Cases reported "Elephantiasis"

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1/3. Elephantiasic pretibial myxedema: insight into and a hypothesis regarding the pathogenesis of the extrathyroidal manifestations of Graves' disease.

    The basis for the extrathyroidal manifestations of Graves' ophthalmopathy (GO) and dermopathy are not well understood. We describe immunohistochemical studies on the skin of a patient with an extreme, elephantiasic form of Graves' dermopathy that developed after periods of prolonged standing with dependent edema. Excision of part of the lesion with subsequent skin grafting from a normal donor site resulted in recurrence of the disease at the original site as well as in development of disease at the donor site. A murine monoclonal antibody reacted with the thyrotropin receptor (TSHR) or a cross-reacting protein in fibroblast-like cells in the patient's upper dermis and, surprisingly, with dermal cells from unaffected individuals. The patient's dermis containing lymphoid follicles comprising B cells and CD3 , CD4 T cells, with few CD8 T cells. CD21 cells (most likely follicular dendritic cells) were also present in the dermis. Based on past and present observations, we raise an unifying hypothesis to explain the diverse extrathyroidal manifestations of Graves' disease and their apparent lack of association with TSHR autoantibodies. As opposed to the present concept that these phenomena relate to site-specific properties on preadipocytes or fibroblasts, we suggest that clinically evidence GO and dermopathy are primarily caused by local factors (particularly in the orbit) superimposed on a systemic, low-grade connective tissue inflammation.
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2/3. soil particles in the tissues of the foot in endemic elephantiasis of the lower legs.

    The presence of microparticles of clay is demonstrated in the dermis of the foot in a patient with endemic elephantiasis. The particles are seen to be in the phagosomes of macrophages or in the cytoplasm of other cells. The conducting lymphatic in the subdermal tissue is found to be impermeable to Patent Blue Violet dye and to be fibrosed. The failure to conduct lymph to the node produces a permanent deposit of silica in the dermal tissues; a parallel is drawn with similar deposits in the lung in pneumoconiosis.
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keywords = dermis
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3/3. elephantiasis nostras verrucosa.

    elephantiasis nostras verrucosa is a rare, chronic, deforming disorder characterized by hyperkeratosis and papillomatosis of the epidermis with underlying woody fibrosis of the dermis and subcutaneous tissue. Chronic lymphedema, either congenital or secondary to infection, surgery, radiation, neoplastic obstruction, obesity, portal hypertension, or chronic congestive heart failure, plays a pivotal role in the pathogenesis. Without appropriate intervention, the slowly progressive cutaneous changes will culminate in massive and grotesque enlargement of the affected body region. In the following case report, the natural history and the management of elephantiasis nostras are discussed.
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