Cases reported "Sjogren's Syndrome"

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1/12. The relationship between facial annular erythema and anti-SS-A/Ro antibodies in three East Asian women.

    A distinct annular erythema developed on the cheeks of three East Asian women who had anti-SS-A/Ro (SSA) antibodies. The erythema was characterized by a wide, elevated border and central pallor. Histologically, there was a coat-sleeve-like infiltration of lymphocytes around the blood vessels, appendages, and secretory gland cells in the dermis. Immunohistological analysis clarified that the majority of infiltrating lymphocytes were CD4-positive T cells. Abnormal expression of hla-dr antigens in the perivascular, appendage, and secretory gland cells in the dermis was also observed. The differential diagnosis of the three patients lay between Sjogren syndrome (SjS), Sjogren/systemic lupus erythematosus overlap syndrome and an asymptomatic clinical state. These results are consistent with recent findings of major histocompatibility complex class II expression on target organs in various autoimmune diseases. Based on these findings, erythema appears to represent a broad cutaneous manifestation of these diseases. Furthermore, the presence of SSA antibodies, aberrant HLA-DR expression, and sun exposure may be responsible for the development of erythema.
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2/12. Large annular purpura and paraneoplastic purpura in a patient with sjogren's syndrome and cervical cancer.

    We report a 79-year-old female with anaphylactoid purpura on her legs and unusual large annular purpura on the trunk. Histopathological characteristics of leukocytoclastic vasculitis were observed in the upper and middle dermis of both types of skin lesions. She was complicated by sjogren's syndrome and advanced cervical cancer. The annular purpura spontaneously resolved in a week and did not recur. However, the anaphylactoid purpura relapsed more frequently and spread more widely following the elevation of her serum SCC antigen levels from the onset of purpura until her death. We consider that the characteristic annular configuration was caused by the complication of sjogren's syndrome and that the recurrent anaphylactoid purpura indicated paraneoplastic vasculitis primarily caused by the tumor specific protein immune complexes. Complication by Sjogren's syndrome many also play a role in the development of allergic vasculitis.
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3/12. Erythematous swelling of the lip associated with sjogren's syndrome and mimicking cheilitis granulomatosa.

    A 64-year-old Japanese woman developed therapy-resistant erythematous swelling of her upper lip. Our tentative clinical diagnosis of cheilitis granulomatosa was ruled out later by the laboratory findings including increased levels of anti-nuclear-antibody (ANA), anti-SSA/Ro antibody, and positive Schirmer test as well as by a histopathological picture showing a dense perivascular infiltration of plasma cells and mononuclear cells in the dermis instead of granulomatous changes. To the best of our knowledge, this is the first patient in whom annular erythema associated with S ogren's syndrome involved only the upper lip and produced clinical features simulating cheilitis granulomatosa.
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4/12. Painful indurated erythema suggestive of Kikuchi-Fujimoto disease in a patient with primary sjogren's syndrome.

    We report a patient with primary sjogren's syndrome who developed pyrexia, cervical lymphadenopathy, and painful indurated erythema on the forehead, back, chest, abdomen, and limbs. Laboratory data showed an elevated erythrocyte sedimentation rate, c-reactive protein and CH50 in addition to existing autoantibodies including anti-nuclear antibody, anti SS-A antibody, and anti SS-B antibody. A skin biopsy specimen showed focal infiltration of histiocytes with non-neutrophilic karyorrhetic debris in the dermis and subcutaneous fat tissue. Immunohistochemically the infiltrated cells were stained for CD68, suggesting cutaneous involvement of Kikuchi-Fujimoto disease. All symptoms and laboratory data improved within three weeks after treatment with 20 mg/day of prednisolone. The present case suggests that a pathophysiological condition similar to Kikuchi-Fujimoto disease can develop during the long-term course of sjogren's syndrome.
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5/12. Primary cutaneous nodular amyloidosis in a patient with sjogren's syndrome.

    A 71-year-old woman with a history of sjogren's syndrome presented for evaluation of a waxy nodule present on the scalp for 6 months. Histopathologic examination revealed deposition of homogenous eosinophilic material throughout the reticular dermis consistent with amyloidosis. Primary cutaneous nodular amyloidosis is a rare phenomenon characterized by the deposition of immunoglobin light chains by a clonal plasma cell population. patients need to be monitored for progression to systemic amyloidosis or plasma cell dyscrasias.
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6/12. Phytohemagglutinin (PHA) skin test. Characterization of immunological properties and clinical application.

