Elastosis perforans serpiginosa elastosis perforans serpiginosa catterall, m. Elastosis perforans serpiginosa an overview sciencedirect topics. Development of elastosis perforans serpiginosa in association with progressive vaso. He named the lesion keratosis follieularis serpiginosa. Find out information about elastosis perforans serpiginosa. This condition occurs in the marfan syndrome, the ehlersdanlos syndrome, osteogenesis imperfecta, pseudoxanthoma elasticum, and down syndrome. Perforating folliculitis an overview sciencedirect topics. Elastosis perforans serpiginosa, a transepithelial elimination skin disease diagnosed by histopathology. Is it really a new condition, or is it an old disease which has only recently been recognized. 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. Collagen fibers and elastic fibers were found upon histopathological examination. All but one case is reported as occurring since 1950. Elastosis perforans serpiginosa in a patient with downs syndrome 693 from 5 to 89 years, as reported in the literature.
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. Pdf elastosis perforans serpiginosa is a rare skin disease characterized by transepidermal elimination. 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. 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. We report elastosis perforans serpiginosa eps arising in three patients with.
Elastosis perforans serpiginosum accessed 27 august 2018. 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. To our knowledge, these are the first two reported cases of elastosis perforans serpiginosa in which the pinna was affected. Chronic diarrhea in an adolescent girl with a genetic skin. We report a case of pxe with associated lesions that were histopathologically compatible with eps. Secondary lesions are vague scars or areas of roughness and hypopigmentation or hyperpigmentation that result after the primary lesions have subsided and disappeared. The perforating lesions resemble elastosis perforans serpiginosa eps, but the eliminated material in pxe is calcified, unlike eps. Elastosis perforans serpiginosa symptoms, diagnosis. 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. Low power of histology of elastosis perforans serpiginosa demonstrates a column of keratotic debris forming a focal invagination through a hyperplastic epidermis figure 1. 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 is a rare complication of dpenicillamine therapy. Elastosis perforans serpiginosa usually is asymptomatic but can be pruritic. It generally affects young male individuals, and is clinically characterized by keratotic papules of verrucous aspect with centrifugal and serpiginous growth. Elastosis perforans serpiginosa eps is a rare chronic perforating dermatosis which was first described in 1953 by lutz. Elastosis perforans serpiginosa eps is a rare disor. Elastosis perforans serpiginosa wikimili, the free. Elastosis perforans serpiginosa is a rare, primary perforating dermatosis, frequently associated with certain genetic diseases and characterized by the transepidermal extrusion of elastic fibers. A 17yearold white boy with no underlying connective tissue disorders presented with flattopped annular plaques, with slight central atrophy on the bilateral neck. 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. Low power view of intrapapillary and intradermal increase of verhoeff positive fibers. Elastosis perforans serpiginosa is a reactive perforating dermatosis in which elastic fibers are extruded through the epidermis. Brightly eosinophilic fibres are seen within the extruded material, mixed with. 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.
The patient reported that she had experienced chronic diarrhea and abdominal. Elastosis perforans serpiginosa als ektodermales randsymptom. Approximately 75% of those affected are male and there appears to be no preference with respect to race. Two cases of dpenicillamineinduced elastosis perforans. 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. Elastosis perforans serpiginosa is a rare skin disease characterized by transepidermal elimination of abnormal elastic fibers. 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. The diagnosis is confirmed by histopathology gold standard.
Elastosis perforans serpiginosa most frequently involves the nape and sides of the neck, but has been described involving the face, trunk, and upper extremities. Elastosis perforans serpiginosa eps on the nape of the neck of a woman. Elastosis perforans serpiginosa in a case of downss syndrome. Patients have also been reported in whom lesions had variably showed the histology of pf, rpc, and kd. Acquired perforating dermatosis in a skin graft mdedge. A case of paraneoplastic elastosis perforans serpiginosa. It has been reported to occur in wilsons disease and cystinuria, usually after many years of highdose therapy. 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. Wellknown examples are perforating granuloma annuiare and pseudoxanthoma elasticum. Dermatopathology case elastosis perforans serpiginosa. Histopathology of pseudoxanthoma elasticum and related. A case of elastosis perforans serpiginosa mdedge dermatology.
Diagnosis is based on clinical and histopathologic aspects. Perforating pseudoxanthoma elasticum with secondary. Retrogressive change in cutaneous connective tissue resulting in excessive amounts of material which give the. Jul 31, 20 dermatopathology reference describes elastosis perforans serpiginosa histopathology including histologic features and provides links to additional medical references. Elastosis perforans serpiginosa article about elastosis. 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. The skin lesions develop during childhood or adolescence, and progress.
Elastosis perforans serpiginosa europe pmc article. Acquired perforating dermatosis apd is characterized by pruritic craterlike lesions with a predilection for patients affected by chronic kidney disease or diabetes mellitus dm. 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 presents as small papules, either grouped or in a circular or. Elastosis perforans serpiginosa in a patient with down syndrome and moyamoya disease is a novel finding. This condition classically presents as small papules arranged in serpiginous or annular patterns on the neck, face, arms, or other flexural areas. 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. Padley departments of dermatology and pathology, southampton university hospitals, southampton accepted for publication 2 january 1978 history m. Disorders of transepidermal elimination the human skin is a complex organ in which the. Elastosis perforans serpiginosa is an uncommon and chronic dermatosis characterized by transepidermal elimination of abnormal. 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. 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. 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.
