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

Angioid streaks are easily recognized although relatively rare. They are typically described as irregularly radiating lines extending from the peripapillary area peripherally. They are usually bilateral, and are characteristically found in the posterior pole. Angioid streaks may occur alone or in association with various systemic diseases. Pseudoxanthoma elasticum is a relatively uncommon disorder of elastic tissue involving dermal, ocular, gastrointestinal, and cardiovascular tissues. We examined a case of angioid streaks associated with pseudoxanthoma elasticum found in a 31 year-old female and presented a brief review of the relating literatures.
Adult ; Angioid Streaks* ; Elastic Tissue ; 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

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

The authors experienced one case of Gronblad-Strandberg syndrome with subretinal neovascularization which showed characteristic signs of pseudoxanthoma elasticum(PXE) and angioid streak. PXE is an uncommon hereditary disorder which involves the elastic tissue of the dermis and may also involve the eye and the cardiovascular system. Angioid streaks are red or dark-brown jagged irregular lines extending in spoke like fashion toward periphery from an area of peripapillary atrophy. We report a case of Gronblad-Strandberg syndrome with subretinal neovasculari zation in a 49 year old korean female.
Angioid Streaks ; Atrophy ; Cardiovascular System ; Dermis ; Elastic Tissue ; Female ; Humans ; Middle Aged ; Pseudoxanthoma Elasticum*

Subretinal neovascular membrane involving macula was noted in two patients of angioid streaks. The neovascular membrane was treated with red-krypton laser(spot size: 200 micro, duration: 0.2 sec, intensity: 200-400mW). One patient received one more additional photocoagulation with red-krypton laser to block the neovascular membrane completely. In follow up fluorescein angiography, the neovascular membrane was blocked successfully. After more than 6 months there showed no recurrence of neovascular membrane and the visual acuity remained same as before treatment.
Angioid Streaks* ; Fluorescein Angiography ; Follow-Up Studies ; Humans ; Light Coagulation* ; Membranes ; Recurrence ; Visual Acuity

PURPOSE: To evaluate the efficacy of photodynamic therapy (PDT) with verteporfin in the management of choroidal neovascularization (CNV) associated with angioid streaks. CASE SUMMARY: Six eyes of four patients with CNV due to angioid streaks were treated with photodynamic therapy with verteporfin. Visual acuity testing, ophthalmologic examinations, photographs, and fluorescein angiograms were used to evaluate the results of PDT. Two of six eyes had subfoveal lesions, while four eyes had juxtafoveal lesions on initial examination. Conversion from a CNV to a fibrous scar lesion following PDT was observed in four eyes. Enlargement of the CNV was documented in two eyes at the final follow-up. Of a total of 6 eyes, the final best-corrected visual acuity decreased in 4 eyes and stabilized in 2 eyes. CONCLUSIONS: Photodynamic therapy can achieve short-term cessation or decrease of fluorescein leakage from chroidal neovascularization associated with angioid streaks. However, it does not alter the course of this disease, and multiple sessions are needed. Visual prognosis is poor.
Angioid Streaks* ; Choroid* ; Choroidal Neovascularization* ; Cicatrix ; Fluorescein ; Follow-Up Studies ; Humans ; Photochemotherapy* ; Prognosis ; Visual Acuity

Pseudoxanthoma elasticum is a rare, hereditable disease of connective tissue, characterzed by abberrant calcification of the elastic tissue of the skin, eyes, vasculature. We report a 61years old female patient with pseudoxanthoma elasticum with typical peau d'orange skin lesion around the neck, angioid streaks in the retina and multifocal cerebral infarction.
Angioid Streaks ; Cerebral Infarction* ; Connective Tissue ; Elastic Tissue ; Female ; Humans ; Neck ; Pseudoxanthoma Elasticum* ; Retina ; Skin

Pseudoxanthoma elasticum is an inherited disease with degenerative changes in elastic tissue of human body. Skin lesion in pseudoxanthoma elasticum is easily understood and histopathologic finding of skin lesion is chracteristic in diagnosis of pseudoxanthoma elasticum. Pseudoxanthoma elasticum combined with the both dermatologic and ocular manifestations is called Gronblad-Strandberg syndrome. Angioid streak appears ophthalmoscopically as a network of red or dark brown streaks mainly around optic disc and may occur alone or in association with various systemic diseases involving pseudoxanthoma elasticum. Fluorescein angiography of fundus is evaluated as a means of detecting early angioid streak or some other ocular manifestations of pseudoxanthoma elasticum which may be observable only by this method and not previously described. To our knowledge, this is the first three cases of Gronblad-Strandberg syndrome in Korean consecutive siblings which are presented with pathologic and fluorescein angiographic studies.
Angioid Streaks ; Diagnosis ; Elastic Tissue ; Fluorescein ; Fluorescein Angiography ; Human Body ; Humans ; Pseudoxanthoma Elasticum* ; Siblings* ; Skin