The complement system in bullous pemphigoid. VI. C3 fixing activity in the absence of detectable antibody.
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Biomedical subjects
Publications and source records attributed to J L Millns.
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A 57-year-old man with generalized, subcutaneous fat necrosis was found at autopsy to have islet cell carcinoma of the pancreas. The histologic diagnosis of islet cell carcinoma was confirmed by the finding by electron microscopy of characteristic intracytoplasmic granules within pancreatic neoplastic cells. Lipase levels were elevated in serum as well as in tissues in areas of subcutaneous fat necrosis. Enzyme histochemical stains for alkaline and acid phosphatase, leucine, aminopeptidase, succinic dehydrogenase, and indoxyl and nonspecific esterase were positive in the areas of subcutaneous fat necrosis. This is the first report of the association of islet cell carcinoma of the pancreas with generalized subcutaneous fat necrosis.
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An unusual case of photosensitive psoriasis and systemic lupus erythematosus-related syndrome was characterized by erythroderma, chronic urticaria, angioneurotic edema, intermittent low-grade fever, and polyarthralgias. Investigation revealed no measurable total hemolytic complement and markedly diminished levels of C4, C2, and C3. Microscopic examination of three skin biopsy sections of sun-exposed skin showed psoriasis. Skin biopsy sections of sun-exposed psoriatic plaques and of non-sun-exposed, uninvolved skin (which were stained with fluorescein-tagged anti-IgG, anti-IgM, anti-IgA, and anti-C3) showed granular deposits of IgM and C3 at the dermal-epidermal junction in the sun-exposed plaques, and IgM alone in a granular pattern at the dermal-epidermal junction in uninvolved skin. Antibodies to single-stranded but not double-stranded DNA were detected in the patient's serum. In addition, serum immune complex-like material was detected by sucrose density-gradient ultracentrifugation, standard anticomplementary assays, and radioimmunoassays using both C1q and monoclonal rheumatoid factor.
The conditions of ten patients were diagnosed as pemphigus vulgaris limited to the oral cavity. In eight patients the histopathologic findings were consistent with oral pemphigus. Direct immunofluorescence demonstrated intercellular substance (ICS) deposition of C3 either alone or in combination with IgG, IgA, IgM, C4, C1q, properdin, and factor B in all nine patients tested. Circulating ICS antibodies were detected initially in eight of the ten patients, nine of whom had positive titers at a later date, without subsequent development of cutaneous lesions. Physicians should be alerted to a diagnosis of pemphigus in patients who have chronic erosive disease confined to the oral cavity. Prompt diagnosis by means of routine histopathologic and immunofluorescence studies followed by early initiation of treatment are warranted.
Henoch-Schönlein purpura is classically described as a systemic vasculitis without known platelet or clotting abnormality. A 15-year-old boy with Henoch-Schönlein purpura experienced a major hemorrhagic diathesis that was responsive to parenterally administered vitamin K. We believe that the gastrointestinal vasculitis prevented the absorption of available vitamin K necessary for the synthesis of clotting factors. Early treatment with parenteral vitamin K in similar cases should be considered.