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Biomedical subjects

G Lorette

Publications and source records attributed to G Lorette.

At least 199 records · Page 11Linked to original sources
PubMed1988

[Autoimmune pemphigus combined with alpha 1-antitrypsin deficiency].

It has been demonstrated experimentally that the bullae observed in autoimmune pemphigus are due to the action of proteases. So far, no case of pemphigus associated with deficiency in antiprotease has been reported. We present a case of pemphigus associated with familial deficiency in alpha 1-antitrypsin (alpha 1-AT), a major human body antiprotease. A 35-year old man presented with pemphigus preceded during 18 months, and accompanied by pruritus. The lesions were polymorphous, being made of solitary bullae, circinate vesiculobullae and squamous scabie plaques. Histopathological examination showed an intraepidermal bulla with acantholysis, a very spongiosis. The diagnosis of autoimmune pemphigus was confirmed by fluorescence of the epidermis in IgG and C3 and by the presence of antibodies directed against the intracellular substance. The initial treatment, which consisted of prednisolone 10 mg/kg/day and 10 plasma exchanges, was rapidly successful, but several relapses occurred thereafter. Two years after the pemphigus was diagnosed, a panlobular emphysema was discovered which made it possible to demonstrate a severe familial deficiency in alpha 1-AT of the Pi phenotype. This is the first published case of alpha 1-AT deficiency associated with autoimmune pemphigus. In our patient the skin disease presented as herpetiform pemphigus (initial features of dermatitis herpetiformis, followed by misleading polymorphous lesions, rare acantholytic cells, good response to corticosteroids), but there was no eosinophilic spongiosis. The frequency of alpha1-AT deficiency (estimated at 1 in 1,000 in northern Europe) and the lack of published cases with such an association may suggest a pure coincidence.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1988

Controlled study of plasma exchange in pemphigus.

To determine the potential steroid sparing effect of plasma exchange in pemphigus we enrolled 40 patients in a multicenter randomized study. Eighteen patients were treated by prednisolone alone, 22 by prednisolone plus ten large-volume plasma exchanges over four weeks. All patients received oral prednisolone in the same initial dosage (0.5 mg/kg/d), which was increased weekly if needed. The number of cases controlled at each therapeutic step did not differ between the two groups. In eight cases, four in each group, the disease was not controlled by the highest therapeutic step of the protocol, with four deaths from sepsis in the plasma exchange group. The controlled cases needed similar cumulative prednisolone doses (5237 +/- 5512 mg in the plasma exchange group vs 4246 +/- 1601 mg in the control group). The evolution of serum pemphigus antibody was not different in the two groups. These findings suggest that plasma exchange in association with low steroid doses alone are not effective in the treatment of pemphigus and may even promote sepsis.

Adult↗
PubMed1987

Erythromycin ethyl succinate: diffusion through interstitial dermal fluid.

Erythromycin is widely used in dermatology. There are few studies of its diffusion in the skin. The diffusion of erythromycin ethyl succinate (EES) in dermal fluid has now been investigated by the suction blister method. Suction blister fluid (SBF) and blood samples were collected from 10 volunteers before administration of 1 g EES and 10 times during the following 24 h. The median peak serum level was 2.05 micrograms/ml and in SBF it was 0.34 microgram/ml. The median ratio of the areas under curves (f SBF/f serum) was 43%. In all subjects EES concentrations in SBF between the second and twelfth hours exceeded 0.1 g/ml. The results show good diffusion of EES through normal skin from the second to the twelfth hours after oral administration.

Adult↗
PubMed1986

[Linear scleroderma in children (apropos of 27 cases)].

Twenty-seven cases of linear morphoea are reported and compared with 218 cases collected from the literature. The various parameters studied, including clinical features and laboratory results, were identical in both series. The aetiology of linear morphoea is unknown, even though injuries or fever have been noted as triggering factors in 20 p. 100 of the cases. Linear morphoea is a childhood disease: it begins at the age of 7 or 8 and predominates in females (63 p. 100 of the cases). Its onset is marked by the abrupt occurrence of sclerosis in most cases, although a solitary morphoea or a trophic plaque may precede the disease proper. In our series, muscular or articular involvement appeared from the start in 40 p. 100 of the patients at the same time as the initial cutaneous lesions. The active stage lasts 3 years and sometimes longer. It is characterized by extension (37 p. 100) or both extension and multiplication (63 p. 100) of the initial lesions. Regional complications aggravate linear morphoea and are more frequent in patients whose lesions extend and multiply. Their incidence was lower among the published cases than in our series. Depending on the author, retractile myositis is present in 37 to 59 p. 100 of the cases, joint stiffness in 18 to 40 p. 100 and shortening of a limb in 10 to 22 p. 100. These complications often regress incompletely, leaving sequelae which persist in the steady state in 75 p. 100 of the patients. Antinuclear antibodies, present in 37 p. 100 of the cases, are either of the homogeneous or of the speckled type. Jablonska has met them more frequently (50 p. 100), and they were often of the speckled type. The significance of these antibodies in linear morphoea is unclear since they appear inconstantly and later than clinical signs. The skin lesions associated with linear morphoea show that the other forms of scleroderma--i.e. plaque morphoea, erythematous atrophic or dyschromic plaques and guttate scleroderma--belong to the same family. The same associations are found in frontoparietal "coup de sabre" scleroderma. The treatment of linear morphoea is not yet standardized. At the moment, the best and most regular results are obtained with systemic corticosteroids and local physiotherapy.

Adolescent↗