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Biomedical subjects

G Lorette

Publications and source records attributed to G Lorette.

At least 55 records · Page 3Linked to original sources

[Cicatricial pemphigoid disclosed by superficial desquamative gingivitis. Clinical and immuno-electron microscopic study of a case].

Six months before consulting, a 71-year old man developed buccal and genital erosions which gradually became worse. Physical examination showed signs of superficial desquamative gingivitis, wide erosions on the bony palate, erythemato-erosive balanoposthitis and 7 bullae or skin erosions on the upper part of the back. At histopathological examination of a cutaneous bulla there was dermoepidermal cleavage and an inflammatory infiltrate without eosinophils. At direct immunofluorescence, linear deposits of IgG and C3 were present along the basement membrane. A search for anti-skin autoantibodies was negative at indirect immunofluorescence but positive at immunoblotting (240 Kd band). The cicatricial pemphigoid was treated with dapsone alone in doses of 100 mg/day. Treatment was continued for 6 months, resulting in complete cure of the mucosal and cutaneous lesions. An immunoelectromicroscopic study, performed according to the technique described by Prost et al., on a fragment of skin from around the bullae, showed deposits of granular IgG in the lamina lucida and the lamina densa and deposits of C3 in the lamina densa. This case of cicatricial pemphigoid exhibited 3 features which are not usually found in bullous pemphigoid. Clinically, the buccal lesions were located on the gums and on the hard palate, i.e. where the mucosa adheres to the underlying bone through the periosteum. This location is habitual in cicatricial pemphigoid and differs from that of the bullous pemphigoid lesions which affect the free mucosa lining the cheeks and the soft palate. Treatment with dapsone was dramatically successful in our patient whose lesions disappeared in 6 months.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged

Cutaneous cryptococcosis resembling molluscum contagiosum: a first manifestation of AIDS.

A 30-year-old homosexual man developed multiple skin umbilicated lesions resembling molluscum contagiosum. Initially the lesions were on his face but they rapidly spread. Histopathology and mycologic cultures of a skin biopsy revealed cryptococcus neoformans which was also identified in cerebrospinal fluid and in bronchoalveolar washings. The patient had fever, weight loss, generalized lymph node enlargement, depletion of the T helper subpopulation and positive HIV-1 serology. During treatment with flucytosine and amphotericin B, the skin lesions regressed in 3 months (cryptococcus neoformans disappeared in the cerebrospinal fluid and skin within one and five weeks, respectively). Our case demonstrates that molluscum contagiosum-like skin manifestations may be caused by cryptococcal infections. So it is necessary to perform skin biopsy in HIV seropositive patients with skin lesions resembling molluscum contagiosum, to diagnose mycotic infections, and especially cryptococcosis. Cutaneous cryptococcosis was, in this case, the first symptom of AIDS.

Acquired Immunodeficiency Syndrome

[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

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