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. Verhoeffvan gieson, acid orceingiemsa, or aldehyde fuchsin. The patient reported that she had experienced chronic diarrhea and abdominal. All but one case is reported as occurring since 1950. 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 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.
Carver college of medicine department of dermatology 200 hawkins drive. Elastoma intrapapillare perforans verruciforme or keratosis follicularis serpiginosa. Jul 31, 20 dermatopathology reference describes elastosis perforans serpiginosa histopathology including histologic features and provides links to additional medical references. 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 elastosis perforans serpiginosa catterall, m. Some associated genetic conditions include down syndrome, pseudoxanthoma elasticum, marfan syndrome, ehlersdanlos syndrome, acrogeria, osteogenesis imperfecta, rothmundthomson syndrome, and moyamoya disease.
Elastosis perforans serpiginosum accessed 27 august 2018. 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 may be distinguished morphologically and histologically from oth. 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 occurs in the marfan syndrome, the ehlersdanlos syndrome, osteogenesis imperfecta, pseudoxanthoma elasticum, and down syndrome. Elastosis perforans serpiginosa is a reactive perforating dermatosis in which elastic fibers are extruded through the epidermis. Low power of histology of elastosis perforans serpiginosa demonstrates a column of keratotic debris forming a focal invagination through a hyperplastic epidermis figure 1. Amyloidosis amyloidosis is a clinical disorder caused by extracellular deposition of insoluble abnormal fibrils, derived from aggregation of misfolded, normally soluble, protein about 23 different unrelated proteins are known to form amyloid fibrils in vivo, which share a pathognomonic structure although they are associated with. This condition classically presents as small papules arranged in serpiginous or annular patterns on. 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. The code is valid for the year 2020 for the submission of hipaacovered transactions. Elastosis perforans serpiginosa is an infrequent skin dermatosis of unknown aetiology characterized by the transepidermal elimination of abnormal elastic fibres. Elastosis perforans serpiginosa in a patient with wilsons disease phoebe a thwaites, saad a khan, khashayar asadi, peter w angus a 31yearold man with wilsons disease presented with a pruritic rash across the neck of 12 months duration. Development of elastosis perforans serpiginosa in association with progressive vaso.
Lung stained with haematoxylin and eosin 1 middle diameter bronchus 2 epithelium of the mucosa 3 lamina propria of the mucosa 4 lamina muscularis of the mucosa 5 tunica submucosa 6 fibroelastic layer 7 tunica adventitia 8 alveoli 9 interstitial connective tissue of the lung 10. Perforating dermatoses, an often overlooked entity comprised of kyrles disease, perforating folliculitis, reactive perforating collagenosis, elastosis perforans serpiginosa, and acquired perforating dermatosis, are succinctly described, focusing attention on their clinical features, histopathology, treatment, and pathogenesis. Elastosis perforans serpiginosa in a patient with wilsons. Elastosis perforans serpiginosa pathology dermnet nz. A case of paraneoplastic elastosis perforans serpiginosa. 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.
A 17yearold adolescent girl visited our clinic to establish care for her genetic skin condition. Elastosis perforans serpiginosa in a patient with downs syndrome. Accumulation of fragmented elastotic material within the papillary corium. Clinically, the differential diagnoses are granuloma annulare, tinea corporis, annular sarcoidosis, cutaneous calcinosis, and porokeratosis of mibelli.
Elastosis perforans serpiginosa eps developed in three. Results from histopathology revealed changes consistent with elastosis perforans serpiginosa eps. Elastosis perforans serpiginosa in a case of pseudoxanthoma. Feb 17, 2014 elastosis perforans serpiginosa is a rare skin disease characterized by transepidermal elimination of abnormal elastic fibers.
Elastosis perforans serpiginosa is a rare skin disease characterized by transepidermal elimination of abnormal elastic fibers. Histopathology of pseudoxanthoma elasticum and related. Jul 12, 2016 pseudoxanthoma elasticum is a rare congenital inherited elastolytic disorder that has sometimes been observed in association with transepidermal elimination of altered and calcified elastic fibers resulting in elastosis perforans serpiginosa. Elastosis perforans serpiginosa definition of elastosis. Although dpenicillamine appears to be a clear trigger for elastosis perforans serpiginosa, dpenicillamineinduced disease has been rarely reported. Chronic diarrhea in an adolescent girl with a genetic skin. Elastosis perforans serpiginosa, a transepithelial elimination skin disease diagnosed by histopathology. Brightly eosinophilic fibres are seen within the extruded material, mixed with. Acquired perforating dermatosis perforating dermatoses. Elastosis perforans serpiginosa presents as small papules, either grouped or in a circular or. Low power view of intrapapillary and intradermal increase of verhoeff positive fibers. It generally affects young male individuals, and is clinically characterized by keratotic papules of verrucous aspect with centrifugal and serpiginous growth.
