Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pseudoxanthoma elasticum (PXE) is a heritable connective tissue disease involving progressive fragmentation and dystrophic calcification of elastic fibers. Periumbilical disease as the exclusive site of cutaneous involvement is most commonly seen in the rare entity termed periumbilical perforating pseudoxanthoma elasticum (PPPXE). Patients with this disorder are generally obese, middle aged, multiparous black women with hypertension. The cutaneous lesions are well-demarcated, hyperpigmented, periumbilical plaques with keratotic papules on the periphery. Extracutaneous manifestations have rarely been described. We describe a patient with periumbilical PXE associated with chronic renal failure and bilateral angioid streaks. Histopathologic examination demonstrated typical calcification of elastic fibers with additional amorphous calcium deposits in the superficial dermis. Transepidermal elimination was not present. Normalization of the serum calcium-phosphate product resulted in regression of the lesions--both clinically and histopathologically. The relation between PPPXE and hereditary PXE is discussed. The role of chronic renal failure in precipitating PPPXE is considered.
J Am Acad Dermatol 1998 Aug
PMID:Periumbilical pseudoxanthoma elasticum associated with chronic renal failure and angioid streaks--apparent regression with hemodialysis. 970 48

Growth factors produced by a variety of cells act as signalling peptides through specific cell surface receptor pathways. Functions such as cell proliferation, migration and differentiation have been assigned to each of them. Here, we report alterations of platelet-derived growth factor receptor alpha (PDGFR-alpha) and beta (PDGFR-beta) and vascular endothelial growth factor (VEGF) expression patterns in the progressive clinical stages of chronic venous insufficiency (CVI). A total of 30 punch biopsies were taken from patients with CVI, and VEGF and PDGFR were detected by indirect immunofluorescence and immunoperoxidase techniques. PDGFR-alpha and PDGFR-beta expression was strongly increased in endothelial cells of capillaries, pericapillary cells and connective tissue cells in the stroma of the skin of venous eczema and venous leg ulcer patients, and to a smaller extend in the dermis of those with lipodermatosclerosis. VEGF staining showed a similar expression pattern in the progressive CVI stages. However, staining of vessels in particular might simply reflect binding of VEGF, secreted by keratinocytes or fibroblasts, to its receptors. Growth factor and receptor expression in specimens from telangiectases and reticular veins, and from pigmented areas, resembled that of normal skin. We conclude that PDGFR-alpha, PDGFR-beta and VEGF play an important role in mediating inflammation and epithelial hyperproliferation in venous eczema, inducing connective tissue sclerosis in lipodermatosclerosis, and causing the reduced reepithelialization tendency in venous ulcers. We speculate that endothelial proliferation with chronic venous hypertension might be mediated by these growth factors.
Arch Dermatol Res 1998 Jun
PMID:Increased expression of platelet-derived growth factor receptor alpha and beta and vascular endothelial growth factor in the skin of patients with chronic venous insufficiency. 970 59

Thirteen psoriatic patients with hypertension during the course of cyclosporin A therapy were treated for 25 months with a calcium channel blocker, sustained-release nifedipine, to study the clinical antihypertensive effects and adverse events during treatment with both drugs. Seven of the 13 patients had exhibited a subclinical hypertensive state before cyclosporin A therapy. Both systolic and diastolic blood pressures of these 13 patients were decreased significantly after 4 weeks of nifedipine therapy, and blood pressure was maintained within the normal range thereafter for 25 months. The adverse events during combined therapy with cyclosporin A and nifedipine included an increase in blood urea nitrogen levels in 9 of the 13 patients and development of gingival hyperplasia in 2 of the 13 patients. Our findings indicate that sustained-release nifedipine is useful for hypertensive psoriatic patients under long-term treatment with cyclosporin A, but that these patients should be monitored for gingival hyperplasia.
Eur J Dermatol 1998 Dec
PMID:Long-term efficacy and adverse event of nifedipine sustained-release tablets for cyclosporin A-induced hypertension in patients with psoriasis. 988 29

