No abstract available.
Pseudoxanthoma Elasticum*

The authors recently experienced a case of typical Gronblad-strandberg's syndrome which showed characteristic signs such as degenerative changes of the skin and angioid streaks without known familial disposition of this disease, and the literatures relating to this disease were briefly reviewed.
Angioid Streaks* ; Pseudoxanthoma Elasticum* ; Skin

No abstract available.
Carotid Artery, Internal ; Pseudoxanthoma Elasticum

We report a case of periumbilical pseudoxanthoma elasticum in a 73-year-old female. It is characterized by a dark-yellowish plaque present on the periumbilical area of the lower abdomen. The plaque is well-demarcated and its surface is grooved or fissured, atrophic and verrucoid. This localized lesion of the periumbilical area occurs in a multiparous woman with no history of hereditary systemic pseudoxanthoma elasticum.
Abdomen ; Aged ; Female ; Humans ; Pseudoxanthoma Elasticum*

No abstract available.
Glaucoma, Angle-Closure* ; Humans ; Pseudoxanthoma Elasticum*

Three cases of pseudoxanthoma elasticum are presented and the literatures are reviewed. Two cases were presented with the marked cutaneous manifestation of this condition and angioid streak. One case was presented with only the marked skin lesion of this condition on both axilla and neck. Typically involved skin was shown to be speckled with pea-sized yellowish numorous papules. Typical elastic tissue degeneration was found in the involved skin. No genetic background was discovered in the patients.
Angioid Streaks ; Axilla ; Elastic Tissue ; Humans ; Neck ; Pseudoxanthoma Elasticum* ; Skin

Pseudoxanthoma elasticum (PXE) is a heritable disorder of elastic tissue and it has many systemic manifestations. PXE is a systemic disorder that most markedly affects the elastic tissues in the skin, retina and blood vessels. Defects in the ABCC6 gene lead to calcification of elastic tissue. We herein report on two cases of pseudoxanthoma elasticum that presented with focal involvement on the skin of the neck without marked wrinkling.
Blood Vessels ; Elastic Tissue ; Neck ; Pseudoxanthoma Elasticum ; Retina ; Skin

Nineteen years old female with typical pseudoxanthoma elasticum without family history of the disease, and 26 years old male with characteristic clinical and histopathologic findings of Groenblad-Strandberg syndrome and family history of pseudoxanthoma elasticum in his 9 years old sister were presented.
Adult ; Child ; Female ; Humans ; Male ; Pseudoxanthoma Elasticum* ; Siblings

Angioid streakes are seen relatively rarely and their basic histopathology seems to be generally accepted as representing ruptures and breaks in lamina vitrea, although the pathogenesis of these ruptures has not been well understood. Angioid streaks appear ophthalmoscopically as a network of red or dark brown streaks emanating from a more or less complete peripapillary ring of the same color. Angioid streaks associated with the cutaneous lesions is called the Groenblad-Strandberg syndrome. Pseudoxanthoma elasticum is a relatively uncommon disorder of elastic tissue involving dermal, ocular, gastrointestinal, and cardiovascular tissues. The purpose of this paper is to describe a case of angioid streakes associated with pseudoxanthomsa elasticum found in a 39 year-old Korean male.
Adult ; Angioid Streaks* ; Elastic Tissue ; Humans ; Male ; Pseudoxanthoma Elasticum* ; Rupture

Pseudoxanthoma elasticum (PXE)-like papillary dermal elastolysis (PDE) is an unusual acquired elastolytic disorder that clinically resembles PXE but shows partial or complete loss of the elastic network in the papillary dermis. PXE-like PDE is characterized by the multiple yellowish nonfollicular papules, small in diameter, on the antecubital fossae, flexor aspect of the forearms, supraclavicular region, sides of the neck, and lower abdominal area. The multiple papules coalesce to form plaques with a cobble stone appearance resembling "plucked chicken skin". No systemic involvement has been noted in this disorder. Histopathologically, Hematoxylin-eosin stain shows nearly normal finding, but elastic stain shows partial or complete loss of elastic fibers focally in the papillary dermis. We report a case of pseudoxanthoma elasticum-like papillary dermal elastolysis with typical clinical and pathological features.
Chickens ; Dermis ; Elastic Tissue ; Forearm ; Neck ; Pseudoxanthoma Elasticum*