Periumbilical perforating pseudoxanthoma elasticum is a rare acquired disorder of connective tissue. It has been suggested that repeated traumas of pregnancy, obesity, or abdominal surgery promote localized degeneration of elastic fibers. Most cases reported in the English literature occurred in obese, middle aged, multiparous women. We describe a 29-year-old male who had two hyperpigmented plaques on the periumbilical area. He had angioid streaks, but was not obese and did not have hypertension. A skin biopsy revealed transepidermal elimination of degenerated and calcified elastic fibers, which was consistent with periumbilical perforating pseudoxanthoma elasticum. We report an interesting case of periumbilical perforating pseudoxanthoma elasticum that occurred in a young man with no known predisposing risk factors.
Adult ; Angioid Streaks ; Biopsy ; Connective Tissue ; Elastic Tissue ; Female ; Humans ; Hypertension ; Male ; Middle Aged ; Obesity ; Pregnancy ; Pseudoxanthoma Elasticum ; Risk Factors ; Skin

A 56-year-old Korean woman presented with decreased visual acuity of the right eye. She had a history of two photodynamic therapy treatments for choroidal neovascularization (CNV) due to angioid streaks in her left eye with central scarring and low visual acuity. She was diagnosed with subfoveal CNV due to angioid streaks in her right eye and treated with six intravitreal bevacizumab (1.25 mg / 0.05 mL) injections over one year. Best corrected visual acuity improved from 20 / 125 at baseline to 20 / 50 at the final visit. The area of CNV had changed into a fibrotic scar by the final visit, and fluorescein angiography and indocyanine green angiography revealed no evidence of leakage. Optical coherence tomography showed that central macular thickness decreased from 311 microm at baseline to 203 microm with complete resolution of subretinal and intraretinal fluid at the final visit. Intravitreal bevacizumab for CNV associated with angioid streaks prevented the progression of disease and resulted in the improvement of visual acuity after one year of follow-up in our patient.
Angiogenesis Inhibitors/*administration & dosage ; Angioid Streaks/*complications ; Antibodies, Monoclonal/*administration & dosage ; Choroidal Neovascularization/*drug therapy/*etiology/physiopathology ; Female ; Follow-Up Studies ; Humans ; Intravitreal Injections ; Macula Lutea/drug effects/pathology ; Middle Aged ; Tomography, Optical Coherence ; Visual Acuity/drug effects

PURPOSE: To report a case of verteporfin photodynamic therapy (PDT) for juxtafoveal choroidal neovascularization (CNV) secondary to angioid streaks. CASE SUMMARY: An 54-year-old male presented with metamorphopsia of the right eye. It was diagnosed as juxtafoveal CNV secondary to angioid streaks and treated with verteporfin PDT-Best corrected visual acuity (BCVA) and macular thickness by optical coherence tomography (OCT) were measured. Over 14 month follow-up period, baseline BCVA was 0.3 and it increased to 0.4 by the final examination, while macular thickness decreased from 264 micrometer to 229 micrometer. No side effects of PDT were noted. CONCLUSIONS: Verteporfin PDT for CNV-associated with angioid streaks does not appear to significantly alter the course of the disease undergoing enlargement and disciform transformation of the neovascular process. However, we expected that the actual clinical findings improved during short term follow-up. The severity of symptoms of CNV-associated with angioid streaks in a single patient varies over time, so long-term follow-up and careful examination is needed.
Angioid Streaks ; Choroid ; Choroidal Neovascularization ; Eye ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Photochemotherapy ; Porphyrins ; Tomography, Optical Coherence ; Triazenes ; Vision Disorders ; 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

PURPOSE: To evaluate the functional and anatomic outcomes of photodynamic therapy (PDT) for choroidal neovascularization (CNV) in patients with angioid streaks. METHODS: The authors retrospectively evaluated 6 consecutive patients (6 eyes) with CNV secondary to angioid streaks. All patients were treated with standard PDT with verteporfin protocol. Standardized protocol refraction, visual acuity testing, ophthalmologic examination, color photographs, fluorescein angiograms and indocyanin angiograms were used to evaluate the results of PDT with verteporfin. Main outcome measures were visual acuity and CNV size. RESULTS: Their mean age was 61.3+/-5.50 years (range, 53-68 years). Follow-up time ranged from 12 to 38 months with mean of 20.5+/-10.91 months. The mean visual acuity at baseline was 20/100 (range 20/25-20/500), and the mean visual acuity at the last examination was 20/320(range 20/125-counting finger). The mean greatest linear dimension (GLD) at baseline was 2400+/-766.81 micrometer, and the mean GLD at the last examination was 3483+/-444.59 micrometer. CONCLUSIONS: PDT for CNV associated with angioid streaks seemed to slow down but not prevent the progression of the disease and associated visual loss.
Aged ; Angioid Streaks/*complications/*drug therapy ; Choroidal Neovascularization/*complications/*drug therapy ; Female ; Humans ; Male ; Middle Aged ; *Photochemotherapy ; Photosensitizing Agents/*therapeutic use ; Porphyrins/*therapeutic use ; Retrospective Studies

Pseudoxanthoma elasticum (PXE) is an uncommon heritable disorder of elastin characterized by cutaneous, ocular and vascular abnormalities. Typical skin lesion shows yellowish papules and redundant skin folds on the neck, axilla and groin due to degeneration and calcification of elastic fibers in deep dermis. As in all heritable disorders of connective tissue, no curative therapy is presently know. Redundant and unsightly skin folds about the neck can be improved by surgery, but irregular papular lesion on skin does not improved by surgery. A-44-year-old female who had a skin lesion and angioid streak was treated as follows: Skin folds on the neck were excised successfully without any significant problem such as intraoperative bleeding, wound dehiscence or hypertrophic scar formation. To evaluate whether we could smooth the irregular skin surface due to multiple papules, laserbrasion with a SLT contack Nd:YAG laser system was performed experimentally on a limited area which was located on the right supracla-vicular area about 2 x 3cm in size. An acceptable aesthetic appearance was achieved with surgical excision 12 months after the operation. Biopsy of the laserbrasion site revealed disappearance of the calcium deposit. This study emphasizes the possibility of using laserbrasion in solving troblesome skin lesion of the PXE.
Angioid Streaks ; Axilla ; Biopsy ; Calcium ; Cicatrix, Hypertrophic ; Connective Tissue ; Dermis ; Elastic Tissue ; Elastin ; Female ; Groin ; Hemorrhage ; Humans ; Neck* ; Pseudoxanthoma Elasticum ; Skin ; Wounds and Injuries

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

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*

we report a case of elastosis perforans serpiginosa associated with pseudoxanthoma e1asticurn on t:he neck of 27-year-old male patient. The lesions showecl erythematous scaly hyperkeratotic papules forming serpiginous pattern on yellowish elevated plaques. A skin biopsy specimen from the neck lesion revealed dense accurmulations of coarse elastic fibers partly cngulfed by epithelium in the papillary dcrrnis and increase cif short, irregulary clumped and degenerated clastic fibers in the middermis. On funduscopic exarnination, angioid streak is noted around the optic disk.
Adult ; Angioid Streaks ; Biopsy ; Elastic Tissue ; Epithelium ; Humans ; Male ; Neck ; Optic Disk ; Pseudoxanthoma Elasticum* ; Skin

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