It was suggestive of elastosis perforans serpiginosa eps and a biopsy was carried out in the right arm lesions. Indian j dermatol venereol leprol mayjune 2005 vol 71 issue 3 184 diagnosis of elastosis perforans serpiginosa figure 3. The three general categories of elastosis perforans serpiginosa are idiopathic, reactive, and drug. Elastosis perforans serpiginosa in a patient with wilsons. Carver college of medicine department of dermatology 200 hawkins drive. Elastosis perforans serpiginosa, down syndrome, and. In this case, histopathology is usually performed to rule out other conditions. 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. If elastosis perforans is increasing in incidence, how can we explain such an unusual condition. Penicillamineinduced elastosis perforans serpiginosa in. 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 in a patient with downs syndrome.
Perforating pseudoxanthoma elasticum with secondary elastosis. Elastosis perforans serpiginosa is a rare skin disease characterized by. 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. Elastosis perforans serpiginosum accessed 27 august 2018 back of neck in teenage boys. Elastosis perforans serpiginosa, he 20x 4661 elastosis. 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. When submitting mnemonics, be sure to utilize bold and different text color to emphasize important letters or phrases within your mnemonic. The code is valid for the year 2020 for the submission of hipaacovered transactions.
Histology eps has a characteristic appearance when stained with hernatoxylin and eosin or stains for elastic fibers fic. In 1955 miescher further characterized this entity on the pathological findings. Pathology outlines elastosis perforans serpiginosum. Elastosis perforans serpiginosa eps is an uncommon and chronic dermatosis characterized by the transepidermal elimination of altered elastic fibers originating in the dermis. A 17yearold adolescent girl visited our clinic to establish care for her genetic skin condition.
Acquired perforating dermatosis perforating dermatoses. It has been reported to occur in wilsons disease and cystinuria, usually after many years of. The histology of the dpenicillamineinduced form is special. Elastosis perforans serpiginosa genetic and rare diseases. Elastosis perforans serpiginosa eps developed in three. 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. A rare skin condition characterized by the development of small, usually red, bumps on the skin. Histology eps has a characteristic appearance when stained with hematoxylin and eosin or stains for elastic fibers fig, 1. Pdf on oct 1, 2016, adriana polanska and others published elastosis perforans serpiginosa. Elastosis perforans serpiginosa may be distinguished morphologically and histologically from oth.
This condition classically presents as small papules arranged in serpiginous or annular patterns on. Elastosis perforans serpiginosa definition of elastosis. Elastosis perforans serpiginosa an overview sciencedirect. Pathology of elastosis perforans serpiginosa 10 important. Elastosis perforans serpiginosa eps, is a rare primary perforating dermatosis characterized by trans. The serpiginous configuration of umbilicaled papules is well demonstrated. Closer inspection identifies material undergoing transepidermal elimination figure 2. Verhoeffvan gieson, acid orceingiemsa, or alde hyde fuchsin. L1 1government medical college, kottayam kerala india corresponding author sheethal b abstract. Accumulation of fragmented elastotic material within the papillary corium. 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. 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 in a case of downss. Elastosis perforans serpiginosa is a rare entity that may present in many different settings.
Elastosis perforans serpiginosa department of dermatology. Departments of dermatology and histopathology addenbrookes hospital. Elastosis perforans serpiginosa presents as small papules, either grouped or in a. The idiopathic form of eps occurs rarely in children. Although dpenicillamine appears to be a clear trigger for elastosis perforans serpiginosa, dpenicillamineinduced disease has been rarely reported. Elastosis perforans serpiginosa is a rare degenerative skin disease. Verhoeffvan gieson, acid orceingiemsa, or aldehyde fuchsin. 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. Results from histopathology revealed changes consistent with elastosis perforans serpiginosa eps. Elastosis perforans serpiginosa eps is a rare skin disorder in which there is transepithelial elimination of elastin fibers. Elastosis perforans serpiginosa pathology dermnet nz. Some associated genetic conditions include down syndrome, pseudoxanthoma elasticum, marfan syndrome, ehlersdanlos syndrome, acrogeria, osteogenesis imperfecta, rothmundthomson syndrome, and moyamoya disease. Elastosis perforans serpiginosa in a case of pseudoxanthoma. Elastosis perforans serpiginosa is an infrequent skin dermatosis of unknown aetiology characterized by the transepidermal elimination of abnormal elastic fibres.
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. Acneiform, scarring, and perforating lesions have been observed within plaques of pxe. 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 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 disorder classified as a primary. The serpiginous configuration of umbilicated papules is well demonstrated. 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. Elastoma intrapapillare perforans verruciforme or keratosis follicularis serpiginosa. Histopathologic picture of eps with thick epidermis 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.
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, a transepithelial. Abstract 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 and associated disorders.
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