepidermal elimination of abnormal elastic fibers1.
A biopsy showed granular cellular debris and fragmented elastic fibers surrounded by acanthotic epidermidis, configuring perforating channels. A 17yearold white boy with no underlying connective tissue disorders presented with flattopped annular plaques, with slight central atrophy on the bilateral neck. Elastosis perforans serpiginosa eps developed in three patients with wilson disease who underwent prolonged treat ment with penicillamine. Background elastosis perforans serpingosa eps is one of four classic per. A 16yearold girl with down syndrome, moyamoya disease, and history of a previous stroke presented with acute onset of left hemiparesis and elastosis perforans serpiginosa. Elastosis perforans serpiginosa als ektodermales randsymptom. Elastosis perforans serpiginosa eps is an uncommon skin disease characterized by transepidermal elimination of abnormal elastic fibers. The recent description of perforating elastosis by hitch and lund1 has clarified a diagnostic problem of long standing. The following is a brief report of a case of elastosis perforans serpiginosa of 14 years duration. Elastoma intrapapillare perforans verruciforme or keratosis follicularis serpiginosa. First described in 1953 by lntz 1, as keratosis follicularis serpiginosa, and retitled elastoma intrapapillare perforans ver. Although dpenicillamine appears to be a clear trigger for eps2, penicillamineinduced eps has been rarely reported. Elastosis perforans serpiginosa, a transepithelial.
Elastosis perforans is a distinctive dermatologie entity, presenting patches of small, keratotie, pale to deep red papnles, arranged in ares and lines, appearing most commonly on the necks of preadoleseent boys. Elastosis perforans serpiginosa may be distinguished morphologically and histologically from oth. Carver college of medicine department of dermatology 200 hawkins drive 40025 pomerantz family pavilion iowa city, ia 522421089 chair and department executive officer. Elastosis perforans serpignosa eps is such an entity. Differential diagnosis of elastosis perforans serpiginosa. Familial elastosis perforans serpiginosa jama dermatology. Elastosis perforans serpiginosa in a patient with downs syndrome. Rare skin disease in which abnormal elastic tissue fibers, other connective tissue elements and cellular debris are expelled from papillary dermis through epidermis emedicine. Elastosis perforans serpiginosa eps is a rare, distinctive perforating disease characterized by asymptomatic or pruritic keratotic papules configured in an arciform or serpiginous pattern. Elastosis perforans serpiginosa is one of four classic primary perforating disorders along with reactive perforating collagenosis, perforating folliculitis, and kyrle disease. Feb 17, 2014 elastosis perforans serpiginosa is a rare skin disease characterized by transepidermal elimination of abnormal elastic fibers. Elastosis perforans serpiginosa eps is a rare skin disorder characterized by transepidermal elimination of abnormal elastic fibres.
Elastosis perforans serpiginosa is a rare skin disease characterized by transepidermal elimination of abnormal elastic fibers. 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. There are 3 clinical variants of elastosis perforans serpiginosa. Elastosis perforans serpiginosa jama pediatrics jama network. Low power view of intrapapillary and intradermal increase of verhoeff positive fibers. Druginduced elastosis perforans serpiginosa bmj case reports.
That group of diseases also includes reactive perforating. He was diagnosed with elastosis perforans serpiginosa on tissue biopsy and underwent pulse dye laser therapy over four years with excellent results. Dpenicillamineinduced pseudopseudoxanthoma elasticum and. Penicillamineinduced elastosis perforans serpiginosa in. Dermoscopy and direct immunofluorescence findings of. Elastosis perforans serpiginosa is a rare degenerative skin disease characterized by a transepidermal elimination of elastic fiber aggregates 18. Elastosis perforans serpiginosa in a patient with down syndrome and moyamoya disease is a novel finding. If a drug is the cause then cessation of the drug can result in more rapid resolution of the condition. Penicillamine, a copper chelator, impairs the activity of this enzyme. Who gets druginduced elastosis perforans serpiginosa. Low power of histology of elastosis perforans serpiginosa demonstrates a column of keratotic debris forming a focal invagination through a hyperplastic epidermis figure 1.