We previously showed in a double-blind, placebo-controlled study that cyclosporin at a dose of 2.5 mg/kg per day is an effective treatment for palmoplantar pustulosis (PPP). In the present randomized, double-blind, placebo-controlled multicentre study we treated 58 PPP patients with placebo or cyclosporin at an initial dose of 1 mg/kg per day. Disease activity was calculated from the number of fresh pustules. Treatment success was defined as the number of fresh pustules not exceeding 50% of the patients' own baseline pustule number. In cases of treatment success the dose of the test medication was not increased and the treatment was kept blinded for a maximum of 12 months. Blinding was broken only on treatment failure of the initial test medication dose. The mean blinded treatment time was 5.1 months for the patients receiving cyclosporin and 2.1 months for placebo (P < 0.01). Treatment was kept blinded for 12 months for seven patients in the cyclosporin and two in the placebo group (P < 0.05). Patients whose treatment code was broken continued in an open dose-finding part of the study with dose adjustments of cyclosporin every second month. In cases of treatment failure the dose of cyclosporin was increased in steps of 1 mg/kg per day; in cases of treatment success the cyclosporin dose was decreased by 1 mg/kg per day. The minimum and maximum doses were 1 and 4 mg/kg per day, respectively. The mean effective dose during the dose-finding part was between 1.2 and 1.7 mg/kg per day. Two patients did not respond to the highest dose of 4 mg/kg per day. In two patients serum creatinine levels increased by > 30% of their own baseline. The other main adverse events were hypertension (seven patients) and hypertrichosis (six patients). After stopping cyclosporin treatment the mean number of fresh pustules showed a maximum after 2 weeks with a continuous decline after that. Twelve months after completing the treatment the mean number of pustules was reduced to 20.0 compared with 63.6 at baseline (P < 0.001); 11 patients were free from pustules and two of these were totally cleared. We conclude that cyclosporin at 1-2 mg/kg per day is an effective and well tolerated treatment for PPP in most patients.
Br J Dermatol 1998 Dec
PMID:Double-blind placebo-controlled study of long-term low-dose cyclosporin in the treatment of palmoplantar pustulosis. 999 Mar 62

We report a patient with anemic macules over cyanotic skin which started in the 8th month of a normal pregnancy. We thought that these lesions were close to Bier's spot description although it has never been described during pregnancy. The relationship with pregnancy is likely because skin lesions regress in post-partum. It probably represents an exaggerated physiological response of small vessels to venous hypertension observed during pregnancy in predisposed women.
Eur J Dermatol
PMID:Numerous and large Bier's spot associated with pregnancy. 1021 Jul 92

An 82-year-old woman was seen at our Dermatology Department for a plaque on the right parietal scalp that had recently increased in size, and bled. The lesion had been present for 3 months. The patient had a previous diagnosis of chronic bronchitis, noninsulin-dependent diabetes mellitus, and hypertension, but no previous history of cancer. Physical examination revealed a 7 x 10 cm plaque, composed of a central necrotic and bleeding surface, surrounded by small purple-red satellite nodules. A biopsy showed an ill-defined infiltrative intradermal mass with a pattern of hypercellular sheets of large cells alternating with areas of dilated, irregular, blood-filled channels, dissecting the collagen bundles. The endothelial cells lining these channels were plump and pleomorphic, surrounded by other spindle-shaped cells with pleomorphic and atypical nuclei. The diagnosis of angiosarcoma was made, and the patient was sent to an oncology center for further evaluation and treatment, where a computed tomography head scan was taken revealing no erosion of the skull. The patient refused surgery, so radiotherapy was proposed. One month later, she developed lymph node enlargement of the left anterior cervical nodes. A needle aspiration biopsy was consistent with sarcoma. Two weeks later, she was started on palliative radiotherapy: a programmed dose of 4500 cGy was proposed of which she only received 3000 cGy because of treatment withdrawal and loss to follow-up. During this time, she showed partial initial response, but despite treatment the disease relentlessly progressed, with hemorrhage and severe pain being the most striking features.
Int J Dermatol 1999 Sep
PMID:Angiosarcoma of the scalp. 1051 88

