Perforating Pseudoxanthoma Elasticum Research Articles

Clinical, genetic, and ultrasonographic features of Periumbilical Perforating Pseudoxanthoma Elasticum

Skin Research and TechnologyVolume 27, Issue 4 p. 646-647 LETTER TO THE EDITOR Clinical, genetic, and ultrasonographic features of Periumbilical Perforating Pseudoxanthoma Elasticum Carlo Alberto Maronese, Corresponding Author Carlo Alberto Maronese [email protected] orcid.org/0000-0002-9449-849X Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy Correspondence Carlo Alberto Maronese. Dermatology Unit - Via Pace, 9, 20122 Milan, Italy. Email: [email protected]Search for more papers by this authorCristina Beatrice Spigariolo, Cristina Beatrice Spigariolo Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, ItalySearch for more papers by this authorFrancesca Laura Boggio, Francesca Laura Boggio Pathology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, ItalySearch for more papers by this authorChiara Moltrasio, Chiara Moltrasio Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy Department of Medical Surgical and Health Sciences, University of Trieste, Trieste, ItalySearch for more papers by this authorMichela Brena, Michela Brena Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, ItalySearch for more papers by this authorEnrico Zelin, Enrico Zelin Dermatology & Venereology Department, Dermatology Clinic, Maggiore Hospital, University of Trieste, Trieste, ItalySearch for more papers by this authorGiovanni Genovese, Giovanni Genovese orcid.org/0000-0002-7636-958X Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, ItalySearch for more papers by this authorAngelo Valerio Marzano, Angelo Valerio Marzano Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, ItalySearch for more papers by this authorGianluca Nazzaro, Gianluca Nazzaro orcid.org/0000-0001-8534-6497 Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, ItalySearch for more papers by this author Carlo Alberto Maronese, Corresponding Author Carlo Alberto Maronese [email protected] orcid.org/0000-0002-9449-849X Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy Correspondence Carlo Alberto Maronese. Dermatology Unit - Via Pace, 9, 20122 Milan, Italy. Email: [email protected]Search for more papers by this authorCristina Beatrice Spigariolo, Cristina Beatrice Spigariolo Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, ItalySearch for more papers by this authorFrancesca Laura Boggio, Francesca Laura Boggio Pathology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, ItalySearch for more papers by this authorChiara Moltrasio, Chiara Moltrasio Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy Department of Medical Surgical and Health Sciences, University of Trieste, Trieste, ItalySearch for more papers by this authorMichela Brena, Michela Brena Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, ItalySearch for more papers by this authorEnrico Zelin, Enrico Zelin Dermatology & Venereology Department, Dermatology Clinic, Maggiore Hospital, University of Trieste, Trieste, ItalySearch for more papers by this authorGiovanni Genovese, Giovanni Genovese orcid.org/0000-0002-7636-958X Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, ItalySearch for more papers by this authorAngelo Valerio Marzano, Angelo Valerio Marzano Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, ItalySearch for more papers by this authorGianluca Nazzaro, Gianluca Nazzaro orcid.org/0000-0001-8534-6497 Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, ItalySearch for more papers by this author First published: 27 February 2021 https://doi.org/10.1111/srt.13014 Maronese and Spigariolo equally contributed to this article and both qualify as first authors. Data sharing is not applicable to this article as no new data were created or analyzed in this study. Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL No abstract is available for this article. Volume27, Issue4July 2021Pages 646-647 RelatedInformation

Periumbilical perforating pseudoxanthoma elasticum: a rare case report.

Periumbilical perforating pseudoxanthoma elasticum (PPPXE) usually presents with well-defined periumbilical yellowish atrophic plaques with keratotic papules at the periphery. It is considered a variant of hereditary pseudoxanthoma elasticum or a localized acquired cutaneous dermatosis. The lesions usually occur in the periumbilical area in obese, multiparous women. Here, we report an additional case of periumbilical perforating pseudoxanthoma elasticum with its dermoscopic features.

Periumbilical perforating pseudoxanthoma elasticum ‐ an acquired perforating disorder

