1/26. diagnosis and physiopathology of laryngeal deposits in autoimmune disease.We report the clinical features and pathology of a previously unreported form of vocal fold disease seen in 4 patients, 3 of whom were diagnosed as autoimmune disease. The characteristic features of these lesions were found as bilateral transverse lesions in the mid portion of the vocal folds. The patients had dysphonia and diplophonia. Stroboscopic examinations showed 180-degree phase shifts between the anterior and posterior portion of the vocal folds. Case 1 had systemic lupus erythematosus, case 2 had Hashimoto's thyroiditis, and case 3 had progressive systemic sclerosis. Prior to the onset of hoarseness, autoimmune antibody titers were increased. These cases need differential diagnosis from vocal fold nodules and cysts. Two cases were recurrent after endoscopic surgery, 1 recurring 3 times. Glucocorticoid was effective in preventing the recurrence in the early phase. recurrence may have occurred because the surgical strategy was the same as that used for vocal fold nodules or cysts.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
2/26. Vasculitis-induced membranous fat necrosis.Membranous fat necrosis (MFN) a distinct degenerative process of adipose tissue, is characterised by the presence of membranocystic lesions (MCLs) superimposed on a background of typical traumatic-type fat necrosis. MCLs are cysts, of varying size and shape, that are lined by an eosinophilic, crenulated membrane, having the staining properties of ceroid. Although MFN has been documented in varying systemic adipose tissue sites and in tumours, the pathogenesis of this pathological curiosity is unknown. To date, an ischemic basis for MFN has been the most proximate, and atherosclerosis and venous insufficiency, due to large and medium vessel disease, have been the most popular underlying clinical disorders. Although systemic vasculitis has been quoted as the underlying ischemic disorder in some patients, vasculitis has not been commented on nor demonstrated in tissue sections in association with MFN. In,reporting vasculitis-induced MFN, we document the occurrence of MFN in association with uncommon causes of vasculitis, namely: 1) Granulomatous vasculitis in a post-herpetic zosteriform scar; 2) cytomegalovirus-induced vasculitis in the clinical setting of systemic lupus erythematosus; and 3) Lymphocytic vasculitis in a tetanus toxoid immunization site reaction.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
3/26. F.I.GU.R.E.: facial idiopathic granulomas with regressive evolution. is 'lupus miliaris disseminatus faciei' still an acceptable diagnosis in the third millennium?We report the case of a 69-year-old woman who presented a papular eruption on the eyelids. Histological features revealed a tuberculoid granuloma with a central caseating necrosis. Laboratory and radiological investigations revealed no tuberculosis and no systemic granulomatosis. Absence of vascular symptoms, inefficiency of cyclines and histopathological findings excluded granulomatous-type rosacea. Lupus miliaris disseminatus faciei (LMDF) was our final diagnosis. On the basis of our findings and a literature review, we believe that LMDF is an entity distinct from either skin tuberculosis or granulomatous-type rosacea. However, its name is confusing, and we propose to change it to 'facial idiopathic granulomas with regressive evolution (FIGURE)'.- - - - - - - - - - ranking = 217.44474769165keywords = disseminatus, lupus (Clic here for more details about this article) |
4/26. Recalcitrant trichophytic granuloma associated with NK-cell deficiency in a SLE patient treated with corticosteroid.Although deep trichophytic infection often occurs in immunocompromised patients, the immune deficiency in such patients has not been clarified. A 28-year-old man who suffered from recalcitrant trichophytic granuloma and tinea universalis during treatment for SLE with corticosteroid is described here to define the immunological abnormalities. In addition to routine immunological tests, we evaluated the patient's innate and specific immune functions to dermatophytes, including T cell, natural killer (NK) cell and neutrophil functions and activation of the complement cascade. We measured the minimum inhibitory concentration (MIC) of itraconazole for the isolated fungus and its concentrations in the patient's serum and pus. trichophyton (T.) rubrum was constantly isolated from the exudates of the patient's skin lesions, although the concentrations of itraconazole in his serum (198 ng/ml) and lesions (210 ng/ml) were sufficient to inhibit the growth of the isolated fungus in vitro. Specific cell-mediated immune responses, determined by T cell stimulation and IFN-gamma production, were evoked following stimulation with trichophytic antigens. The patient's innate immunity, assessed by activation of the complement cascade and neutrophil-mediated phagocytosis, was not impaired. The number of circulating NK cells was markedly decreased (0.2% of the peripheral blood mononuclear cells), and was associated with low NK cell activity against K-562 cells even though lymphopenia had improved. The deficiency of innate immunity mediated by NK cells might be responsible for a part of the persistence of trichophytic granuloma in our case. Dermatophytes usually affect the horny layer of the skin and do not invade the living layers because the host immune system uses various mechanisms to eliminate the fungi. Both specific T cell-mediated immunity and nonspecific immunological mechanisms provide host defense against fungal infections. An adaptive immune response is usually preceded by innate immune responses mediated by neutrophils, NK cells, and circulating proteins such as complement components and anti-microbial peptides. However, in patients with localized or systemic immunological defects, granulomatous cutaneous infection of dermatophytes mostly caused by trichophytic fungi may occur [1]. Trichophytic granuloma includes Majocchi's granuloma [2] and disseminated trichophytic granuloma [3]. Recently, we experienced a patient with trichophytic granuloma and tinea universalis caused by trichophyton (T.) rubrum infection during treatment with corticosteroid for systemic lupus erythematosus (SLE). We describe the clinical details of this patient, focusing on his immunological defects which led to the persistence of the fungal infection.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
