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1/7. Systemic sclerosis sine scleroderma: is it always the same disease? Report of three patients and discussion.

    The recent description of a large cohort of patients with the diagnosis of systemic sclerosis sine scleroderma (ssSSc) provided significant progress in our understanding of this entity. The prognosis of patients with ssSSc is, however, very variable, from benign in most cases to rapidly disabling in others. By reporting three new cases and analyzing previously published data, we discuss possible subsets and variants of the disease form.
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2/7. Cardiac anomalies complicating congenital contractural arachnodactyly.

    A newborn boy with congenital contractural arachnodactyly (CCA) was found to have an atrial septal defect, ventricular septal defect, patent ductus arteriosus, and aortic arch anomalies. These resulted in congestive failure but subsequent progressive dilatation of both great arteries and development of aortic regurgitation were associated with eventual cardiorespiratory failure and death at 11.5 months. Others have noted that cardiac anomalies in CCA may be comparable with those of infantile Marfan's syndrome. Our case further extends the spectrum of cardiac abnormalities in CCA and substantiates that there is overlap with infantile Marfan's syndrome. This fact and our literature review suggest that early and complete cardiac assessment is necessary for appropriate management and evaluation of prognosis in any newborn infant with findings suggestive of either condition.
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3/7. Anti-Ku antibodies in connective tissue diseases. Report of three cases.

    Three patients are described with clinical features of connective tissue diseases, namely, dermatomyositis, progressive systemic scleroderma, and systemic lupus erythematosus. In two patients the symptoms of disease overlapped. The rare anti-Ku antibody was found in all of them. Anti-Ku antibody characterizes those patients, with polymyositis-scleroderma overlap syndromes who have a good prognosis. One of our patients, who also had severe anti-Sm-positive systemic lupus erythematosus, seems to be an exception.
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4/7. Congenital heart block and cardiac anomalies in the absence of maternal connective tissue disease.

    The association of congenital heart block with maternal connective tissue disease and autoimmunity has been recently reported. Two cases of heart block were diagnosed in utero at 23 and 24 weeks' gestation. Both mothers had extensive workups for connective tissue disease that were negative. Two-dimensional and Doppler echocardiography were performed in utero and both infants had left atrial isomerism, atrioventricular canal defects, and severe hydrops in addition to heart block. review of the recent literature identifies a subset of congenital heart block in which the mothers have no evidence for connective tissue disease, and the fetus or neonate has complex cardiac malformations and hydrops. The common features in these babies are atrioventricular canal defects and isomerism. No reports of congenital heart disease among the children of mothers with connective tissue disease fit this description. Our recent experience and literature review emphasize the previously known association between congenital heart block and congenital heart disease. This association is especially important in light of the poor prognosis for this group of fetuses and neonates.
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5/7. The Wagner-Stickler syndrome: a study of 22 families.

    The Wagner-Stickler syndrome is a hereditary progressive arthro-ophthalmopathy with an autosomal dominant pattern of inheritance. Affected persons may have a wide variety of ocular, orofacial, and skeletal problems. We examined 22 index patients and 68 of their relatives. Of these 90 persons (41 in the pediatric age group), 70 were found to have the syndrome. We determined the frequency of the various problems and identified several progressive features. We established an approximate age of onset in the group with known retinal disease and in the asymptomatic group identified by family screening. Although the latter group was initially more mildly affected, they were at risk to develop serious ocular problems. Screening all relatives of affected persons for nonocular features of the syndrome should permit early diagnosis in the asymptomatic group and improve the long-term prognosis.
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6/7. "Great mimicry" in a patient with tetraparesis: a case report.

    The patient is a 63-year-old Chinese man who presented with tetraparesis and urinary incontinence. The initial diagnosis was cord compression from cervical spondylosis. The patient relapsed 3 months after cervical laminectomy. The transverse myelitis picture, left optic atrophy and suggestive brainstem evoked potentials led to treatment of a presumptive demyelinating process. The presence of vitiligo, however, led to detection of high titers of antinuclear antibodies (ANA) and presence of anti-nonhistone antibodies. The patient was then diagnosed to have a lupus (SLE)-like disease, which has not fully evolved. He was prescribed pulsed cyclophosphamide and prednisolone with significant gains both neurologically and functionally up to 1 year of follow-up. This report highlights the befuddling impact the disease process have on the clinicians in terms of diagnosis, treatment, and prognosis. That it can occur in men in the seventh decade of life heightens the need for awareness in our approach to the myelopathic patient.
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7/7. adult T-cell leukemia/lymphoma associated with noninfectious epithelioid granuloma in the skin: a clinicopathologic study.

    Hodgkin's disease (HD) and non-Hodgkin's lymphoma (NHL) are infrequently associated with noninfectious granulomas in involved or noninvolved organs. adult T-cell leukemia/lymphoma (ATLL) is an aggressive lymphoproliferative neoplasm associated with T-lymphotropic virus type 1 (HTLV-1). We describe a case of cutaneous type ATLL, affecting mainly the skin as a maculopapular eruption, in which some skin biopsies contained epithelioid cell granulomas in the lymphoma cutis (ATLL) lesion. These Lennert's-like epithelioid clusters were also present in lymph nodes, which showed some degree of invasion by the ATLL lymphocytes. Although prognosis of ATLL is generally poor, our patient has had a less aggressive course, with a survival time to date of 13 years. Our findings suggest that the presence of epithelioid granulomata in an ATLL patient may be a manifestation of a host response which confers some protection against the disease progression. To our knowledge, this is the first report of a case of ATLL with a noninfectious granuloma similar to a Lennert's lesion.
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