When submitting mnemonics, be sure to utilize bold and different text color to emphasize important letters or phrases within your mnemonic. Elastosis perforans serpiginosa is a reactive perforating dermatosis in which elastic fibers are extruded through the epidermis. An increased prevalence of common skin conditions and other peculiar and rare dermatosis such as elastosis perforans serpiginosa has been described in down. Although dpenicillamine appears to be a clear trigger for eps2. Elastosis perforans serpiginosa an overview sciencedirect topics. It is a chronic dermatosis characterised by extrusion of elastic.
Elastosis perforans serpiginosa, down syndrome, and moyamoya. Of every possible solution for elastosis perforans. Lysyloxidase is a copperdependent enzyme required for dermal elastic fibre crosslinking. Druginduced elastosis perforans serpiginosa bmj case. Pulse dye laser therapy successful for elastosis perforans. Retrogressive change in cutaneous connective tissue resulting in excessive amounts of material which give the. Elastosis perforans serpiginosa eps is an uncommon cutaneous disorder classified under perforating diseases pd. Four types of elastosis perforans serpiginosa eps have been described in literature. Elastosis perforans serpiginosa pdf elastosis perforans serpiginosa eps is a rare disorder classified as a primary perforating dermatosis. Elastosis perforans serpiginosa occurred in a 15yearold caucasian male treated with penicillamine 20 mgkgday for 3 years 42a. This is a secondary phenomenon not uncommonly encountered in lesions of prurigo nodularis. Eps present as asymptomatic to pruritic groups of keratotic papules coalescing in a arcuate serpiginous distribution. We describe a case of elastosis perforans serpiginosa and its successful management with pdl laser. Elastosis perforans serpiginosa eps is a rare skin disorder seen during adulthood.
Pathology of elastosis perforans serpiginosa 10 important. Prognosis, clinical course, and treatment the perforating disorders are difficult to treat. Elastosis perforans serpiginosa eps represents a distinct entity within the spectrum of perforating dermatoses. Penicillamineinduced elastosis perforans serpiginosa and. Elastosis perforans serpiginosa is a rare, primary perforating dermatosis. Cutaneous lesions appeared on the patients neck and right arm. The code is valid for the year 2020 for the submission of hipaacovered transactions. Although lesions may resolve spontaneously, often with scars, they are usually long. Penicillamineinduced elastosis perforans serpiginosa in wil. Elastosis perforans serpiginosum accessed 27 august 2018. Elastosis perforans serpiginosa article about elastosis. Results from histopathology revealed changes consistent with elastosis perforans serpiginosa eps.
What is elastosis perforans serpiginosa 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. 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 pdf datsumo sapporo. Elastosis perforans serpiginosa is a rare complication of penicillamine therapy for wilsons disease and might also be seen in association with systemic diseases such as ehlers danlos and downs syndrome. Our results show that pulse dye laser therapy is a safe and effective treatment for elastosis perforans serpiginosa. Elastosis perforans serpiginosa eps is a rare skin condition included among the primary perforating dermatoses of dermal origin. Two cases of dpenicillamineinduced elastosis perforans. Three eps subtypes have been reported that differ according to aetiology, associated diseases, and histopathological features. Elastosis perforans serpiginosa is a rare chronic dermatosis characterized by extrusion of altered elastic fibers through the e pidermis. Elastosis perforans serpiginosa genetic and rare diseases. We report a case of dpainduced pseudopxe with extensive eps who had an excellent rapid response to acitretin. This process is known as transepithelial elimination, an unusual method for removing foreign debris from the skin when the body. John libbey eurotext european journal of dermatology. Read more about symptoms of elastosis perforans serpiginosa.
It is a rare condition characterised by transepidermal elimination of elastic fibre aggregates. Report of case a white woman, age 22 years, a mongoloid, was first seen at. It often occurs in association with a variety of connective tissue disorders, and may develop following penicillamine therapy. Treatment of elastosis perforans serpiginosa using a fractional carbon dioxide laser. The topic keratosis follicularis et parafollicularis serpiginosa you are seeking is a synonym, or alternative name, or is closely related to the medical condition elastosis perforans serpiginosa. Jun 19, 2019 elastosis perforans serpiginosa eps is a rare disorder classified as a primary perforating dermatosis. A rare skin condition characterized by the development of small, usually red, bumps on the skin. Keratosis follicularis et parafollicularis serpiginosa. Nov 14, 2018 elastosis perforans serpiginosa eps is an uncommon cutaneous disorder classified under perforating diseases pd. Elastosis perforans serpiginosa definition of elastosis. It takes place within the primary perforating dermatoses and may be seen associated with hereditary disorders of.