International Journal of DermatologyVolume 51, Issue 4 p. 439-441 Morphology Periumbilical perforating pseudoxanthoma elasticum - an acquired perforating disorder Anil Budania MBBS, Anil Budania MBBS Dermatology, Venereology and LeprologySearch for more papers by this authorDipankar De MD, Dipankar De MD Dermatology, Venereology and LeprologySearch for more papers by this authorUma N. Saikia MD, Uma N. Saikia MD Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, IndiaSearch for more papers by this authorAmrinder J. Kanwar MD, FAMS, Amrinder J. Kanwar MD, FAMS Dermatology, Venereology and LeprologySearch for more papers by this authorRahul Mahajan MD, Rahul Mahajan MD Dermatology, Venereology and LeprologySearch for more papers by this authorSavita Yadav MD, Savita Yadav MD Dermatology, Venereology and LeprologySearch for more papers by this author Anil Budania MBBS, Anil Budania MBBS Dermatology, Venereology and LeprologySearch for more papers by this authorDipankar De MD, Dipankar De MD Dermatology, Venereology and LeprologySearch for more papers by this authorUma N. Saikia MD, Uma N. Saikia MD Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, IndiaSearch for more papers by this authorAmrinder J. Kanwar MD, FAMS, Amrinder J. Kanwar MD, FAMS Dermatology, Venereology and LeprologySearch for more papers by this authorRahul Mahajan MD, Rahul Mahajan MD Dermatology, Venereology and LeprologySearch for more papers by this authorSavita Yadav MD, Savita Yadav MD Dermatology, Venereology and LeprologySearch for more papers by this author First published: 21 March 2012 https://doi.org/10.1111/j.1365-4632.2011.05276.xCitations: 4 Dipankar De, mdDepartment of Dermatology Venereology and Leprology Postgraduate Institute of Medical Education and Research Chandigarh 160012 India E-mail: dr_dipankar_de@yahoo.in Conflicts of interest: None. Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Citing Literature Volume51, Issue4April 2012Pages 439-441 RelatedInformation

Pseudoxantoma elástico perfurante periumbilical e periareolar

The periumbilical perforating pseudoxanthoma elasticum or perforating calcific elastosis is a rare disorder and its pathogenesis is associated with the alteration of elastic fibers, which may probably result from trauma. We present the case of a 70-year-old woman with long-time blackish maculas and keratotic surface papules on the periareolar and periumbilical regions. The histopathological examination revealed altered elastic fibers, replete with calcium and formation of a path in the dermis with elastic fibers degraded in their interior, confirming the clinical diagnosis.

Periumbilical perforating pseudoxanthoma elasticum

A 58-year-old, gravida 6, obese woman presented with a pruritic yellowish plaque around the umbilicus, which first appeared about 3 years ago. She also had flat yellow papules on the axilla and neck. After a burn from a heating device, a few perforating papules and a violaceous hue occurred on the plaque. Histological examination revealed pathological elastic fibers with a keratotic plug and perforation in the deep dermis, which was consistent with perforating pseudoxanthoma elasticum. This case indicates that perforating pseudoxanthoma elasticum, a variant of hereditary pseudoxanthoma elasticum may perforate due to mechanical factors.

Periumbilical pseudoxanthoma elasticum associated with chronic renal failure and angioid streaks—apparent regression with hemodialysis

Pseudoxanthoma elasticum (PXE) is a heritable connective tissue disease involving progressive fragmentation and dystrophic calcification of elastic fibers. Periumbilical disease as the exclusive site of cutaneous involvement is most commonly seen in the rare entity termed periumbilical perforating pseudoxanthoma elasticum (PPPXE). Patients with this disorder are generally obese, middle aged, multiparous black women with hypertension. The cutaneous lesions are well-demarcated, hyperpigmented, periumbilical plaques with keratotic papules on the periphery. Extracutaneous manifestations have rarely been described. We describe a patient with periumbilical PXE associated with chronic renal failure and bilateral angioid streaks. Histopathologic examination demonstrated typical calcification of elastic fibers with additional amorphous calcium deposits in the superficial dermis. Transepidermal elimination was not present. Normalization of the serum calcium-phosphate product resulted in regression of the lesions—both clinically and histopathologically. The relation between PPPXE and hereditary PXE is discussed. The role of chronic renal failure in precipitating PPPXE is considered. (J Am Acad Dermatol 1998;39:338-4.)

Periumbilical perforating pseudoxanthoma elasticum

Perforating pseudoxanthoma elasticum is a rare disorder that can occur in skin lesions associated with hereditary pseudoxanthoma elasticum and as a localized acquired cutaneous form. The localized lesion usually occurs in a periumbilical location in obese, multiparous black women. Here we report an additional case of periumbilical perforating pseudoxanthoma elasticum and discuss its potential relationship to systemic disease (hereditary pseudoxanthoma elasticum).

Perforating Pseudoxanthoma Elasticum Associated With Chronic Renal Failure and Hemodialysis

A patient with hypertension and chronic renal failure of an undetermined cause who was undergoing hemodialysis developed bilateral crusted, focally eroded plaques on her breasts. A biopsy specimen of the lesional skin revealed typical histologic changes of pseudoxanthoma elasticum, with epidermal perforation. A biopsy specimen of lesion-free skin revealed characteristics typical of pseudoxanthoma elasticum. Perforating pseudoxanthoma elasticum should be added to the growing list of cutaneous disorders that occur in patients with chronic renal failure who are undergoing hemodialysis.

Perforating pseudoxanthoma elasticum associated with chronic renal failure and hemodialysis

Perforating pseudoxanthoma elasticum associated with chronic renal failure and hemodialysis