If you have problems viewing pdf files, download the latest version of adobe reader. Two uncommon cases of idiopathic atrophoderma of pasini and. Dermatopathology reference describes atrophoderma of pasini and pierini histopathology including histologic features and provides links to additional medical references. Because there is clinical and histopathologic overlap between these conditions, it is argued that they may exist on a spectrum. The concept of atrophoderma of pasini and pierini as. The red marks on her body cover 23s of her back, buttocks and thighs. Keywords atrophoderma of pasini pierini, dermal atrophy, morphea original research paper introduction idiopathic progressive atrophoderma as described by pasini1 in 1923 and later by pierini and vivoli2 in 1936 is characterized by single or multiple, sharply but often irregularly demarcated, gray. In 1923, pasini 1 described a case of pigmentary atrophoderma that was both clinically and histologically unique from any other known atrophy, including localized scleroderma, under the name of progressive idiopathic atrophoderma. May 03, 2018 since the original description, much debate has occurred regarding whether idiopathic atrophoderma of pasini and pierini is a distinct entity or an atrophic, nonindurated variant of morphea, despite differences in the origin, development, and outcome of the lesions. Jan 21, 2010 atrophoderma of pierini and pasini is thought to possibly represent a late stage of morphea a type of localized scleroderma. Progressive idiopathic atrophoderma of pasini and pierini. Abstract atrophoderma of pasini and pierini is a rare form of cutaneous atrophy.
Buechner sa, rufli t 1994 atrophoderma of pasini and pierini. Atrophoderma of pasini and pierini also known as dyschromic and atrophic variation of scleroderma, morphea plana atrophica, sclerodermie atrophique demblee is a disease characterized by large lesions with a sharp peripheral border dropping into a depression with no outpouching, which, on biopsy, elastin is normal, while collagen may. Congenital atrophoderma of pasini and pierini europe pmc. Similarities of sala, atrophoderma of pasini pierini and localized morphea are discussed, and the conclusion that sala, atrophoderma of pasini pierini and this case may. Findings similar to our case were documented in case reports of 2 women aged 34 and 42 years 1 presenting with asymptomatic, atrophic, welldemarcated, shiny, hypopigmented macules over the trunk and upper extremities, which demonstrated a thinned epidermis with coarse hyalinized collagen bundles in the mid and lower dermis. The clinical aspect of the lesion and the nonspecific findings on the histological report suggested the diagnosis of idiopathic atrophoderma of pasini and pierini. Idiopathic atrophoderma of pasini and pierini is typically a benign, asymptomatic condition.
Its clinical appearance varies and may closely resemble that of atrophoderma of pasini and pierini app and linear scleroderma. Atrophoderma of pasini and pierini is a skin atrophy presenting as single or multiple sharply demarcated, hyperpigmented, nonindurated patches, with a slight depression of the skin, that can. Little is known about the clinical variants of iapp, and limited data are available on antibiotic therapy for this condition. The argentine dermatologists, especially luis pierini, have, since. The gulf journal of dermatology and venereology volume 22, no. Our purpose was to define the various types of iapp clinically and histologically and. Idiopathic atrophoderma of pasini and pierini iapp is an uncommon cutaneous disease, readily identified by its unique clinical features, particularly pigmented ovularround depressed plaques. It is characterised by one or more asymptomatic, bilaterally symmetrical, well defined, hyperpigmented, non sclerotic patches with an abrupt edge, distributed mostly over the trunk. Selfinvoluting atrophoderma of the lateralupper arm sala, first described by inazumi et al. They believed that the idiopathic atrophoderma of pasini and pierini differed sufficiently from morphea to classify it as a distinct entity.
