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Query: UMLS:C0851184 (thinning)
 11,252 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Progressive hemifacial atrophy is an unusual craniofacial deformity that is amenable to surgical reconstruction. The disease may involve any layer of the superficial facial tissues as well as cartilage and bone. While the cause of the disease is unknown, several hypotheses exist to account for the progressive nature of the disorder. Histologically, the skin undergoes thinning of the Malpighian stratum and atrophy of the skin adenexa and dermal units. The subcutaneous tissue shows chronic inflammatory changes. This picture closely resembles that of scleroderma, and some authors believe the disorder to be an isolated form of scleroderma. Various hypotheses have been promulgated as to the cause of this disorder, its pathologic process, and varied clinical presentation.
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PMID:Hemifacial atrophy. A review of an unusual craniofacial deformity with a report of a case. 68 22

Raynaud's phenomenon, uncommon in childhood, often heralds connective tissue disorder. Since microvascular abnormalities can be detected at an early stage of the connective tissue disease, especially in scleroderma, a specific diagnosis can be made in patients presenting with Raynaud's phenomenon alone or Raynaud's phenomenon associated with symptoms suggestive of connective tissue disease. Raynaud's phenomenon was studied in 11 consecutive children, 10 girls and 1 boy, ages 6 to 15. One child had a definite diagnosis of cutaneous polyarteritis nodosa. In 6 others connective tissue disease was suspected: 4 had arthritis, 2 has telangiectasia, leg ulcers and antinuclear antibodies. Of the remaining 4, one had hemiplegia and 3 Raynaud's phenomenon only. Oscillometry of the radial artery was reduced in 7 of 9. Decreased capillary resistance was found in 2 of 6, while abrupt thinning in conjunctival vessels was seen in 3 of 7. On nailfold capillaroscopy, reduced vascularity was noted in 5 of 11, dilated capillaries in 4 of 11, tortuousity in 2 of 11, capillary thinning in 1 of 11, capillary spasm in 1 of 11 and normal pattern in 3 of 11. Two patients presenting with Raynaud's phenomenon were found to have "scleroderma-like pattern" on nailfold capillaroscopy. One of them died 2 years later of cardiopulmonary sclerosis, and another developed esophageal stricture and Barrett's esophagus. Neither has sclerodermatous skin. In childhood Raynaud's phenomenon, nailfold capillaroscopy is a non-invasive examination enabling early diagnosis of "systemic scleroderma sine scleroderma".
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PMID:Raynaud's features in childhood. Clinical, immunological and capillaroscopic study. 149 54

Scleroderma or Systemic Sclerosis (SSC) is a disorder characterized by fibrosis of the skin and multiple internal organs. The pathological lesion is a triad of small artery intimal proliferation, medial thinning and adventitial scarring. Autoamputation of fingers and toes is often seen, but only a few cases of limb amputation in scleroderma patients have been reported. The Pittsburgh Scleroderma databank includes 1,030 patients with SSC. Among these were seven patients who sustained lower limb amputation. There were four patients with the CREST variant of SSC, two with diffuse scleroderma, and one who had SSC/rheumatoid arthritis/polymyositis overlap who sustained limb amputation. Of the seven, three were male and five had a significant smoking history. Ages ranged from 46 to 71 years. All patients underwent amputation for nonhealing ulcerations. No problems with postoperative wound healing were seen. Pathologic changes typical of SSC in addition to atherosclerotic peripheral vascular disease were described in one case. Three patients were successfully fitted with prostheses and became independent ambulators. Four patients could not be fitted with prostheses. No skin problems were reported related to prosthetic use. Our review demonstrates that SSC patients who undergo amputation can become successful prosthetic users and should be considered for prosthetic prescription.
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PMID:Lower extremity amputation in scleroderma. 151 89

