Cases reported • Crest Syndrome; CRST Syndrome

1/45. peritoneal dialysis-associated peritonitis caused by Propionibacteria species.

There are an increasing number of reports about unusual causes of peritonitis in peritoneal dialysis (PD) patients. The Propionibacteria species is a microorganism that is a normal skin flora. Under the presence of certain risk factors, it may produce serious infections. patients at risk of having Propionibacteria sp infections have malignancy, diabetes mellitus, foreign bodies, or immunodeficiency. We describe a PD-associated peritonitis in a 51-year-old woman that was caused by Propionibacteria sp. This patient's risk factors for developing Propionibacteria sp peritonitis include a history of crest syndrome, malignancy of the breast, and recent catheter surgery. To our knowledge, this is the first case of a PD-associated peritonitis caused by Propionibacteria sp reported in the literature.

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keywords = syndrome
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2/45. "Centripetal flagellate erythema": a cutaneous manifestation associated with dermatomyositis.

We describe 3 patients with dermatomyositis who presented with flagellate erythema. This cutaneous eruption is characterized by erythematous linear lesions on the trunk and proximal extremities. Histologic examination of this eruption in one of our cases revealed an interface dermatitis. Review of the literature and records of 183 patients with connective tissue diseases from our institution has shown that this peculiar eruption has been reported only in dermatomyositis. Because of the location of this eruption, we encourage the use of the term "centripetal flagellate erythema" to distinguish this entity from other linear eruptions seen in patients with connective tissue diseases.

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keywords = disease
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3/45. crest syndrome and periodontal surgery: a case report.

crest syndrome is a slowly progressive form of systemic scleroderma. It is characterized by calcinosis cutis, Raynaud's phenomenon, esophageal involvement, sclerodactyly, and telangiectasia. There are limited reports of dental treatment for patients with this syndrome, and no reports of periodontal surgical procedures. This paper presents a case report of periodontal surgical treatment in a 38-year-old female patient with crest syndrome, and a discussion of the clinical manifestations of the syndrome as they relate to dental treatment.

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keywords = syndrome
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4/45. Phlebosclerosis of the colon with positive anti-centromere antibody.

A 56-year-old woman with symptoms of chronic bowel disease presented a peculiar calcification of the mesenteric vein of the ascending to transverse colon on barium enema study. The resected colon was hard and black. Histo-pathologic examinations demonstrated fibrous change of the colon with a calcified and hyaline-deposited mesenteric vein. No cell infiltration was observed. These findings were compatible with phlebosclerosis and also with systemic sclerosis. Positive anti-centromere antibody and Raynaud's phenomenon, hallmarks of a variant systemic sclerosis, the crest syndrome were observed. We therefore speculated that the pathogenesis of the phlebosclerosis of the colon is related to the crest syndrome.

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ranking = 2.0013987725286
keywords = syndrome, disease
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5/45. A case of crest syndrome and myeloperoxidase-specific anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis.

We report the first case of myeloperoxidase-specific anti-neutrophil cytoplasmic autoantibody (MPO-ANCA)-associated glomerulonephritis in a patient with crest syndrome. A 74-year-old Japanese man with crest syndrome (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia) developed rapidly progressive renal failure without elevation of blood pressure. Renal biopsy revealed glomerular sclerosis and fibrous crescents. The MPO-ANCA titer was elevated to 145 EU/ml. When patients with collagen diseases develop rapidly progressive glomerulonephritis, the possibility of MPO-ANCA-associated glomerulonephritis should be kept in mind.

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ranking = 6.0013987725286
keywords = syndrome, disease
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6/45. Painful trigeminal neuropathy caused by severe mandibular resorption and nerve compression in a patient with systemic sclerosis: case report and literature review.

Systemic sclerosis is a multi-system disorder characterized by abundant fibrosis of the skin, blood vessels, and visceral organs. Although resorption of the mandible has been commonly observed and reported, we found no report of resorption leading to a painful neuropathy of the inferior alveolar nerve. We report a case of a patient with systemic sclerosis, severe resorption of the angles, and inferior alveolar border of the mandible, resulting in a compression neuropathy of the inferior alveolar nerve. Diagnostic tests, medical treatment, and surgical treatment are discussed, and the relevant literature is reviewed.

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keywords = disorder
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7/45. Pulmonary hypertension with limited cutaneous scleroderma (crest syndrome).

A patient is described with a typical manifestation of pulmonary hypertension associated with limited cutaneous scleroderma, also known as crest syndrome. The patient was treated with a calcium antagonist, oral anticoagulation and, because of evidence for parenchymal inflammation of the lung, with low-dose prednisone and cyclophosphamide. This treatment resulted in initial improvement of diffusion capacity and exercise tolerance, however, 1 year after diagnosis the patient died of progressive pulmonary hypertension.

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ranking = 5
keywords = syndrome
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8/45. Granulomatous anterior uveitis in a patient with crest syndrome.

The crest syndrome is a variant form of progressive systemic sclerosis. Apart from the occurrence of keratoconjunctivitis sicca, other types of ocular involvement associated with this variant are quite rare. We present the case of a 73-year-old woman with the CREST variant of progressive systemic sclerosis who developed unilateral granulomatous anterior uveitis. Systemic and laboratory testing failed to suggest evidence for any other associated systemic disease as a possible cause of the granulomatous uveitis. The inflammation was successfully controlled with topical steroids and mydriatics. While a small number of cases of uveitis have been reported in other variant forms of progressive systemic sclerosis, to date there have been no descriptions of uveitis associated with the crest syndrome.

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ranking = 6.0013987725286
keywords = syndrome, disease
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9/45. Autoimmune hepatitis and systemic sclerosis: a new overlap syndrome?

OBJECTIVE: We report the cases of two patients with the complete CREST variant (calcinosis, Raynaud's phenomenon, oesophageal dysmotility, sclerodactyly, telangiectasia) of systemic sclerosis (SSc) who developed autoimmune hepatitis. RESULTS: Our findings suggest that autoimmune hepatitis can be considered to be one of the liver manifestations associated with SSc. Our data also indicate that, because liver involvement may precede skin manifestations, evaluation for SSc is appropriate when autoimmune hepatitis is noted, and that the evaluation should include clinical examination, testing for antinuclear antibodies (especially for anticentromere antibodies) and nailfold capillaroscopy. CONCLUSIONS: From a practical point of view, our two cases emphasize that suspicion of autoimmune hepatitis in SSc patients presenting with cytolytic hepatitis will help to achieve both accurate diagnosis and optimal management.

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ranking = 4
keywords = syndrome
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10/45. A case of porphyria cutanea tarda in association with idiopathic myelofibrosis and crest syndrome.

We report a 56-year-old Korean woman with porphyria cutanea tarda (PCT), showing multiple scarring bullae and hypertrichosis on sun-exposed areas of skin with postinflammatory hyperpigmentation. Sclerodermoid changes were also found on both hands, the face and neck. The patient had suffered from crest syndrome, manifesting with Raynaud's phenomenon and sclerodactyly, for more than 15 years. Anticentromere antibody was positive. She had presented with splenomegaly 3 years before the development of PCT, and was diagnosed as having idiopathic myelofibrosis, based on bone marrow biopsy. In summary, she had had crest syndrome for 15 years and later developed idiopathic myelofibrosis and PCT. This is the first reported case of PCT in association with idiopathic myelofibrosis and crest syndrome.

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ranking = 7
keywords = syndrome
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