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*

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

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)-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*

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

Periumbilical perforating pseudoxanthoma elasticum (PPPXE) is a localized acquired disorder found most frequently in obese, multiparous, middle-aged women. It is characterized clinically by yellowish, lax, well-circumscribed, reticulated or cobblestoned patches or plaques in the periumbilical region. Multiparity, obesity, massive ascites, and abdominal surgery are thought to the initiating factors. There is controversy about the etiology of PPPXE. Some authors have classified it as a separate entity from hereditary pseudoxanthoma elasticum (PXE), while others speculate that this condition merely represents a variable expression of PXE in which systemic associations are likely. We report a case of periumbilical perforating pseudoxanthoma elasticum associated with a clinical PXE lesion on the anterior neck.
Ascites ; Female ; Humans ; Neck ; Obesity ; Parity ; Pseudoxanthoma Elasticum*