Scleroderma is a rare disease in children: the clinical presentation in childhood is even more varied than in adult life. It is characterized by 'hard skin' with cutaneous features including hypo- and hyperpigmentation, thickening or thinning and loss of elasticity. It ranges from circumscribed and self-limiting pigmentary disorders to disabling and disfiguring involvement of an extremity and a rapidly fatal outcome. Scleroderma must be differentiated from many scleroderma-like conditions. Therapeutic problems are also discussed.
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PMID:[Localized scleroderma in childhood]. 318 14

Although nailfold capillary abnormalities associated with connective tissue disease (CTD) have been studied by direct in vivo microscopy, little is known of the underlying histology and morphology of this tissue. This report summarizes light microscopic study of glycolmethacrylate embedded nailfold biopsies from 13 CTD patients (9 scleroderma, 2 CREST, 2 undifferentiated CTD), 2 subjects with Raynaud's phenomenon alone, and 9 normal volunteers of similar age and sex distribution. The most striking and consistent finding was the presence of globular, eosinophilic, PAS-positive deposits in the cuticles of 14 of 15 patients and none of the controls. This material, identified by immunofluorescent staining as serum protein exudates, was associated with pronounced parakeratosis and elevated epithelial mitotic activity. Capillary ectasia with thinning of the basement membrane was often present in CTD biopsies. Occasional signs of endothelial swelling and proliferation were encountered in both populations. Inflammatory changes were rarely seen. In quantitative comparison with control tissues, the superficial dermis from CTD patients contained significantly fewer capillaries, cutaneous nerve bundles, and interstitial fibroblasts per unit area and fewer papillary capillaries per unit of epidermal length. Measures of capillary density in sectioned tissue correlated well with the results of in vivo microscopic examination.
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PMID:Nailfold biopsy in scleroderma and related disorders. Correlation of histologic, capillaroscopic, and clinical data. 669 62

A prospective study in 30 patients with systemic sclerosis included phonocardiographic (PCG) recordings in all cases and an echocardiogram (ECHO) in 18 patients. A specific diagnosis of myocardial fibrosis was established in 2 cases, based on anatomical criteria, and was suspected in 5 patients from combined clinical, electrical, and radiological findings. Ventricular diameters, the slope of mitral valve closure, and systolic parameters of ventricular function (haemodynamic quotient, left ventricle ejection fraction, and mean rate of circumferential shortening) were rarely abnormal, and did not correlate with possible myocardial fibrosis. Diastolic pick-up (PCG) was abnormal in 30 p. cent of the cases but this is not specific to myocardial fibrosis. Total myocardial relaxation interval, as measured by PCG, was significantly increased to 146 +/- 23 ms (mean +/- one standard deviation) in relation to a control group (p less than 0.001). A relaxation interval (measured by PCG and/or ECHO) superior to 150 ms is suggestive of myocardial fibrosis. The rate of thinning of the left ventricular posterior wall was diminished in the 14 cases in which it was measured. These results are in favour of a myocardial relaxation disorder during scleroderma.
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PMID:[Sclerodermic cardiomyopathy : prospective study employing phonocardiography and echocardiography. Value of diastolic parameters of ventricular function (author's transl)]. 708 70

Werner's syndrome is a rare autosomal recessive disorder that affects connective tissue throughout the body. The genetic basis is not yet known, although many laboratory abnormalities have been reported. The manifestations are widespread, and many organs may prematurely undergo changes usually associated with aging. The disease generally becomes apparent around puberty, with growth arrest and thinning and graying of hair. Rapidly progressing bilateral cataracts typically occur when patients are in their 20s and 30s. A dermatologist may be consulted because of the scleroderma-like appearance of the skin, lower-extremity ulcers or calluses, thinning and graying of hair or baldness, nail dystrophy or loss, wrinkling and aging of the face, or skin cancers. Patients should have a thorough clinical and laboratory work-up, keeping in mind their elevated risk for neoplasms.
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PMID:Werner's syndrome. 771 42

