Pseudoxanthoma elasticum with generalized cutaneous laxity

Uenishi, T.

doi:10.1001/archderm.133.5.664

Cited by 11 publications

(4 citation statements)

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“…Skin lesions are commonly seen in PXE cases, especially between age of 20-25 in the early period of the disease [10][11][12][13][14] . Generally, skin is observed to be wrinkled and flaccid.…”

Section: Discussionmentioning

confidence: 99%

“…As a result of the disruption of calcium-phosphate balance; calcium phosphate salts are built-up in trauma area, injection focus, and small to midsize subcutaneous vessels. Due to long-term penicillamin usage, lesions such as anetoderma and elastosis perforans serpiginosa may be see [12] . Cardiovascular system results may not be as apparent as ocular and cutaneous results.…”

Section: Discussionmentioning

confidence: 99%

“…However, there is a reported case including a sudden death secondary to cardiac involvement in the literature [15,16] . Clinically, besides presence of angina pectoris and intermittent claudication due to involvement of both internal and external elastic laminas of coronary arteries and peripheral large arteries, as a result of the degeneration of only the internal elastic membrane of thin-walled vessels in stomach mucosa, hematochezia or melena may be determined [6,7,12] . In our case, there was no angina pectoris history.…”

Section: Discussionmentioning

confidence: 99%

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Pseudoxantoma elasticum, as a repetitive upper gastrointestinal hemorrhage cause in a pregnant woman

Göral¹

2007

WJG

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P s e u d ox a n t o m a e l a s t i c u m i s a ra re , h e re d i t a r y, multisystemic disease affecting the skin, eye, and cardiovascular system. A twenty-eight-year-old female has presented to emergency unit with the complaint of gastrointestinal hemorrhage. This patient, who had been monitored in the gastroenterology clinic more than 10 times in the past 8 years, noted a repetitive hemorrhage during her previous pregnancy in her history. The examination of the patient revealed the following signs and symptoms: atrophy in the epithelium of the retina pigment; typical angioid streaks and peau d'orange finding in the fundus; thinning of the retinal nerve fiber in OCT (optic coherence tomography); bilateral and reticular papillary lesions with yellowishcolor in the neck region (plucked chicken appearance); presence of bleeding foci in fundus; and nephrocalcinosis in kidneys. In light of these symptoms, the patient was diagnosed with pseudoxantoma elasticum. Skin biopsy confirmed the pseudoxantoma elasticum diagnose. PXE is an uncommon, hereditary disease. Early diagnosis of pseudoxantoma elasticum cases, is important for minimalizing systemic complications and informing the other family members through genetic counseling.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Pseudoxantoma elasticum, as a repetitive upper gastrointestinal hemorrhage cause in a pregnant woman

Göral¹

2007

WJG

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“…Typically, these changes are first noted on the lateral part of the neck and later involve the antecubital fossae; the axillae; the popliteal spaces; the inguinal and periumbilical areas; the oral mucosa involving the lower lip, cheek, and palate; and the vaginal and rectal mucosae. 10 As the disease progresses, the skin of the neck, the axillae, and the groin may become soft, lax, and wrinkled, hanging in folds. The extent of these changes is usually limited, but generalized cutis laxa-like pseudoxanthomaelasticum has also been reported.…”

Section: Case Reportmentioning

confidence: 99%