A case of pseudoxanthoma elasticum with proliferative diabetic retinopathy.

BACKGROUND: To report the case of a patient with pseudoxanthoma elasticum (PXE) and proliferative diabetic retinopathy (PDR), and discuss the relationship between PXE and diabetic retinopathy (DR). CASE PRESENTATION: A 47-year-old man with PXE presented with angioid streaks and DR in both eyes, and bilateral panretinal photocoagulation was performed for treatment. Vitrectomy had previously been performed in his right eye for vitreous hemorrhage due to PDR. Systemic findings included multiple, discrete, symmetrical, small yellow papules bilaterally in the axilla and inguinal region. Examination on presentation showed vitreous hemorrhage in his left eye, and vitrectomy was performed for treatment. Intraoperative findings showed fibrovascular membrane around the optic disc and vascular arcade. A mottled fundus (peau d'orange appearance) associated with angioid streaks was also present, yet there was no evident choroidal neovascularization (CNV). The postoperative course was satisfactory, and corrected visual acuity improved from 0.02 to 0.7 diopters. CONCLUSION: Despite the peau d'orange appearance in both eyes of this case, no CNV was evident. The vitreous hemorrhage was thus attributed to PDR. Moreover, we reviewed the published literature and discuss the relationship between PXE and DR.

Pseudoxanthoma elasticum-like change adjacent to a benign adnexal neoplasm: a histopathologic reaction pattern.

The progressive calcification and fragmentation of elastic fibers, primarily affecting the skin, are histopathologic features typically associated with pseudoxanthoma elasticum (PXE), pseudo-pseudo PXE, or even acquired PXE. We present a case of a 65-year-old woman, with a known history of breast carcinoma, status after wide excision (T1N0Mx) in 2010, who presented with a 0.6 × 0.5-cm, nontender pink nodule in the right inferior axilla of 1 week duration on the same side as her mastectomy. Histopathologically, the lesion revealed a circumscribed, nonencapsulated bland dermal tumor with poroid and clear cells and foci of ductal differentiation-features diagnostic for hidradenoma. Of note, immediately adjacent to the hidradenoma, the specimen also exhibited short, frayed elastic tissue fibers (positive for EVG and Von Kossa stains)-features reminiscent of PXE. With this case, we expand the spectrum of reaction patterns observed in the skin to include a PXE-like change, a reaction pattern that, to the best of our knowledge, has not been previously reported in association with a benign cutaneous tumor.

Evaluation of arterial grafts prior to coronary bypass in pseudoxanthoma elasticum.

Pseudoxanthoma elasticum is a rare, inherited connective tissue disorder associated with coronary and peripheral arterial disease and accelerated atherosclerosis in medium sized arteries. We describe 110-month symptom-free survival in a patient with pseudoxanthoma elasticum who underwent coronary bypass using the left internal mammary artery at 56 years of age. The vessel was evaluated pre-operatively with angiography to determine suitability, and he has not required further investigation or intervention due to lack of symptoms.

Pseudoxanthoma elasticum.

We report a case of pseudoxanthoma elasticum in a 44-year-old Afro-Caribbean woman who presented with excessive wrinkling and loosening of the skin of the neck. She was treated by platysmoplasty and bilateral neck lift followed by latent excision of nuchal skin folds.

Tumefactive lipedema with pseudoxanthoma elasticum-like microscopic changes.

BACKGROUND: Lipedema is a condition characterized by diffuse, bilaterally symmetrical, painful swelling of the legs and buttocks. Microscopically, there are dermal and septal edema, adipocyte degeneration, and numerous mast cells, features held in common with lipedematous alopecia. CASE REPORT: We present the case of a 60-year-old woman with a long history of bilateral leg masses with microscopic features of lipedema. In addition, elastic-fiber changes typical of pseudoxanthoma elasticum (PXE) were discovered within the subcutaneous septa in three separate specimens obtained from an affected extremity. The patient did not have other clinical findings of PXE, although there was a history of both hypertension and congestive heart failure. CONCLUSION: This tumefactive presentation of lipedema has not been previously described. Regarding the elastic-tissue abnormalities, the patient could have either a subclinical form of PXE, perhaps predisposing to lipedema, or secondary elastic-tissue changes resulting from the massive edema. If the latter is the case, then this could represent an unusual manifestation of localized acquired cutaneous PXE (calcific elastosis).

Cervical midline Z-plasty revision surgery for pseudoxanthoma elasticum.

A 30-year-old woman with known pseudoxanthoma elasticum was referred for surgical correction of extensive loose neck skin. She underwent a standard rhytidectomy, which produced minimal improvement. Revision surgery using a vertical elliptical skin excision, incorporating a Z-plasty was undertaken 3 months later and resulted in a satisfactory outcome.

Pseudoxanthoma elasticum: is the left internal mammary artery a suitable conduit for coronary artery bypass grafting?

Coronary artery revascularization remains a feasible and beneficial treatment for coronary artery disease in patients with pseudoxanthoma elasticum. Careful angiographic evaluation of the left internal mammary artery and coronary arteries is required in patients with pseudoxanthoma elasticum with suspected coronary artery disease. A nonstenosed left internal mammary artery at angiography may be used as a conduit for coronary artery revascularization; however, both the effect of harvest and anastomosis on the disease process in the left internal mammary artery and the long-term patency of left internal mammary artery grafts remain unknown.

Plastic surgery and pseudoxanthoma elasticum.

Pseudoxanthoma elasticum is a cause of abnormal skin laxity. A case of lower face and neck skin laxity is presented with its treatment by cutaneous rhytidectomy.

Pseudoxanthoma elasticum. Case report.