Systemic scleroderma is a progressive multi-system connective tissue disease. Ocular involvement includes keratoconjunctivitis sicca, progressive shallowing of conjunctival fornices, peripheral ulcerative keratitis and eyelid tightness. No association has been reported between scleroderma and pellucid marginal degeneration, which is a rare bilateral corneal ectasia. Pellucid marginal degeneration is characterised by non-inflammatory and progressive peripheral corneal thinning inferiorly, often with high against-the-rule astigmatism. We report a case of a 55-year-old woman with systemic scleroderma who presented with rapidly progressing against-the-rule astigmatism. The differential diagnosis of peripheral corneal thinning and the challenge of the surgical management of pellucid marginal degeneration are briefly discussed.
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PMID:Pellucid marginal degeneration and scleroderma. 1518 10

A 49-year-old man was first seen in our department for the evaluation of scleroderma-like skin changes and a nonhealing ulcer on his leg from years before referral. His medical history was of long duration. His growth was stunted at the age of 12. At 21 years of age, the patient noted graying of the scalp hair, most prominent on his temples, and the process was progressively completed by the age of 23. At the same age, atrophy and thinning of the skin and loss of subcutaneous fat resulted in a tense, shining, and adherent appearance of his skin, most obvious on his face and extremities. Soon after, he developed a high-pitched, hoarse voice. He had undergone bilateral cataract surgery at the age of 30. Around the age of 46, he developed a unilateral nonhealing chronic leg ulcer (Figure 1). He had separated from his wife because of infertility. He was the first offspring of his second-degree healthy relative parents. The other 3 siblings had similar signs and symptoms. Our patient gave the history of premature graying of the hair of his younger brother at the age of 18 and his 2 younger sisters at the age of 12 and 16. His brother had recently received diagnoses of bilateral cataract and diabetes mellitus. All of the siblings had ceased growth from early adolescence. On physical examination, our patient's weight was 48 kg and his height was 150 cm. He had normal intelligence. He was speaking with a high-pitched and childish voice. He had a bird-like appearance with a beak-shaped nose. Mottled and diffuse pigmentation and poikiloderma appearance was conspicuous on his neck (Figure 2). The entire skin was smooth, shiny, and scleroderma-like, and a marked decrease in the subcutaneous fat was noted over the extremities. A deep cutaneous ulcer was evident on his slimmed leg. Digital ulcers were not found, and radial and dorsalis pedis pulses were palpable. Clinodactyly of the toes were conspicuous. His nails were dystrophic and he had used dentures from the age of 20. On examination of the external genitalia, his testes were smaller than normal. In the biopsy taken from the leg ulcer, there were no signs of malignancy. There were no signs ofosteomyelitis on x-ray. Biopsy of the normal skin revealed atrophic epidermis and thick dermis with hyalinization of the collagen fibers and absence of pilosebaceous structures (Figure 3). The patient's scalp hair was thin and sparse and there were few axillary and pubic hairs. His fasting plasma glucose level was normal.
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PMID:Werner syndrome in an Iranian family. 2113 29

Linear scleroderma is a rare variant of localised scleroderma, which is usually seen in childhood and during the adolescent period, and can cause severe functional morbidity as well as cosmetic and psychological problems. Although its ethiopathogenesis is yet obscure, autoimmunity, local ischaemia and injuries, vaccination, irradiation, vitamin K injections, Borrelia burgdorferi and Varicella infections have been incriminated. A 4-year-old girl who had been followed up for about 18 months with diagnosis of epilepsy had a colour discolouration and depression that first appeared 1 year ago and then progressed on her left frontal region. Her CT scan showed a thinning in the frontal bone and depression in the frontal region. These findings are described as 'en coup de sabre' a rare form of linear scleroderma localised at the frontal region of the scalp. In this paper, we present clinical and radiological findings of a 4-year-old girl with epileptic seizures that started 1 year before the onset of the lesion of linear scleroderma.
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PMID:Frontal linear scleroderma en coup de sabre associated with epileptic seizure. 2323 Feb 61


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