Cases reported "Scleroderma, Localized"

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1/19. Macroscopic and microscopic mucinosis in chronic sclerodermoid graft-versus-host disease.

    Secondary cutaneous mucinosis is a well-recognized feature of connective tissue diseases such as lupus erythematosus and dermatomyositis. We report the first three cases of dermal mucinosis in association with severe chronic cutaneous graft-versus-host disease of the sclerodermoid variety. One patient had clinical changes due to abundant mucin accumulation within the papillary dermis (mucinoma). In the other two patients histological examination revealed extensive deposits of mucin predominantly within the reticular dermis. The microscopic appearances were striking, with numerous vacuolated spaces interspersed between collagen bundles. We speculate that this appearance is the result of ground substance becoming trapped within grossly sclerodermoid connective tissue.
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ranking = 1
keywords = lupus erythematosus, erythematosus, lupus
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2/19. lichen sclerosus et atrophicus, bullous morphea, and systemic lupus erythematosus: a case report.

    lichen sclerosus et atrophicus (LSA) rarely coexists with morphea, especially when bullae occur in lesions of morphea. Here we report the case of a 15-year-old girl with this condition, who also fulfilled four out of 11 diagnostic criteria for systemic lupus erythematosus (SLE). Tissue biopsy of different skin lesions showed LSA in the regions of bullous morphea, that has rarely been reported in the literature.
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ranking = 5.2697002080739
keywords = lupus erythematosus, erythematosus, lupus, systemic lupus erythematosus, systemic lupus
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3/19. Coexistent linear scleroderma and juvenile systemic lupus erythematosus.

    We describe a girl who initially presented with linear scleroderma. Five and a half years later she developed systemic lupus erythematosus (SLE). Previous descriptions of the coexistence of linear scleroderma and SLE in childhood are reviewed.
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ranking = 5.2697002080739
keywords = lupus erythematosus, erythematosus, lupus, systemic lupus erythematosus, systemic lupus
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4/19. Necrotizing vasculitis in a patient with generalized morphea.

    Generalized morphea is rarely associated with systemic overlap. We report an unusual case with generalized morphea involving cutaneous large vessel vasculitis, mononeuritis multiplex, and lupus anticoagulant without any evidence of the coexistent systemic lupus erythematosus.
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ranking = 1.1026595947598
keywords = lupus erythematosus, erythematosus, lupus, systemic lupus erythematosus, systemic lupus
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5/19. Linear morphea with secondary cutaneous mucinosis.

    Secondary mucinosis is a common finding in connective tissue diseases, especially in lupus erythematosus and dermatomyositis, but is seen only rarely in morphea. We report the case of a 9-year-old boy who presented with linearly arranged, flesh-colored to erythematous, indurated, very tender plaques on his right arm. He had similar lesions on his midchest and upper back. Histopathology revealed the characteristic findings of morphea and mucin deposition between thickened collagen bundles. This is an unusual case of linear morphea with hyaluronic acid deposition.
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ranking = 1
keywords = lupus erythematosus, erythematosus, lupus
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6/19. Sclerodermic linear lupus panniculitis: report of two cases.

    Lupus erythematosus panniculitis is a rare disease characterized by deep subcutaneous nodules, most commonly localized on the upper limbs and face. Unique clinical presentations, such as linear configuration or 'overlap' forms between lupus erythematosus panniculitis and localized scleroderma have been reported. We present here the clinical characteristics, course and laboratory findings of 2 patients having linear lupus erythematosus panniculitis with localized scleroderma-like changes. The 2 patients (of the 14 patients with lupus erythematosus panniculitis seen by us since 1990) were females with a young age at the onset of disease (median, 25 years). In 1 case, evolution into systemic lupus erythematosus with severe renal involvement occurred whereas the other patient, who had a spontaneous abortion and exhibited anticardiolipin antibodies, should be followed and screened for the emergence of antiphospholipid syndrome. Thus, the clinical behavior of this variant seems to be more aggressive, as compared with the usual course of lupus erythematosus panniculitis, which is considered to be a benign disease, although some reports have suggested that its prognosis is not always favorable. The linear distribution could be the clinical hallmark of such a unique, 'sclerodermic' subset of lupus erythematosus panniculitis.
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ranking = 6.5515184203413
keywords = lupus erythematosus, erythematosus, lupus, systemic lupus erythematosus, systemic lupus
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7/19. Systemic sclerosis with unusual panniculitis and overlying discoid lupus erythematosus-like lesions.

    A 33-year-old men presented with systemic sclerosis accompanied by both unusual panniculitis and overlying discoid lupus erythematosus (DLE)-like skin changes on the left buttock. The lesion did not completely match either lupus erythematosus profundus or morphea profunda but featured clinical and pathological findings halfway between the two entities. No case report of co-existence of systemic sclerosis and morphea profunda was found in the literature, but there are three case reports of patients with systemic sclerosis and lupus erythematosus profundus. One of the three cases had DLE-like changes on the surface of the lesion, a clinical picture similar to our case. We speculated that the DLE-like changes in the overlying skin were due to local tissue ischemia. The accompanying panniculitis in our case was considered to be unique and somehow different from morphea profunda or lupus erythematosus profundus.
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ranking = 8
keywords = lupus erythematosus, erythematosus, lupus
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8/19. Nodular regenerative hyperplasia of the liver in rheumatic diseases: report of seven cases and review of the literature.

    Nodular regenerative hyperplasia (NRH) of the liver is an uncommon pathologic finding associated, in most cases, with rheumatic and hematologic diseases. Although its pathogenesis remains unclear, NRH probably results from liver regeneration to maintain its functional capacity after ischemia-induced injury. An intrahepatic microvascular occlusive mechanism has been considered most likely pathogenetically. NRH may lead to portal hypertension. Thus, the diagnosis of Felty's syndrome must be considered with caution in patients with rheumatoid arthritis (RA) and NRH of the liver. We report seven additional cases of NRH in patients with rheumatic disorders and review the literature to determine the patterns of clinical presentation and natural history of this condition. We also report four patients (three systemic lupus erythematosus [SLE] and one primary antiphospholipid syndrome [PAPS]) in whom antiphospholipid antibodies may have played a role in the genesis of NRH.
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ranking = 1.0539400416148
keywords = lupus erythematosus, erythematosus, lupus, systemic lupus erythematosus, systemic lupus
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9/19. Subacute cutaneous lupus erythematosus lesions progressing to morphea.

    A women had annular lesions of subacute cutaneous lupus erythematosus that slowly resolved and were replaced by plaques of morphea. The immunologic implications of this unique transitional case of subacute cutaneous lupus erythematosus to morphea are discussed.
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ranking = 6
keywords = lupus erythematosus, erythematosus, lupus
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10/19. Linear scleroderma, hemiatrophy and systemic lupus erythematosus.

    A young woman, with linear scleroderma accompanied by body hemiatrophy since childhood, developed systemic lupus erythematosus. The rare coexistence of the 2 diseases is discussed.
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ranking = 5.2697002080739
keywords = lupus erythematosus, erythematosus, lupus, systemic lupus erythematosus, systemic lupus
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