Atrophoderma of pasini and pierini primary care dermatology. Atrophoderma of pasini and pierini atrophoderma of pasini and pierini cliff, s harland, c. Idiopathic atrophoderma of pasini and pierini iapp is a distinctive form of dermal atrophy, usually appearing as one or more sharply demarcated depressed areas. Pdf atrophoderma of pasini and pierini researchgate. Idiopathic atrophoderma of pasini and pierini iapp usually manifests as one or multiple depressed and hyperpigmented patches, with a predilection to the trunk. Linear atrophoderma of moulin lam is an acquired skin condition that manifests in early childhood and adolescence. Pasini first described this condition in 1923 as progressive idiopathic atrophoderma1 and later pierini suggested its link to morphea.
Only in one patient was iapp coexistent with morphea. Atrophoderma of pasini and pierini differential diagnoses. The histologic findings were consistent with atrophoderma. They believed that the idiopathic atrophoderma of pasini and pierini app differed sufficiently from morphea to classify it as a distinct entity. Atrophoderma of pasini and pierini and systemic scleroderma. Normally it spreads slowly in a bilateral symmetrical distribution and. Idiopathic atrophoderma of pasini and pierini is a form of dermal atrophy of unknown etiology, usually affecting women during their adolescence and young adulthood. May 03, 2018 exacerbated course of atrophoderma of pasini and pierini in patient with papillary cancer of the thyroid gland.
Histopathological examination revealed normal epidermis, hyalinization of collagen in dermis with minimal scattered inflammatory infiltrate. Atrophoderma of pasini and pierini garg a, kumar p indian. Electron microscopic study demonstrated macrophages and lymphocytes around vessels and between fibers in the dermis. Atrophoderma of pasini and pierini mahe digital repository. Atrophoderma of pasini and pierini garg, anubhav and, pramod kumar 2011 atrophoderma of pasini and pierini. The appearances are similar to those described for atrophoderma. Unusual presentations may include congenital lesions 1 or with a blaschkoid pattern. Atrophoderma of pasini and pierini is a skin disease manifested with depressed skin in areas that have a histopathology con rmed as atrophy with hyperpigmentation. Selfinvoluting atrophoderma of the lateralupper arm.
At that time the disease was linked to localized scleroderma, and in. In 1936, in argentina, pierini and vivoli 2 extensively studied and defined the condition and its possible link to morphea. Atrophoderma of pasini and pierini, cliffdrop borders. Thickening and hyalinization of connective tissue of deep dermis, subcutaneous fat and muscular fascia, with perivascular and focal interstitial lymphocytic and plasma cell infiltrate in subcutaneous fat. Pdf on jul 1, 2011, anubhav garg and others published atrophoderma of pasini and pierini find, read and cite all the research you need on researchgate. Atrophoderma of pasini and pierini is a rare dermatologic condition that was first described. Other conditions that follow blaschko linessuch as pigmentary mosaicismmay also be considered. We describe the clinical and histological symptoms of four women with iapp. At that time the disease was linked to localized scleroderma, and in 1958 it. Atrophodermalike guttate morphea mdedge dermatology. Atrophoderma of pasini and pierini classically presents as asymptomatic depressed areas on the trunk and extremities. It is believed to be a distinct entity from morphoea. Atrophoderma definition of atrophoderma by medical.
Atrophoderma of pasini pierini tolat s n, kotkar d. Archives of dermatology and syphilology an interesting article entitled idiopathic atrophoderma of pasini and pierini was published by dr. Idiopathic atrophoderma of pasini and pierini iapp is a rare, exclusively cutaneous disease. Idiopathic atrophoderma of pasini and pierini iapp is a localized form of dermal atrophy seen. Atrophoderma of pasini and pierini is a skin disease manifested with depressed skin in areas that have a histopathology con firmed as atrophy with. Saleh z1, abbas o, dahdah mj, kibbi ag, zaynoun s, ghosn s. What is atrophoderma of pasini and pierini atrophoderma of pasini and pierini is a rare dermal atrophy disorder characterised by wellcircumscribed slightly depressed areas, usually on the patients trunk. It likely represents a form of cutaneous mosaicism that presents with linear, hyperpigmented and atrophic lesions appearing on the trunk and limbs.
