Actinic reticuloid with Sézary cells.
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Biomedical subjects
Publications and source records attributed to J J Cream.
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Methotrexate depresses motility of neutrophils in chamber membranes, but does not interfere with random motility in tubes. Hydroxyurea has no such effect. The therapeutic response to methotrexate in psoriasis may be due in part to a reduction in migration of neutrophils into the epidermis.
In the Boyden's chamber method to assess chemotaxis, when used to detect drug induced impairment of chemotaxis, the double filter counting method shows no advantage over the single filter technique.
Superior vena cava (SVC) obstruction may occur in several disorders (Urschel & Paulson, 1966) and may be associated with arteritis in Behçet's disease (Chajek & Farinaru, 1975). We report a man who had arteritis and SVC obstruction without any of the known causes.
Paraproteinaemia may be associated with xanthomatous skin deposits and these can arise in the absence of elevated lipid levels. Two cases of benign monoclonal gammopathy with diffuse plane xanthomatosis are reported. Case 1 exhibited hypolipidaemia and a functional deficiency of C1 esterase inhibitor. Case 2 showed a normal lipoprotein profile, abnormal platelet aggregation, and a cutaneous vasculitis with evidence of complement consumption via the classical pathway. The significance of these abnormalities is discussed.
A study of the family of a patient who had an SLE-like syndrome and an extremely low serum C4 revealed an inheritance of C4 types and HLA region markers which indicated that the patient had 60--70% of "normal" C4 level prior to the onset of disease. Thus the extremely low C4 level during her disease may result from a combination of genetically determined low normal C4 and increased consumption/hyposynthesis secondary to her SLE.
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Thirty-five patients with cutaneous vasculitis were studied. Sixteen had raised serum levels of cryoglobulins. In all but one the cryoglobulin was an immune complex. Eleven patients had anticomplementary sera and five had a low serum IgM. Clinically, the cryoglobulinaemic patients were not readily distinguishable from the non-cryoglobulinaemic in that cold sensitivity occurred in only six of sixteen patients with cryoglobulins. Peripheral neuropathy and lesions in the upper respiratory tract were confined to those with cryoglobulinaemia. An immunopathological classification for the purpuric vasculitides is suggested.
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Three patients with peripheral neuropathy and mixed or immune complex cryoglobulinaemia are reported. The significance of mixed cryoglobulinaemia and the pathogenesis of the peripheral neuropathy are discussed.
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