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

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

Late-onset focal dermal elastosis is a recently described disorder afflicting elderly patients with yellow papules on the neck and flexural areas. Although there are similar clinical findings to pseudoxanthoma elasticum, no stigmata of pseudoxanthoma elastimum are not evident. Histologically the papules are characterized by increased elastic fibers of normal appearance in the mid and deep dermis and no evidence of solar elastosis. Late-onset focal dermal elastosis may be caused by focal accumulation of normal appearing elastic tissue due to loss of age related homeostatic growth regulating gene control mechanism. We report a case of pseudoxanthoma elasticum-like late-onset focal dermal elastosis and suggest that this condition may be more common than has been reported.
Aged ; Christianity ; Dermis ; Elastic Tissue ; Humans ; Neck ; Pseudoxanthoma Elasticum

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*

Two cases of pseudoxanthoma elasticum associated with angioid streaks in both eyes were presented and the literatures were reviewed. The affected sisters were 15 and 18 years female among the five siblings. They had pin head sized yellowish maculopapular eruptions on the sides of the neck and axillae, and also visual distubance of the both eyes. They seemed to be autosomal recessive inberitance in their family background. There were no other complications.
Angioid Streaks ; Axilla ; Female ; Head ; Humans ; Neck ; Pseudoxanthoma Elasticum* ; Siblings

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