Generalized Pseudoxanthoma Elasticum with Perforating lesions-An Extremely rare entity with unusual presentation
Journal Title: Journal of Medical Science And clinical Research - Year 2017, Vol 5, Issue 7
Abstract
Pseudoxanthoma elasticum (PXE) is a genetically inherited disorder of elastic fibers. Occurrence of perforating lesions is quite uncommon and further rarer is cutis laxa like generalized skin involvement in PXE. It is characterized by elastorrhexia of cutaneous, ocular and cardiovascular elastic tissue. Skin is affected in flexures like neck, axilla and periumbilical region. Classical cutaneous lesions are yellow papulo-plaques over lax skin giving a characteristic plucked chicken appearance to affected area. Reports of cutis laxa like generalized PXE are very sparse, further associated perforating lesions have not been described in generalized PXE till now. Here we report a 53 year old female with generalized lax skin along with erythematous and atrophic plaques surmounted with keratotic papules over the trunk. Clinical possibility of generalized PXE with perforating lesions was further confirmed on histopathology. Haematoxyline and eosin staining revealed fragmented and swollen elastic fibers in mid dermis while dystrophic calcification was demonstrated with vonkossa stain. The highlight of our case is cutis laxa like generalized skin involvement coexisting with perforating lesions in a patient of PXE.
Authors and Affiliations
Dr Sandhya Chauhan
Keywords
Related Articles
- EP ID EP526580
- DOI -
- Views 80
- Downloads 0
Efficacy of Phenytoin on Chronic Non Healing Diabetic Ulcer - A Prospective Study
Background: Chronic non healing ulcer is one of the major cause for morbidity in diabetic patients. In spite of various types of dressing agents available, choosing an appropriate dressing is a challenging aspect in the...
Descriptive Epidemiology and Complications of Haemophilia in Assiut, Egypt
Background: Haemophilia is a group of rare congenital disorders of blood where there’s a defect in mechanism of clotting due to deficiency in factor VIII (Haemophilia A) or factor IX (Haemophilia B). It’s inherited as xl...
Case Report: Acute Chordae Tendineae Rupture and Mitral Regurgitation in Pregnancy
A 29 year old woman complained of breathlessness, chest pain and palpitations at 36 weeks of gestation. A pansystolic murmur alongwith bilateral basal crepitations were heard on auscultation. Echocardiography revealed a...
Ancient Concept of Grudhrasi
The disorder which impair the movement of leg are as old as the existence of human being, as walking is an inevitable function since the existence of man on earth to search for his food. Vata is responsible for all the m...
A Study of Comparison of Lung Function Test between Male and Female Young Adults
The lung function tests are broad range of tests that provide an assessment of the respiratory system in terms of its function. There are a few variables such as age, gender and body size which have impact on the lung fu...