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Subepidermal autoimmune bullous dermatoses are defined by clinical, histological and immunological criteria, notably the presence of anti-basement membrane antibodies detectable in vivo by direct immunofluorescence. We report three cases where anti-basement membrane antibodies were not detectable in vivo by direct immunofluorescence but were detected in high titres by indirect immunofluorescence. This situation is extremely rare in the literature. The first case concerns a 69-year old woman seen for bullous and pruriginous lesions of the lower limbs of 2 months' duration. Histological examination found dermoepidermal bullae with, in their lumen, a serous fluid spotted with numerous polymorphonuclears. A search for anti-basement membrane antibodies was positive in significant titres (1,280; 320; 480) at indirect immunofluorescence on rabbit lip whereas five successive direct immunofluorescence test in perilesional skin and on the thigh medial surface remained negative. The second case is that of a 91-year old woman suffering from generalized pruritus associated with erythematous lesions predominant on the extension surfaces of the forearms and thighs, without any bullous lesion. Pathological examination only showed a superficial dermal lymphocytic infiltrate. Four direct immunofluorescence tests were negative, whereas a search for anti-basement membrane antibodies on rat oesophagus was positive at 1/1,280. The third case resembles the second one. It concerns a 72-year old woman who consulted for generalized pruritus of several months' duration which interfered with sleep and was incompletely relieved by emollients. There was no specific skin lesion. Pathological examination revealed nothing more than a discrete perivascular mononucleate infiltrate. Direct immunofluorescence tests performed on two occasions on the skin of the abdomen and of the medial thigh surface were negative.(ABSTRACT TRUNCATED AT 250 WORDS)

A retrospective study of 134 cases of circulating "lupus anticoagulant" (LA) observed between 1975 and 1985 in the haemostasis laboratory of Henri Mondor Hospital is reported. In 66 p. 100 of cases the circulating anticoagulant was discovered fortuitously and auto-immune diseases were associated with the inhibitor only in 25 p. 100 of cases (mainly systemic lupus erythematosus). Thrombosis (venous, arterial or both) was found in 26 p. 100 of cases when auto-immune diseases were present and in 13.5 p. 100 of cases in the absence of these diseases. Spontaneous abortion was observed in 42 p. 100 of the women when the anticoagulant occurred in the course of an auto-immune disease and in 4 p. 100 when another or no underlying disease were identified. False-positive VDRL and thrombocytopenia were found associated with LA mainly in auto immune diseases. Anticardiolipin antibodies were positive in 37 p. 100 of 32 patients without any difference between auto-immune diseases and other pathologies. These results show that the "lupus anticoagulant" is a frequent coagulation abnormality even when auto-immune diseases are absent. The differences observed between auto-immune diseases and other pathologies in respect to clinical manifestations and biological findings associated with lupus anticoagulant suggest that the so-called "lupus anticoagulant" represents a group of antibodies with probably different specificities but which act in a similar manner in "in vitro" coagulation tests.

An authentic case of Waldenstrom's macroglobulinaemia without a tumoural syndrome is reported, with initial signs of peripheral pancytopenia. Complete and persistent remission appears to have followed multiple sequential chemotherapy. This very satisfactory and, according to data in the published literature, very rare result suggests that this type of treatment should be prescribed for all patients with macroglobulinaemia with poor prognostic clinical and histological characteristics.

Hereditary angioedema (HANE) is a rare, life-threatening disease due to the deficiency of C1 inhibitor (C1 Inh). Androgen therapy has been recently shown to be effective for prophylaxis of Hane attacks. Since life-long androgen therapy may be hazardous, this study was designed to define the minimal doses required for effectiveness. Ten patients from six different families were treated during cumulative 73 months by danazol and/for methandrostenolone. One tablet/day of either drug was the minimal requirement to prevent any attack in all patients. When 3 tablets/day were given, complement abnormalities were simultaneously rapidly reversed. When 1 tablet/day was given the biological effect was barely detectable, except for C2. Serum C2 levels may, therefore, represent the best criteria of androgen therapy effectiveness. Thus, an excellent clinical result can be obtained with much lower doses of androgens than previously stated. This result seemed important with respect to the serious dose-dependent risk of androgens.

Measurement of serum C3 does not provide precise informations concerning an eventual consumption of this complement component during an immunological process. An increased synthetic rate may compensate an accelerated catabolism. The study of breakdown products of C3, such as C3d is a more sensitive approach of the role of complement in some immunological disorders. Therefore C3d was measured in the serum of patients with chronic non systemic glomerular diseases. High values of serum C3d were found in all cases of hypocomplementemic glomerulonephritis. Circulating C3d was also increased to a lower extent, in patients with normocomplementemic nephritis such as minimal change disease, mesangial nephritis with IgA deposits and membraneoproliferative (type I) glomerulonephritis. The data suggested the involvement of complement in a number of glomerulonephritis. Participation of complement in immunological disorders particularly in chronic non systemic glomerulonephritis could require a reevaluation when functional tests are performed in addition to static measurements.

A case of benign monoclonal gammapathy associated with an auto-immune hyperlipidaemia and a chronic glomerulopathy is reported. Extraction of the monoclonal immunoglobulin made it possible to demonstrate its anti-lipoprotein antibody activity. Parallel changes in serum lipid levels and monoclonal immunoglobulin levels under the influence of melphalan confirmed the direction relationship between the lipid and protein disorders. The pathogenesis of the glomerulopathy and of the massive and prolonged activation of the classical complement pathway is more difficult to affirm. It is nevertheless possible that the renal involvement is due to an auto-immune complex disease.