    Immunological properties of phytohemagglutinin (PHA) skin reaction were investigated by animal and clinical experiments. In the guinea pigs an intradermal dose of PHA-P produced erythema and induration with a maximal response at 24 hours after the injection. Histologically it was characterized by perivascular infiltration of lymphoid cells in the dermis and subcutis, being similar to that of tuberculin (PPD) skin reaction. PHA skin reaction, however, showed some difference from that of PPD in the initial cellular response in that the former was composed of small mononuclear cells and granulocytes with rapid development and the latter was composed of large mononuclear cells (macrophages) and granulocytes with slow development. Intradermal injection of 1:1000 dilution of PHA-P produced a similar erythema in man. In 39 of 59 patients with connective tissue diseases, the results of the in vivo (skin test) and in vitro (lymphocyte transformation) response to PHA correlated well. In the 59 patients, the incidence of the positive rate of the PHA tests (55.9%) was significantly higher than that of the DNCB test (33.9%) and of the PPD test (23.7%). These observations suggest that the PHA skin test has properties of delayed hypersensitivity and is highly sensitive and that it may be a useful measure of cell-mediated immunity.
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7/12. pemphigus foliaceus in a patient with rheumatoid arthritis and sjogren's syndrome. A case report.

    A 74-year-old male patient with rheumatoid arthritis and sjogren's syndrome developed pemphigus foliaceus. Clinically healthy skin tissue revealed a fluorescent band of IgM along the basal membrane and an intercellular deposit of C3 and IgG in the deepest areas of the epidermis. Similar investigations of diseased skin revealed no deposits of IgM, and C3 and IgG were spread over a large area of the epidermis, though following the same pattern. In sero-positive rheumatoid patients with and without vasculitis, C3 and IgM are frequently found in small dermal vessels immediately below the epidermis in apparently normal skin. The fluorescent band of IgM along the basal membrane is thought to be an expression of the patient's rheumatoid arthritis, in spite of the fact that this IgM site is not typically associated with rheumatoid arthritis. The change in the pattern of deposition is thought to be due to pemphigus foliaceus.
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8/12. Anhidrosis (hypohidrosis) in sjogren's syndrome.

    There has been a relative lack of literature on the association of hypohidrosis in sjogren's syndrome with any lesion having specific histologic findings. We looked at a recent case presentation of a 55-year-old man with complaints of dry mouth and dry eyes, becoming easily overheated in direct sunlight, and having difficulty in perspiring. physical examination showed fissuring and atrophy of the tongue and angular cheilitis. A punch biopsy of the skin showed a moderate number of eccrine gland and ductal structures in the lower reticular dermis, each surrounded by a dense cellular infiltrate of plasma and lymphocytic cells. Our patient also had a markedly decreased sweating response to methacholine. In reviewing the literature as far back as 1951 and on the basis of findings in our present case study, we conclude that it seems probable that the severity of the skin disease is an important determining factor in predicting whether the sweat gland lesion does exist.
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9/12. association of the acral type of pustular psoriasis, sjogren's syndrome, systemic lupus erythematosus, and Hashimoto's thyroiditis.

    We describe a case of a 53-year-old Japanese female suffering from sjogren's syndrome, systemic lupus erythematosus, and Hashimoto's thyroiditis who developed pustules, erythema, and erosions on her fingers and toes. The histological specimen showed psoriatic changes. Indirect immunofluorescent study using anti-human IL (interleukin)-8 antibody produced positive staining patterns in the lesional epidermis. These findings suggested the diagnosis of acral pustular psoriasis. Diaminodiphenylsulfone at 75 mg orally daily for 20 days and the application of 0.12% betamethasone valerate ointment led to gradual improvement.
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10/12. Acral ichthyosiform mucinosis in association with sjogren's syndrome: a peculiar form of pretibial myxedema?

    Two Japanese women developed well-demarcated ichthyosiform plaques on the lateral aspect of their lower legs. Deposition of mucin was demonstrated throughout the papillary dermis, unlike the site of mucin deposition seen in pretibial myxedema. Their thyroid function was normal. The condition of both women was complicated by sjogren's syndrome. One of them who presented with positive anti-microsomal and anti-thyroglobulin antibodies had goiter, suggesting that her malady was also complicated by Hashimoto's thyroiditis. Their skin manifestations differed from those described in cutaneous mucinosis including pretibial myxedema, specifically with regard to the well-demarcated ichthyosiform appearance, the mucin deposition in the papillary dermis, and the association with sjogren's syndrome. To the best of our knowledge, our cases may thus be considered to be a previously undescribed form of cutaneous mucinosis associated with sjogren's syndrome.
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