Pseudoxanthoma Elasticum: A Pediatric Case
| Autor: | Semra Acer, M. Levent Taşlı, Neşe Çallı Demirkan, Şeniz Ergin, Melis Çoban |
|---|---|
| Rok vydání: | 2015 |
| Předmět: |
skin
retina medicine.medical_specialty Dermatology fibers Retina Article Pseudoxanthoma elasticum child blood vessels elastic clinical examination Blood vessels male lcsh:Dermatology medicine case report human Skin business.industry lcsh:RL1-803 school child medicine.disease human tissue elastic fiber clinical feature neck rash histopathology papule business Elastic fibers |
| Zdroj: | Turk Dermatoloji Dergisi, Vol 9, Iss 4, Pp 195-197 (2015) |
| ISSN: | 1307-7635 |
| Popis: | Pseudoxanthoma elasticum (PXE) is a multisystemic, metabolic and autosomal recessive inherited disorder affecting especially elastic fibers of skin, retina and blood vessels. The prevalence varies from 1:25,000 to 1:100,000. The average age of onset is 13.5 years. Yellowish papules 1-3 mm in diameter and plaques merging as linear or reticular pattern are mostly on antecubital fossae, popliteal fossae, inguinal region, lower clavicle, neck, axilla, flexural regions as umbilicus and trauma sites. Of the patients, 85% have eye involvement. The first symptom of eye involvement is spot retinal pigmentation. Cardiovascular complications occur usually in adults. The most common and early cardiovascular complication is intermittent claudication. There is no specific treatment for skin signs. Lifestyle changes may have important effects on prognosis. A male patient with 3-year history of yellowish papules on his neck and 1-year history of yellowish papules on his groins, was presented in this case report. This 7-year-old patient received a diagnosis of PXE based on medical story, clinical examination and histopathological findings. This case was presented as PXE is a rare disease and should be diagnosed by the clinician at early ages. © 2015 by Turkish Society of Dermatology. |
| Databáze: | OpenAIRE |
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