Elastosis perforans serpiginosaelastosis perforans. Elastosis perforans serpiginosa in a patient with wilsons. Although dpenicillamine appears to be a clear trigger for elastosis perforans serpiginosa, dpenicillamineinduced disease has been rarely reported. Elastosis perforans serpiginosa is an infrequent skin dermatosis of unknown aetiology characterized by the transepidermal elimination of abnormal elastic fibres. It is found in the 2020 version of the icd10 clinical modification cm and can be used in all hipaacovered transactions from oct 01, 2019 sep 30, 2020. Elastosis perforans serpiginosa eps is a rare skin condition of unknown aetiology characterized by the transepidermal elimination of abnormal elastic. 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. In the present article, we present a wd adolescent with elastosis perforans serpiginosa eps, induced by penicillamine, which did not disappear following penicillamine withdrawal and persisted unchanged on zinc monotherapy during 3 years of observation. There have been a number of case reports of elastosis perforans serpiginosa in the setting of penicillamine therapy for both. Development of elastosis perforans serpiginosa in association with progressive vasoocclusive disease may be a biological marker of progression of. Lysyloxidase is a copperdependent enzyme required for dermal elastic. Our results show that pulse dye laser therapy is a safe and ezective treatment for elastosis perforans serpiginosa. Images in druginduced elastosis perforans serpiginosa. Elastosis perforans serpiginosa in a patient with wilsons disease previous article the role of the gut microbiota in sepsis a 31yearold man with wilsons disease presented with a pruritic rash across the neck of 12 months duration.
Elastosis perforans serpiginosa eps is a rare reactive perforating dermatosis that is characterized by the transepidermal elimination of abnormal elastic fibers1. Acquired perforating dermatosis perforating dermatoses. There have been a number of case reports of elastosis perforans serpiginosa in the setting of penicillamine therapy for both wilsons disease and systemic sclerosis. A 15yearold male presented with a history of itchy, raised, red and unsightly lesions on the back of his neck. The idiopathic form of eps occurs rarely in children. It has been reported to occur in wilsons disease and cystinuria, usually after many years of highdose therapy.
Elastosis perforans serpiginosa in a patient with downs. Elastosis perforans serpiginosa and pseudopseudoxanthoma elasticum were diagnosed in a 57yearold woman treated with dpenicillamine for 25 years 43 a. Elastosis perforans serpiginosa symptoms, diagnosis. Elastosis perforans serpiginosa is a form of perforating dermatoses, which has a characteristic clinical presentation of grouped keratotic papules coalescing into serpiginous or annular. Elastosis perforans serpiginosa american osteopathic. A skin eruption serves its greatest purpose when it signals an association with systemic disease. Development of elastosis perforans serpiginosa in association with progressive vasoocclusive disease may be a. Elastosis perforans serpiginosum accessed 27 august 2018 back of neck in teenage boys. Find out information about elastosis perforans serpiginosa. It is a rare disorder characterized by transepidermal elimination of altered elastic fibers. A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for elastosis perforans serpiginosa. Elastosis perforans serpiginosa eps is an uncommon and chronic dermatosis characterized by the transepidermal elimination of altered elastic fibers originating in the dermis.
Pubmed is a searchable database of medical literature and lists journal articles that discuss elastosis perforans serpiginosa. Pdf on oct 1, 2016, adriana polanska and others published elastosis perforans serpiginosa. Pdf elastosis perforans serpiginosa is a rare skin disease characterized by transepidermal elimination of abnormal elastic fibers. 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 perforans serpiginosa department of dermatology.
Pathology outlines elastosis perforans serpiginosum. It is all the more valuable if it is clinically distinctive and histologically pathognomonic. Elastosis perforans serpiginosa pathology dermnet nz. 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. Elastosis perforans serpiginosa eps is rare disease characterized by hyperkeratotic papules, transepidermal elimination of abnormal elastic fibers and focal dermal elastosis. Click on the link to view a sample search on this topic. This dermatoses was not recognized until 1953 when. 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. Development of elastosis perforans serpiginosa in association with progressive vasoocclusive disease may be a biological marker of progression of moyamoya disease.
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