A 58-year-old man had long-standing lesions of presumed large plaque parapsoriasis. Following treatment for nodal Hodgkin's disease (HD), these became more infiltrated, with a diagnosis of mycosis fungoides (MF). A few months later, nodules appeared on the right leg, which was lymphoedematous after inguinal irradiation for HD. Histopathological examination showed CD3+, CD30-, CD15- large pleomorphic lymphocytes, leading to the diagnosis of transformed MF. The cutaneous lesions were successfully treated with topical nitrogen mustard and interferon alfa-2b then methotrexate, but his general health worsened with depression and malaise, without specific neurological symptoms or extracutaneous spreading of the lymphoma. Cerebral computed tomographic scan revealed a cerebellar subdural collection, arachnoid cyst and quadriventricular hydrocephaly, initially considered to be non-specific. After a few weeks, clinical symptoms of intracranial hypertension appeared, and a cerebrospinal fluid (CSF) examination revealed meningeal involvement by the lymphoma. These cells were CD3-negative and the diagnosis was confirmed by polymerase chain reaction (PCR) study, which revealed an identical clonal rearrangement of the T-cell receptor gamma gene between cutaneous biopsies and the CSF. Repeated intrathecal injections of methotrexate and cranial irradiation were performed and the patient was still alive after 13 months. This case illustrates the possible meningeal involvement of MF that may be preceded by atypical and mild neurological or psychiatric symptoms, which may be dissociated from the evolution of the cutaneous lesions. Moreover, PCR study may be useful for both diagnosis and monitoring.
Br J Dermatol 1999 Nov
PMID:Meningeal involvement by a transformed mycosis fungoides following Hodgkin's disease. 1058 78

Pseudo-Kaposi's sarcoma which develops due to arteriovenous fistulae for haemodialysis is relatively well known. In contrast, nail changes associated with pseudo-Kaposi's sarcoma or venous hypertension are rare. We report a novel case of pincer nail deformity associated with pseudo-Kaposi's sarcoma and venous hypertension, complications of an arteriovenous fistula for haemodialysis, and review eight similar cases reported in the literature. Most of the subjects presented with similar findings, having circulatory disturbance due to an arteriovenous fistula and/or increased venous pressure, and swelling, discoloration and papules/nodules of the skin distal to the shunt. Of the nine patients, three had overcurvature of the nails, in which the lateral edge of the nail pressed deeply into the lateral nail fold. Pincer nail deformity associated with pseudo-Kaposi's sarcoma after placement of an arteriovenous fistula may be relatively common and should be recognized as a specific sign of circulatory disturbance due to the arteriovenous fistula.
Br J Dermatol 1999 Dec
PMID:Pincer nail deformity and pseudo-Kaposi's sarcoma: complications of an artificial arteriovenous fistula for haemodialysis. 1060 67

We studied 20 Caucasian middle-aged patients, mostly women, who had asymptomatic, gray-brown patches with geographic borders, occasionally interspersed with hypopigmented macules, on the dorsum of the forearms, mostly bilaterally. The distal aspect of the arms was also involved in 2 patients; the face was always spared. Civatte's poikiloderma was associated with this hyperpigmentation in 9 patients. Epidermal atrophy, basal layer hyperpigmentation, elastosis and angiectases were histopathologic features. Pregnancy, menopause, local inflammation, and cosmetics were not predisposing factors. Undue sun exposure, oral contraceptives or estrogens were found only occasionally. Sixty-five percent of patients had hypertension and had been taking antihypertensive drugs, especially angiotensin-converting enzyme inhibitors, for years before pigmentation began. Acquired brachial cutaneous dyschromatosis (ABCD) is the suggested title of this disorder, which is not rare. Its prevalence in postmenopausal women, the hypopigmented macules, and the absence of a relation with estrogens, pregnancy, or hormone replacement therapy, all help to distinguish ABCD from melasma. An association with Civatte's poikiloderma as well as hypertension and/or antihypertensive drugs is suggested.
J Am Acad Dermatol 2000 Apr
PMID:Acquired brachial cutaneous dyschromatosis: a common pigmentary disorder of the arm in middle-aged women. 1072 20