Departments of dermatology and histopathology addenbrookes hospital. Feb 21, 2016 dermatopathology case 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. Elastosis perforans serpiginosa eps on the nape of the neck of a woman. Pseudoxanthoma elasticum is a rare congenital inherited elastolytic disorder that has sometimes been observed in association with transepidermal elimination of altered and calcified elastic fibers resulting in elastosis perforans serpiginosa.
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. A case of elastosis perforans serpiginosa mdedge dermatology. Elastosis perforans serpiginosa genetic and rare diseases. Acquired perforating dermatosis apd is characterized by pruritic craterlike lesions with a predilection for patients affected by chronic kidney disease or diabetes mellitus dm. Elimination of both collagen and elastin has been documented in a number of cases, providing further support to the concept of a unified diagnosis of acquired perforating dermatosis. He named the lesion keratosis follieularis serpiginosa. Padley departments of dermatology and pathology, southampton university hospitals, southampton accepted for publication 2 january 1978 history m. Elastosis perforans serpiginosa department of dermatology. Perforating pseudoxanthoma elasticum with secondary elastosis. Elastosis perforans serpiginosum accessed 27 august 2018 back of neck in teenage boys. Histology eps has a characteristic appearance when stained with hematoxylin and eosin or stains for elastic fibers fig, 1. Retrogressive change in cutaneous connective tissue resulting in excessive amounts of material which give the. 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, down syndrome, and. Rare skin disease in which abnormal elastic tissue fibers, other connective tissue elements and cellular debris are expelled from papillary dermis through epidermis emedicine. Pathology of elastosis perforans serpiginosa 10 important. Elastosis perforans serpiginosa als ektodermales randsymptom. Elastosis perforans serpiginosa, a transepithelial. It has been reported to occur in wilsons disease and cystinuria, usually after many years of. It has been reported to occur in wilsons disease and cystinuria, usually after many years of highdose therapy. L1 1government medical college, kottayam kerala india corresponding author sheethal b abstract. If elastosis perforans is increasing in incidence, how can we explain such an unusual condition. The serpiginous configuration of umbilicated papules is well demonstrated. Verhoeffvan gieson, acid orceingiemsa, or alde hyde fuchsin. Pathology outlines elastosis perforans serpiginosum. The diagnosis is confirmed by histopathology gold standard.
Acneiform, scarring, and perforating lesions have been observed within plaques of pxe. In 1955 miescher further characterized this entity on the pathological findings. Abstract elastosis perforans serpiginosa is a disease that belongs to the group of perforating dermatoses, in which dermal elastic tissue extrusion occurs through epidermis. Acquired perforating dermatosis in a skin graft mdedge.
Elastosis perforans serpiginosa is a rare degenerative skin disease. 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. The idiopathic form of eps occurs rarely in children. Elastosis perforans serpiginosa article about elastosis. We report elastosis perforans serpiginosa eps arising in three patients with. Elastosis perforans serpiginosa eps is a rare disor. In 1952 lutz 1 described a peculiar serpiginous keratotie eruption that was present on the sides of the neck of a 21yearold man for 8 years. Pdf on oct 1, 2016, adriana polanska and others published elastosis perforans serpiginosa. Disorders of transepidermal elimination the human skin is a complex organ in which the. Elastosis perforans serpiginosa eps is a rare skin disorder in which there is transepithelial elimination of elastin fibers.
Elastosis perforans serpiginosa is a disease that belongs to the group of perforating dermatoses, in which dermal elastic tissue extrusion. We present a case of a 57yearold black woman admitted for chest pain and dyspnea who was found to have 2 teardropshaped yellowwhitechalky plaques consistent with apd that developed at the site. Histopathologic picture of eps with thick epidermis and. Elastosis perforans serpiginosa is a rare skin disease characterized by. Find out information about elastosis perforans serpiginosa. Perforating pseudoxanthoma elasticum with secondary. In this case, histopathology is usually performed to rule out other conditions. Elastosis perforans serpiginosa usually is asymptomatic but can be pruritic.
