Pseudoxanthoma elasticum

Laube, S.

doi:10.1136/adc.2004.062075

Cited by 100 publications

(107 citation statements)

References 26 publications

(20 reference statements)

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“…Les sujets de sexe fé minin sont en fait plus fré quemment atteints que les sujets de sexe masculin. L'anomalie gé né tique correspond à une mutation sur le chromosome 16p13.1 codant une proté ine transmembranaire appelé e MRP6 [1]. La maladie est caracté risé e par une atteinte des fibres é lastiques du tissu conjonctif.…”

Section: Discussionunclassified

“…La calcification dans le tissu conjonctif peut aboutir à des hé morragies ré tiniennes, une né ovascularisation et une perte de la vision centrale. La lé sion qui apparaît en premier est un aspect de « peau d'orange » au niveau du fond d'oeil [1].…”

Section: Discussionunclassified

“…Le premier signe typique de la maladie est fré quemment une claudication intermittente des membres infé -rieurs. Une maladie coronarienne peut apparaître chez ces patients de maniè re pré coce et aboutir à un angor, un infarctus myocardique, une insuffisance cardiaque, un prolapsus valvulaire mitral, une sté nose mitrale et/ou une cardiomyopathie restrictive [1]. Enfin, l'hypertension ré novasculaire et les accidents vasculaires cé ré braux (isché miques ou hé morragiques) sont plus fré quents chez le patient PXE [3].…”

Section: Discussionunclassified

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Analgésie épidurale obstétricale et pseudoxanthome élastique : à propos d’un cas

Clanet

Chantraine

Dewandre

et al. 2011

Annales Françaises d'Anesthésie et de Réanimation

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Section: Discussionunclassified

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Analgésie épidurale obstétricale et pseudoxanthome élastique : à propos d’un cas

Clanet

Chantraine

Dewandre

et al. 2011

Annales Françaises d'Anesthésie et de Réanimation

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“…In some cases, the perforating PXE is only a cutaneous manifestation of the systemic herededitary form of PXE, secondarily occuring the transepidermal elimination. 3 In other cases, the lesion is situated and restricted to the periumbilical region, has singular epidemiologic characteristics and occasionally might present a systemic form. In this last form, the disease has been denominated periumbilical perforating pseudoxanthoma elasticum (PPPE).…”

Section: Discussionmentioning

confidence: 99%

Pseudoxantoma elástico perfurante periumbilical e periareolar

Bressan

Vasconcelos

Silva

et al. 2010

An. Bras. Dermatol.

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Abstract: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. Keywords: Breast; Pregnancy; Pseudoxanthoma elasticum; Umbilicus Resumo: O pseudoxantoma elástico perfurante periumbilical (PEPP), ou elastose cálcica perfurante, é distúrbio raro e sua patogênese está associada a alteração da fibra elástica, tendo o trauma provável participação. Apresenta-se caso de paciente de 70 anos com quadro de máculas enegrecidas e pápulas encimadas por crostas na região periareolar e periumbilical há mais de dez anos. O exame histopatológico revelou fibras elásticas alteradas, repletas de cálcio, e formação de pertuito na derme com fibras elásticas degradadas no seu interior. O caso descrito corresponde ao PEPP, que nesta paciente também acomete área periareolar bilateralmente.

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“…8 Pseudoxanthoma elasticum, a rare condition associated with characteristic skin lesions, is associated with degeneration of elastic tissue in medium-sized arteries and focal calcification of the internal elastic lamina. 9 In the orbital and temporal arteries, internal elastic lamina calcification has also been thought to be related to vascular degeneration and arteritis. [10][11][12] Here we describe a new finding-calcification limited to the internal elastic lamina of coronary arteries of adult patients not associated with the known disorders discussed above and to our knowledge not previously reported elsewhere.…”

mentioning

confidence: 99%

Calcification of the internal elastic lamina of coronary arteries

et al. 2008

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Two well-recognized patterns of calcification occur in large-and medium-sized arteries, intimal calcification associated with atherosclerosis and medial calcification described by Mö nckeberg. Calcification limited to the internal elastic lamina is a third pattern of calcification not previously reported in coronary arteries. Here we describe 19 cases of coronary artery internal elastic lamina calcification. We serially sectioned and examined the coronary arteries of 66 patients with advanced AIDS and 27 HIVÀ controls with other chronic illnesses. We observed calcification of the internal elastic lamina in 10 HIV þ patients and 9 controls. HIVÀ patients with internal elastic lamina calcification were significantly older than HIVÀ patients without it (P ¼ 0.008) and HIV þ patients with it (P ¼ 0.006). Occasionally, calcification encroached on adjacent intimal or medial tissue with mild fibrosis. There was frequent disruption of the internal elastic lamina but no evidence of inflammation. Calcification was the dominant histologic feature in all cases. Von Kossa, Alizarin red, and trichrome/elastic stains confirmed these findings. Patients with internal elastic lamina calcification often had extensive medical histories but did not suffer from chronic renal failure or other conditions known to cause calcium dysregulation. We describe coronary internal elastic lamina calcification in HIV þ patients and older HIVÀ adults. The clinical significance of this finding is unknown. It could lead to arterial stiffening and increased pulse pressure and could be mistaken for intimal calcification on coronary imaging. Internal elastic lamina calcification may result from premature aging due to HIV disease and chronic illness or from metabolic disorders in HIV þ patients.

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Pseudoxanthoma elasticum

Cited by 100 publications

References 26 publications

Analgésie épidurale obstétricale et pseudoxanthome élastique : à propos d’un cas

Analgésie épidurale obstétricale et pseudoxanthome élastique : à propos d’un cas

Pseudoxantoma elástico perfurante periumbilical e periareolar

Calcification of the internal elastic lamina of coronary arteries

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