A 70-year-old patient with a history of hypertension and hypercholesterolemia was referred for evaluation of necrotic toes. The patient had a history of several cerebrovascular accidents during the previous month. Initially, she developed sudden-onset left upper extremity weakness which, over the ensuing 4 days, progressed to complete left-sided weakness. This was followed by the development of acute dysarthria. A transesophageal echocardiogram revealed moderate left ventricular hypertrophy, several vegetations on her tri-leaflet aortic valve associated with moderate aortic regurgitation, and a large right atrial thrombus with a mobile component. Bubble studies failed to reveal any septal defects. The patient's electrocardiogram was nonspecific. As serial blood cultures were negative despite fevers of up to 39.8 degrees C, the patient was treated with a 6-week course of intravenous ceftriaxone, ampicillin, gentamicin, and ciprofloxacin for a presumed diagnosis of culture-negative endocarditis. Fungal cultures of the blood were negative. The patient, however, progressed and developed several necrotic toes. Physical examination was significant for ischemic changes of the left first, second, third, and fifth toes, as well as the right first and second toes. Diffuse subungual splinter hemorrhages in the toenails, numerous 2-4-mm palpable purpuric papules on the lower extremities, and nontender hemorrhagic lesions of the soles were also noted. Peripheral and carotid pulses were intact and no carotid bruits were heard. Cardiopulmonary and abdominal examinations were unremarkable. Neurologic examination revealed a disoriented, dysarthric patient with left central facial nerve paralysis, as well as spasticity, hyperactive reflexes, and diminished strength and sensation in the left upper and lower extremities. A left visual field defect and left hemineglect were also present. The patient's last brain computerized tomogram revealed areas of low attenuation consistent with cerebral infarctions in three distinct areas of the brain. These included the left occipitotemporal area, the right parieto-occipital area, and the right posterior frontal region. The regions affected were in the distribution of both the anterior and posterior circulation. No evidence of hemorrhage was noted. The patient subsequently complained of abdominal discomfort. A computerized tomogram of the abdomen with oral and intravenous contrast revealed a 4-cm x 3-cm irregular mass in the tail of the pancreas with several low-attenuation lesions throughout the liver which were consistent with infarctions or metastases. Several splenic infarctions were also present. A biopsy of the tumor revealed pancreatic adenocarcinoma. The patient's carcinoembryonic antigen level was 18. 4 ng/mL (0-3) and the CA 19-9 antigen level was 207,000 U/mL (0-36). The alpha-fetoprotein level was normal. Other significant laboratory findings included a prothrombin time of 16.7 (international normalized ratio, 1.4), an activated partial thromboplastin time of 32 (ratio, 1.3), and a platelet count of 85,000/mm3. The Russell viper venom time, sedimentation rate, and C3 levels were normal, and the patient was negative for antinuclear antibodies, anticardiolipin antibodies, and antibodies to extractable nuclear antigens. Of note, the patient was not receiving any anticoagulation. Blood cultures for mycobacteria and fungi, human immunodeficiency virus serology, and urinalysis and culture were negative. The patient subsequently developed an inferior wall myocardial infarction and was transferred to the coronary care unit. In line with the family's request, aggressive care was ceased and the patient expired. The patient's family refused an autopsy.
Int J Dermatol 2000 Apr
PMID:Cutaneous manifestations of marantic endocarditis. 1080 80


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