We report two patients who developed benign plasmacytosis with multiple skin lesions. The cases were characterized by hyperplasia of mature plasma cells, and polyclonal hypergammaglobulinaemia. One patient had hyperplasia of mature plasma cells not only in the skin, but also extensively in lymph nodes and the retroperitoneal areas around the ureters. The other had plasma cell hyperplasia limited to the skin. Extensive investigations failed to reveal any clinical or laboratory evidence suggesting the presence of any underlying disease accompanying the hypergammaglobulinaemia and/or plasma cell proliferation, such as chronic infectious disease, collagen disease or other chronic inflammatory disorder. Clinically and histologically, the first patient showed features compatible with a diagnosis of systemic plasmacytosis and the second with a diagnosis of cutaneous plasmacytosis. Significant serum interleukin-6 (IL-6) levels were detected in both patients, suggesting that IL-6 may be involved in the pathogenesis of these conditions.
We report the case of a 49-year-old woman suffering from a malignant neoplasm of Langerhans cells (LC), documented by immunohistochemical and ultrastructural analysis, and review the literature to examine and characterize the clinical and laboratory features, therapy, and prognosis of malignant neoplasms of LC. Langerhans cell histiocytosis is now regarded as a disorder of immune regulation or an inflammatory process, rather than as a malignant neoplasm. Although LC share certain features in common with ordinary histiocytes or interdigitating dendritic cells, they also differ significantly from these cells in other respects. Therefore, we propose designating a malignant neoplasm of LC 'malignant Langerhans cell tumour' and that it should be considered as a separate entity from Langerhans cell histiocytosis or other malignant histiocytoses.
SUMMARY
In an uncontrolled study of twelve patients with miliary lupus of the face, dapsone gave good therapeutic results.
Miliary lupus of the face (MLF) is an asymptomatic papular eruption affecting the central area of the face, which runs a chronic course and involutes spontaneously with scarring. Histopathologically, the papules usually show scattered masses of tuberculoid granulomata composed of epithelioid cells, giant cells, and an encircling rim of small round mononuclear cells in the dermis. The actiology and pathogenesis of this condition are still unknown. The treatment of MLF has not been satisfactory despite the use of many topical and systemic agents.
In this paper we report that dapsone has been found to be beneficial in the treatment of MLF and has accelerated its resolution in twelve patients.
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