Microchimerism in human diseases.
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Biomedical subjects
Publications and source records attributed to S Aractingi.
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Yellow nail syndrome is an uncommon diagnosis established on the basis of clinical presentation with slow-growing yellow discolored nails, lymphedema, and pulmonary manifestations. We report 3 new cases with their pulmonary component.
The development of several types of autoimmune disorders is well known in the course of B-cell lymphoproliferative diseases. In order to investigate whether these autoimmune diseases may target cutaneous antigens, especially those of the dermal-epidermal junction, we conducted a retrospective analysis of all cutaneous direct immunofluorescence studies performed in patients with B-cell neoplasia who presented with cutaneous lesions requiring a skin biopsy. Patients with linear deposits of immunoglobulin or C3 molecules at the dermal-epidermal junction were studied further, using immunoblotting and immunoelectron microscopy. Patients with no overt hematologic malignancies but with cutaneous lesions requiring direct immunofluorescence were studied as a control population. During the study period, the diagnosis of autoimmune blistering diseases of the dermal-epidermal junction was established in 9/102 (8.9% +/- 2%) patients with a B-cell lymphoid disorder, compared with 288/8,189 (3.5% +/- 0.04%) patients without underlying hemopathy (p < 0.01). Among the 9 patients, 7 were studied in detail. Immunologic studies demonstrated epidermolysis bullosa acquisita in 6 patients and cicatricial pemphigoid in 1. Autoimmune blistering diseases, especially epidermolysis bullosa acquisita, are part of the spectrum of autoimmune disorders associated with B-cell neoplasia.
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BACKGROUND: Polymorphic eruptions of pregnancy (PEP) are common cutaneous disorders of unknown origin that occur usually after week 34 of gestation. Since pregnancy is associated with peripheral-blood chimerism, particularly during the third trimester, we studied the role of fetal cells in the development of the skin lesions. METHODS: We studied samples of skin from ten women with PEP who were carrying male fetuses and 26 women with normal skin or non-PEP skin disorders (13 carrying male and 13 carrying female fetuses). Epidermis and dermis were dissected from the samples, and the DNA was extracted. PCR with primers specific for the SRY gene was used to detect male DNA. FINDINGS: Male DNA was detected in dermis or epidermis from skin lesions of six of the ten women with PEP. No male DNA was detected in any of the 26 women without PEP. INTERPRETATION: Fetal cells can migrate to skin during gestation, where they seem to be associated with the development of cutaneous disorders of pregnancy.
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Graft-versus-host disease (GvHD) includes inflammatory and/or fibrosing manifestations that may arise at various times after transplantation of any organ containing lymphoid cells. For the dermatologist, the importance of GvHD is real and current. Indeed, because it has become easier to perform bone marrow transplantation (EMT), the indications of BMT have become broader, making follow-up of patients receiving grafts a widespread practice. Nonetheless, GvHD remains a frequent complication of BMT and its principal target organ is the skin. Furthermore, recent innovations, such as grafting of umbilical cord blood and the mobilization of peripheral blood progenitor cells, will be the source of new questions concerning the development of GvHD under these conditions. Finally, because of its analogies with other spontaneous idiopathic skin diseases, GvHD constitutes a model that may lead to a better understanding of the pathophysiological features of these diseases. In this review, the cutaneous aspects of GvHD are emphasized.
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Anogenital warts have become one of the most common sexually transmitted diseases reported in the Western World. The frustration of treatment for both patient and carer is well recognised. Current available methods rely principally on ablation of visible lesions, with hospital-based treatments often requiring multiple attendance by out-patients. Overall, the current failure rate, recurrence rate, and side-effects of these treatments are highly unsatisfactory. Imiquimod, recently launched in the US under the brand name Aldaratrade mark cream, represents the most interesting and innovative approach to therapy to become available in many years. Imiquimod is an immune response modifier, therefore this symposium report addresses the vital issues of the immune based response to human papilloma virus (HPV) infection, as well as the problems of persistence caused by HPV disease. The mechanisms by which imiquimod can induce an inflammatory and cell-mediated response are discussed. Also reviewed are the consequent imiquimod clinical results and the reasons why they show great cause for optimism in HPV treatment. As a home-based effective treatment with a low recurrence rate, imiquimod has already generated enthusiasm on an international scale. This symposium report presents the thoughts and experiences of medical specialists from various countries regarding the treatment of genital HPV infections and the place for imiquimod in clinical practice.
Drawing the meeting to a close, co-chairman Dr. Selim Aractingi said that it is now clear that local immune factors are important in determining the regression of warts, and that imiquimod appears to mimic the mechanism of immunity occuring in people in whom warts regress without treatment. He summarised the main presentation points, stating that topical imiquimod 5% - when applied three times each week overnight at home - is an effective therapy with acceptable side effects. Dr. Aractingi noted that the recurrence rate is lower than those reported with most other available wart therapies. Further studies to specifically compare recurrence rates are warranted, he concluded.
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BACKGROUND: Epitheloid sarcoma is an uncommon malignant soft tissue tumor observed in the distal extremities of young men. We report a case of long standing ulceration of the sole which was found to be an epithelioid sarcoma. CASE REPORT: A 78-year-old woman had an indolent ulceration of the left sole for several months. Physical examination disclosed a well demarcated 3-cm ulcerated lesion with a red center and flat edges. Skin sections confirmed the diagnosis of epithelioid sarcoma. Cells stained positively for anti-vimentin, anti-cytokeratin and anti-epithelial membrane antigen, but not for anti-S100 protein and anti-actin antibodies. Wide local excision was performed. DISCUSSION: Epithelioid sarcoma is an uncommon malignant tumor which apparently differentiates from mesenchymatous cells. It is usually observed in the distal extremities in young males, predominantly the hands and forearms. The tumor presents a firm, flesh-colored indolent nodule. Ulceration usually develops and involves the subcutis and deeper soft tissue, particularly fascial planes, aponeuroses and tendon sheaths. Treatment is wide surgical excision with or without radiotherapy. The case reported here on the sole of the foot in a 78-year-old women is unusual. Clinicians should be aware that the initial biopsy may not be contributive and that repeated biopsies may be necessary for positive diagnosis.
BACKGROUND: Neutrophilic eccrine hidradentitis is a recently described clinical entity. Most reported cases have occurred in patients given chemotherapy for acute myelogenous leukemia, suggesting a drug induced mechanism. Some authors have considered however that neutrophilic eccrine hidradenitis belongs to the group of neutrophilic dermatoses. CASE REPORT: We observed neutrophilic eccrine hidradentitis in a 48-year-old man which developed when he suffered a relapse of acute leukemia. He had not been given chemotherapy in the preceding months. DISCUSSION: This case favors the hypothesis that neutrophilic eccrine hidradenitis is associated with myeloid hemotology disorders and not a complications secondary to treatment.