The border between normal skin and the area of atrophy has a welldefined, cliffdrop edge atrophoderma of pasini and pierini. Idiopathic atrophoderma of pasini and pierini with coexistent. Case report a 35yearold man presented with a 8 years history of hyperpigmented, atrophic lesions limited to his back, which had been insidious but. She is 16 years of age and was first diagnosed 2 years ago. Atrophoderma of pierini and pasini genetic and rare. What are the clinical features of atrophoderma of pasini and pierini. Atrophoderma of pasini and pierini anubhav garg 1, pramod kumar 2 1 department of dermatology, venereology and leprosy, rnt medical college, udaipur, rajasthan, india 2 department of dermatology, venereology and leprosy, kasturba medical college, mangalore, karnataka, india. Idiopathic atrophoderma of pasini and pierini iapp is a form of dermal atrophy that manifests as single or multiple sharply demarcated, hyperpigmented, nonindurated patches. Idiopathic atrophoderma of pasini and pierini is a rare skin disease which is thought to affect dermal collagen organization resulting in dermal atrophy.
There was upper and diffuse dermal elastolysis patient 1 and. The disorder usually begins during adolescence or early adulthood with a slightly erythematous lesion appearing on the trunk, commonly on the back or lumbosacral region, followed in frequency by the chest, arms, and abdomen. Atrophoderma of pasini and pierini refers to asymptomatic, depressed, slightly hyperpigmented lesions usually located on the back and most often occurring in young people. Multiple and giant zhang ruzhi 1, zhu wenyuan 2 1 department of dermatology, first affiliated hospital, bengbu medical college, anhui, china 2 department of dermatology, first affiliated hospital, nanjing medical university, nanjing, china. Atrophoderma the only comfort that i get is that my doctor told me that it should never spread to my. Atrophoderma of pasini and pierini american osteopathic. The clinical aspect of the lesion and the nonspecific findings on the histological report suggested the diagnosis of idiopathic atrophoderma of pasini and pierini iapp. Atrophoderma of pasini and pierini wiley online library. Atrophoderma of pasini and pierini dermatology advisor. Atrophoderma of pasini and pierini clinical and histopathologic findings and antibodies to borrelia burgdorferi in thirtyfourpatients stanislaw a. Idiopathic atrophoderma of pasinipierini iapp is an uncommon dermatologic condition of unknown etiology, characterized by asymptomatic,single or multiple sharply demarcated, round to oval, hyperpigmented, nonindurated depressed patches of varying sizes. Mother of atrophoderma of pierini and pasini patient my 17yearold daughter was told she had atrophoderma of pierini and pasini app last year toot.
When initially diagnosed she also had a linear morphea down one leg but this h. We report here a 19 year old female presenting with single, asymptomatic. Idiopathic atrophoderma of pasini and pierini iapp is an infrequent form of cutaneous atrophy, of unknown etiology. Atrophoderma of pasini and pierini app is a disease that causes thinning in a layer of skin called the dermis. Atrophoderma of pasini and pierini in a young adult acta. These patches are marked by a slight depression of the skin with an abrupt edge, usually on the backs of adolescents or young adults. Improvement in atrophoderma of pasini and pierini has occurred during treatment with doxycycline in a patient with igm antibodies to b. Three of them had lesions on the back, while the fourth had them on the buttocks. Histopathological examination of a specimen from the involved. Atrophoderma elastolytica discreta is the clinicopathologic name for a unique entity herein first described in a patient with cutaneous lesions simulating atrophoderma of pasini and pierini but coupled with histopathologic changes of anetoderma. Pasini first described this condition in 1923 as progressive idiopathic atrophoderma 1 and later pierini suggested its link to morphea.