Elastosis perforans serpiginosa in a case of downss syndrome. Elastosis perforans serpiginosa in a patient with down syndrome and moyamoya disease is a novel finding. Elastosis perforans serpiginosa is one of four classic primary perforating disorders along with reactive perforating collagenosis, perforating folliculitis, and kyrle disease. Secondary lesions are vague scars or areas of roughness and hypopigmentation or hyperpigmentation that result after the primary lesions have subsided and disappeared. Full length report elastosis perforans perforating elastosis the association of congenital anomalies, salient facts in the histology, studies of enzyme digestion and a report of necropsy in a case from the section of dermatology, mayo clinic and. Indian j dermatol venereol leprol mayjune 2005 vol 71 issue 3 184 diagnosis of elastosis perforans serpiginosa figure 3. To our knowledge, these are the first two reported cases of elastosis perforans serpiginosa in which the pinna was affected. Histology eps has a characteristic appearance when stained with hernatoxylin and eosin or stains for elastic fibers fic. Elastosis perforans serpiginosa europe pmc article. It belongs to a group of perforating disorders of which there are four classic types. Closer inspection identifies material undergoing transepidermal elimination figure 2. The histology of the dpenicillamineinduced form is special. She exhibited red scaly plaques and patches over much of the body surface area consistent with atopic dermatitis but also had areas on the trunk with serpiginous red plaques with scale on the leading and trailing edges. Penicillamineinduced elastosis perforans serpiginosa in.
The three general categories of elastosis perforans serpiginosa are idiopathic, reactive, and drug. Elastosis perforans serpiginosa, he 20x 4661 elastosis. 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. 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 symptoms, diagnosis. Elastosis perforans serpiginosa wikimili, the free. Dermatopathology case elastosis perforans serpiginosa. It was suggestive of elastosis perforans serpiginosa eps and a biopsy was carried out in the right arm lesions. Wellknown examples are perforating granuloma annuiare and pseudoxanthoma elasticum. Elastosis perforans serpiginosa and associated disorders. Elastosis perforans serpiginosa eps is a rare chronic perforating dermatosis which was first described in 1953 by lutz. Elastosis perforans serpiginosa histopathology dpenicillamineinduced elastosis perforans serpiginosa dpenicillamineinduced elastosis perforans serpiginosa 2 tinea corporis plaques granuloma annulare plaques cutaneous sarcoidosis annular plaques on neck porokeratosis of mibelli plaques discoid lupus erythematosus atrophic.
Elastosis perforans serpiginosa an overview sciencedirect. Elastosis perforans serpiginosa eps, is a rare primary perforating dermatosis characterized by trans. Low power of histology of elastosis perforans serpiginosa demonstrates a column of keratotic debris forming a focal invagination through a hyperplastic epidermis. Elastosis perforans serpiginosa eps is a rare disorder classified as a primary. 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. Elastosis perforans serpiginosa is a rare entity that may present in many different settings. A rare skin condition characterized by the development of small, usually red, bumps on the skin. Diagnosis is based on clinical and histopathologic aspects. Elastosis perforans serpiginosa most frequently involves the nape and sides of the neck, but has been described involving the face, trunk, and upper extremities. 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. Perforating folliculitis an overview sciencedirect topics. Elastosis perforans serpiginosa presents as small papules, either grouped or in a. 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.
Jan 01, 2020 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. We report a case of pxe with associated lesions that were histopathologically compatible with eps. Is it really a new condition, or is it an old disease which has only recently been recognized. Approximately 75% of those affected are male and there appears to be no preference with respect to race. The skin lesions develop during childhood or adolescence, and progress.
Eps present as asymptomatic to pruritic groups of keratotic papules coalescing in a arcuate serpiginous distribution. The serpiginous configuration of umbilicaled papules is well demonstrated. Collagen fibers and elastic fibers were found upon histopathological examination. 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. 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. Elastosis perforans serpiginosa in a patient with downs syndrome 693 from 5 to 89 years, as reported in the literature. Two cases of dpenicillamineinduced elastosis perforans. Compared with other forms of elastosis perforans serpiginosa, there is no elastic fiber hyperplasia in the papillary dermis and a variable degree of granulomatous inflammation is often observed in the dermis. Elastosis perforans serpiginosa is a rare, primary perforating dermatosis, frequently associated with certain genetic diseases and characterized by the transepidermal extrusion of elastic fibers. Elastosis perforans serpiginosa an overview sciencedirect topics.
Elastosis perforans serpiginosa is an uncommon and chronic dermatosis characterized by transepidermal elimination of abnormal. 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 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 in a case of downss. The perforating lesions resemble elastosis perforans serpiginosa eps, but the eliminated material in pxe is calcified, unlike eps. Elastosis perforans serpiginosa is a rare complication of dpenicillamine therapy.
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