Pseudoxanthoma elasticum is an uncommon disorder of elastin with characteristic skin laxity. Rhytidectomy of the excessive redundant skin is necessary for cosmetic improvement, and reports of surgical management are rare. We report a case of pseudoxanthoma elasticum with involvement of neck, axillae, breasts, abdomen, thighs, and perineum, together with angioid streaking of fundi. Rhytidectomy of the lax cervical skin was undertaken with satisfactory results after a 13 month follow-up. We have reviewed other published reports to obtain a better understanding of the genetics, histopathology, vascular and ophthalmological manifestations, and also the surgical implications of pseudoxanthoma elasticum.

[Grönblad-Strandberg syndrome from the angiological viewpoint]. / Das Grönblad-Strandberg-Syndrom aus angiologischer Sicht.

HISTORY AND CLINICAL FINDINGS: A 42-year-old man was admitted for treatment of peripheral vascular disease in the left leg (stage III of Fontaine). A year before he had undergone a right aortofemoral bypass operation. On admission there was stenosis of the left pelvic axis and bilateral femoral artery occlusion. In addition there were changes in the skin with abnormal folds, loss of elasticity and yellowish spots over the sides of the neck and the flexor surfaces of all large joints. In addition vision in the left eye was impaired. These findings suggested connective tissue disease involving the skin, eye and arterial system. INVESTIGATIONS: Routine haematological tests were normal as were clotting parameters. Serum concentration of GOT, GPT, gamma-GT were slightly increased. There was a dysproteinaemia with raised HDL and LDL levels. Resting electrocardiogram was normal, showing sinus rhythm and left axis deviation. The crurobrachial pressure index was clearly abnormal: 0.6 on the right and 0.5 on the left. Angiography of the pelvic and left arteries revealed long-segment femoral and partial lower-leg occlusions bilaterally. Abdominal sonography indicated diffuse parenchymal calcifications in both kidneys and angioid streaks on bilateral fundoscopy. Skin biopsy showed defects of elastic fibres and perivascular inflammatory infiltration, while capillary microscopy revealed twisting of the capillaries, most of them with normal lumen. These findings taken together indicated pseudoxanthoma elasticum (PXE) or Grönblad-Strandberg syndrome. TREATMENT AND COURSE: A thrombendarterectomy was performed on the left superficial femoral artery, after which the left popliteal artery became palpable, the pressure indices for the left leg were slightly better, and the patient was discharged home without further complications and improved leg perfusion. CONCLUSION: Possible cardiovascular involvement had to be taken into account in patients with PXE, and long-term angiological monitoring is indicated.

A case of actinic granuloma associated with periumbilical perforating pseudoxanthoma elasticum.

We report an unusual case of actinic granuloma of the face and periumbilical perforating pseudoxanthoma elasticum located superior to the umbilicus in a 57-year-old Korean woman. Histopathologically, these two dermatoses have a similar degeneration of elastic fibers, but they show different host reactions to the altered elastic fibers. In the actinic granuloma, actinically damaged elastic fibers were followed by granulomatous infiltration on the sun-exposed area, while in the perforating pseudoxanthoma elasticum, the altered elastic fibers induced a foreign body reaction, with subsequent transepidermal elimination. This is the first case report showing both actinic granuloma and periumbilical perforating pseudoxanthoma elasticum in the same patient, which suggests that the basic mechanism eliciting these dermatoses is similar.

[A case of pseudoxanthoma elasticum underwent coronary artery bypass grafting].

A case of pseudoxanthoma elasticum (PXE) underwent coronary artery grafting is reported. Coronary angiography of a woman with age of 41 with PXE revealed three vessels lesions. Coronary artery bypass grafting using saphenous vein grafts was performed. The left internal mammary artery was not able to use because of obstruction. Her postoperative course is uneventful for 3 years to date. Histological examination of a segment of ascending aorta revealed changes of PXE underwent CABG is extremely rare. This is the third reported case in Japan.

Penicillamine-induced pseudoxanthoma elasticum-like skin changes requiring rhytidectomy.

A 42-year-old woman with pronounced skin laxity of her neck underwent a rhytidectomy and was found to have pseudoxanthoma elasticum-like changes of her skin. Her medical history was significant for Wilson's disease, requiring that she take penicillamine for 26 years. In patients on long-term penicillamine therapy, 20% to 33% will develop a dermatopathy. The drug has been used to alter scar formation in various surgical conditions. Penicillamine is known to alter cross-linking of elastin and collagen fibers. A review of the literature reveals other penicillamine-related dermatopathies that may present to the surgeon.

[Acute arterial thrombosis of the extremity in pseudoxanthoma elasticum]. / Trombosis arterial aguda extremitaria en el pseudoxantoma elástico.

We report a case of a patient with an elastic pseudoxanthoma (PXE) who presented an acute ischaemia at the left lower limb. The cause of such ischaemia was a thrombosis into the iliac and femoropopliteal arteries. Patient underwent a surgical procedure. The arteriopathy associated with a PXE rarely cause an arterial major occlusion. We did not found a case of acute arterial thrombotic ischaemia and PXE, treated with direct arterial revascularization in the reviewed literature.

Plastic surgery in pseudoxanthoma elasticum: experience in nine patients.

Nine women between the ages of 22 and 56 years underwent cosmetic surgery for correction of the severe cutaneous stigmata of pseudoxanthoma elasticum (PXE). The outcome was generally favorable, and follow-up for up to 15 years showed only moderate regression of the skin manifestations. No serious intraoperative or postoperative complications occurred, although tissue friability, poor wound healing, and keloid formation were noted in a minority of persons. An unexpected problem of extrusion of calcium particles through the surgical wound occurred in two individuals. This resulted in delayed healing and unsightly scars.