5/26. Cutaneous granulomas masquerading as tuberculoid leprosy in a patient with congenital combined immunodeficiency.Combined immunodeficiency disorders are characterized by abnormalities in cellular and humoral immunity. This classification includes common variable immunodeficiency (CVI), a primary immunodeficiency disorder characterized by hypogammaglobulinemia, recurrent bacterial infections, and significant T-cell abnormalities. Associated autoimmune diseases include rheumatoid arthritis, pernicious anemia, idiopathic thrombocytopenic purpura, and systemic lupus erythematous. Granulomatous lesions in lymphoid tissues, solid organs, and skin have been reported. We describe a patient with CVI who developed cutaneous granulomas with perineural invasion; to our knowledge, this is a previously undescribed feature.- - - - - - - - - - ranking = 0.11918680639926keywords = lupus, systemic lupus (Clic here for more details about this article) |
6/26. Extravascular necrotizing palisaded granulomas as the presenting skin sign of systemic lupus erythematosus.We recently examined a patient who presented clinically with tender, erythematous papules associated with a febrile illness and polyarthralgias. Histopathological examination revealed extravascular palisaded neutrophilic granulomas, a skin reaction pattern originally described in Churg-Strauss disease but subsequently observed in a variety of other systemic disorders characterized by immune complex generation. A diagnosis of systemic lupus erythematosus (SLE) was established. To our knowledge, this is the first report of extravascular necrotizing palisaded granulomas as the presenting skin sign of SLE.- - - - - - - - - - ranking = 5keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
7/26. Tuberculous choroidal granulomas in a patient with systemic lupus erythematosus. A case report.AIM: We report a case of tuberculous choroidal granulomas in a patient with systemic lupus erythematosis (SLE) who developed miliary tuberculosis (TB) and bilateral granulomas while on immunosuppressive medication. methods: The case notes of this patient were reviewed. RESULTS: The patient was first seen for base-line ophthalmic screening before anti-tuberculous medication was started. The patient was subsequently followed up at 2 weeks, 4 weeks and 2 months with fundal examination and photography. One choroidal granuloma was found in the right eye while another peripheral lesion involved the left eye. Both resolved in response to anti-tuberculous medication over a 2-month period with resultant chorioretinal atrophy and scarring. CONCLUSION: This case demonstrates the need to screen immuno-suppressed SLE patients who have miliary TB for possible eye involvement.- - - - - - - - - - ranking = 4.1191868063993keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
8/26. Lupus miliaris disseminatus faciei: a distinctive rosacea-like syndrome and not a granulomatous form of rosacea.BACKGROUND: Lupus miliaris disseminatus faciei is an eruption of discrete red-brown, dome-shaped papules, histologically characterized by epithelioid cell granulomas. The pathogenesis of the disorder remains controversial. OBJECTIVE: The authors discuss the place of lupus miliaris disseminatus faciei among granulomatous disorders. methods: This report reviews the available literature and presents 3 patients with lupus miliaris disseminatus faciei. RESULTS: The histopathologic characteristics of lupus miliaris disseminatus faciei--an epithelioid cell granuloma with central necrosis--may be found in granulomatous rosacea, whilst the clinical features and course of lupus miliaris disseminatus faciei are often similar to cutaneous sarcoidosis. CONCLUSIONS: We put forward lupus miliaris disseminatus faciei as a distinctive rosacea-like syndrome and not as a granulomatous form of rosacea.- - - - - - - - - - ranking = 434.62442089842keywords = disseminatus, lupus (Clic here for more details about this article) |
9/26. Immunofluorescence findings in granuloma faciale: report of two cases.BACKGROUND: Immunofluorescence findings in granuloma faciale have been infrequently described. Reported findings include granular IgA, IgG, IgM, and C3 deposits in the dermoepidermal junction, in blood vessel walls, and on connective tissue fibers; IgG in the basement membrane zone, and IgG around blood vessels. OBSERVATIONS: We report two cases of granuloma faciale in which biopsy specimens were sent for routine pathology as well as immunofluorescence. Additional work-up included serologic testing for markers of lupus erythematosus (LE). Immunofluorescence in both cases revealed granular IgA, IgG, IgM, C3, and C5-9 deposits along the dermoepidermal junction only. Blood vessel walls and connective tissue fibers were spared. Serologic studies for markers of LE were negative. CONCLUSION: Immunofluorescence findings were essentially identical to those which would be expected in cutaneous LE; however, the combination of negative serologic studies for LE, histopathologic findings typical of granuloma faciale, and clinical findings most consistent with granuloma faciale allowed the diagnosis of granuloma faciale to be rendered with certainty. Interpretation of results of direct immunofluorescence of skin should be correlated with clinical presentation, histopathological findings and other laboratory results in order to render final diagnosis of a given patient.- - - - - - - - - - ranking = 0.85186074798395keywords = lupus erythematosus, erythematosus, lupus (Clic here for more details about this article) |
10/26. Palisaded neutrophilic and granulomatous dermatitis.A 46-year-old man with a history of systemic lupus erythematosus presented with a 6-month history of scaly, erythematous, tender nodules on his elbows and hands. physical examination showed erythematous, umbilicated papules, with crusts on the elbows and the dorsa of the hands. Histopathologic examination demonstrated leukocytoclastic vasculitis with dense neutrophilic infiltrates and degenerated collagen, consistent with early palisaded neutrophilic and granulomatous dermatitis. The individual lesions were treated with intralesional glucocorticoid injections with some improvement.- - - - - - - - - - ranking = 1keywords = lupus erythematosus, erythematosus, systemic lupus erythematosus, lupus, systemic lupus (Clic here for more details about this article) |
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