Two uncommon cases of idiopathic atrophoderma of pasini. Atrophoderma of pasini and pierini app is a rare skin disorder affecting dermal collagen and presenting with dermal atrophy. Atrophoderma of pierini and pasini is thought to possibly represent a late stage of morphea a type of localized scleroderma. Atrophoderma refers to conditions involving skin atrophy. The histopathologic changes, usually subtle and nondiagnostic. The epidermis appears normal except for rare reports of atrophy. The clinical and histological findings seen here have not been previously seen in the many variants of anetoderma, and, as such, they are sui generis. Two uncommon cases of idiopathic atrophoderma of pasini and pierini. Idiopathic congenital atrophoderma of pasini and pierini. The differential diagnosis of lam includes atrophoderma of pasini and pierini app and linear morphea.
Buechner, md, and thea rum, md basel, switzerland background. Pasini and pierini described a form of atrophoderma in 1923 and 1936, respectively. Idiopathic atrophoderma of pasini and pierini lapp is a distinctive form of dermal atrophy, usually appearing as one or more sharplydemarcateddepressed areas. A case of unilateral idiopathic atrophoderma of pasini and pierini. Atrophoderma of pasini and pierini sclero forums main. Abe i, ochiai t, kawamura a, muto r, hirano y, ogawa m. It is more frequent in females, with incidence peak in the second and third decades of life. Atrophoderma of pasini and pierini is a rare variant of dermal atrophy with unknown etiology. Idiopathic atrophoderma of pasini and pierini iapp usually manifests as one or multiple depressed and hyperpigmented patches. A case of atrophoderma of pasini and pierini associated. May 15, 2007 i have atrophoderma of pasini and pierini several years ago which resulted in several pigmented areas in my trunk back and arms i have tried antibiotics and creams for bleaching a cream is working but very slow and it doesnt completely removes the colour i read on the net about q switch. Calcium deposits were scattered throughout the dermis and extended into the subcutis. For language access assistance, contact the ncats public information officer. A 2yrold girl was presented with erythematous atrophic lesion on the right shoulder, which appeared from birth.
Idiopathic atrophoderma of pasini and pierini jama. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. No diagnostic changes are usually seen on histology. Microscopic examination showed small collections of mononuclear cells around dermal blood vessels. A 2yrold girl was presented with erythematous atrophic lesion on the right shoulder. References this cutaneous condition article is a stub. The term congenital linear atrophoderma has been used for an infant with depressed, hypopigmented, linear plaques of congenital onset on the lower extremity. Diagnosing atrophoderma of pasini and pierini app is reasonably straightforward it usually presents in adolescents and young adults, more often in females, with sharply defined, single or multiple round to oval, hyperpigmented, depressed lesions with a cliffdrop border. The disease was rst described by pasini and later by pierini and vivoli 1.
Journal of the american academy of dermatology 303. The clinical appearance of atrophoderma of pasini and pierini has been likened to footprints in snow or depressions with cliff drop borders4. The typical presentation is of single or multiple sharply demarcated, mildly depressed, hyperpigmented lesions on the trunk. Congenital atrophoderma of pasini and pierini idiopathic atrophoderma of pasini and pierini is a form of dermal atrophy of unknown etiology, usually affecting women during their. Department of dermatology, sree balaji medical college and hospital, bharat university, chromepet, chennai 600044, india. The classic clinical manifestations of app are hyperpigmented or hypopigmented, depressed areas of skin on the trunk or extremities. Department of dermatology and pathology, rajis skin care. Clinical and histopathologic findings and antibodies to borrelia burgdorferi in thirtyfour patients. In this article we shall discuss a peculiar cutaneous atrophy described by pasini 1 in 1923, under the title of progressive idiopathic atrophoderma, which has received no attention as such in the american dermatologic literature and is not mentioned in any textbooks in english. Congenital atrophoderma of pasini and pierini ajou open. A case of atrophoderma of pasini and pierini associated with.
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