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C Prost

Publications and source records attributed to C Prost.

At least 73 records · Page 4Linked to original sources
PubMed1993

A missense mutation in the rod domain of keratin 14 associated with recessive epidermolysis bullosa simplex.

Epidermolysis bullosa simplex (EBS) is a group of epidermal blistering diseases almost invariably transmitted as a dominant trait, which has recently been shown to arise from mutations in keratins 14 and 5 (K14 and K5). We describe a family with recessive EBS in which the disease is tightly linked to the substitution of the highly conserved glutamic acid-144 to alanine in the first helical segment of the rod domain of keratin 14. In contrast, linkage with keratin 5 was excluded. The loss of an ionic interaction with keratin 5 is likely to affect K14-K5 heterodimer formation. Our data suggest that this mutation underlies EBS in our family, and that mutations in keratin genes may impair the mechanical integrity of basal keratinocytes in a recessive as well as dominant fashion.

Alanine↗
PubMed1993

[Acquired localized epidermolysis bullosa. A case with scalp involvement and immunoelectron microscopic study].

The case of a 59-year old man who had a clinical Brunsting-Perry pemphigoid localized to the scalp is described. Direct immunoelectron microscopy demonstrated IgG and C3 immune deposits in the anchoring fibril zone, as in epidermolysis bullosa acquisita. No circulating antibodies were detected by Western immunoblotting on epidermal and dermal extracts. These findings show that Brunsting-Perry pemphigoid is not an immunological entity and that it widens the clinical spectrum of epidermolysis bullosa acquisita.

Autoantibodies↗
PubMed1993

Controlled trial of azathioprine and plasma exchange in addition to prednisolone in the treatment of bullous pemphigoid.

BACKGROUND AND DESIGN: Bullous pemphigoid is usually treated with systemic corticosteroids. Side effects are common in elderly patients, justifying the search for adjuvant therapy. This randomized, multicentric unblind study was designed to assess the efficacy of azathioprine or plasma exchange when added to conventional doses of prednisolone. One hundred patients with active disease entered the study. They were randomly allocated to receive 28 days of treatment with oral prednisolone sodium metasulfobenzoate (1 mg/kg per day) either alone or in combination with oral azathioprine (100 to 150 mg/d) or four large-volume plasma exchanges. After 28 days, the prednisolone doses were progressively decreased according to the same strict regimen in the three groups (in combination with oral azathioprine in group 2). RESULTS: The clinical results were evaluable in 98 of the 100 patients included in the study. There was no appreciable difference in the percentages of complete remission of the disease in the three therapeutic groups at 28 days (71%, 80%, and 71%, respectively) or at 6 months (42%, 39%, and 29%, respectively). Severe complications were more often observed among patients receiving azathioprine. At 6 months, 14 of 98 patients had died, without any differences noted among the three study groups. CONCLUSIONS: We conclude that neither azathioprine nor plasma exchange is effective enough to be used routinely as an adjuvant to corticosteroids in the management of bullous pemphigoid.

Aged↗
PubMed1993

Bullous amyloidosis. Report of 3 cases and review of the literature.

We report 3 cases of bullous immunoglobulinic amyloidosis and review 25 published cases. In 2 of our patients, amyloid deposits were not detected with special staining, but by means of ultrastructural methods. Investigations of the skin lesions permitted the diagnosis of associated plasma cell dyscrasia in 2 patients. Unexplained bullous lesions should be investigated for amyloid deposits and the presence of monoclonal gammopathy by methods including electron microscopy and immunochemical analysis of serum and urine.

Aged↗
PubMed1992

Epidermolysis bullosa acquisita in a 3 1/2-year-old girl.

A 3 1/2-year-old girl had a subepidermal bullous eruption with immunopathologic features that were consistent with epidermolysis bullosa acquisita or bullous systemic lupus erythematosus. This report highlights the difficulty encountered in distinguishing between epidermolysis bullosa acquisita and other bullous disorders that involve the dermoepidermal junction and the need for modern immunologic investigations in the diagnosis of bullous diseases in children.

Child, Preschool↗
PubMed1992

Localized pretibial pemphigoid and pemphigoid nodularis.

We describe a 75-year-old woman with a chronic, blistering eruption on the left leg whose clinical and immunopathologic features were consistent with a diagnosis of localized pretibial pemphigoid. After a disease-free interval of 5 years she developed a generalized prurigo nodularis-like eruption. Immunofluorescence studies revealed deposition of IgG and C3 along the dermoepidermal junction, and circulating autoantibodies against the dermoepidermal junction were demonstrated. Indirect immunoelectron microscopic examination of saponin-treated skin samples showed deposits of immunoreactants over the intracellular part of the hemidesmosomes. By Western immunoblotting the 230 kd bullous pemphigoid antigen was recognized by circulating autoantibodies. Thus our patient had two unusual clinical variants of bullous pemphigoid: localized pretibial pemphigoid and pemphigoid nodularis.

Aged↗
PubMed1992

The major cicatricial pemphigoid antigen is a 180-kD protein that shows immunologic cross-reactivities with the bullous pemphigoid antigen.

Recent studies have shown that sera from patients with cicatricial pemphigoid (CP) contained autoantibodies against epidermal antigens of molecular weight 230 kD and/or 180 kD by immunoblotting, similar to those recognized by bullous pemphigoid (BP) sera. Previous immunoprecipitation studies have shown that BP sera only precipitated the 230-kD antigen. To characterize the CP antigen(s) we tested 10 CP sera, 10 BP sera, and four controls by both immunoprecipitation of radiolabeled cells and immunoblotting of epidermal extracts. For immunoprecipitation, we used 0.5% NP-40 extracts of both normal human keratinocytes and Pam cells. All CP sera precipitated a 180-kD protein that co-migrated with the BP180 antigen precipitated by some individual BP sera. Two of these CP sera also faintly bound a 230-kD protein of similar molecular weight as the major BP230 antigen. CP and BP sera with an immunoblotting pattern of 180 kD immunoprecipitated a co-migrating 180-kD protein. CP sera reacting by immunoblotting with the 230-kD antigen precipitated the 180-kD and/or the 230-kD antigen. In contrast, BP sera reacting with the 230-kD antigen only precipitated this antigen. In further experiments, labeled 0.5% NP-40 extracts from Pam cells were first preabsorbed with a reference BP serum and then immunoprecipitated with CP sera. Under these conditions, CP sera that immunoprecipitated both 180-kD and 230-kD proteins with the standard procedure no longer precipitated these proteins. Our results suggest that a 180-kD protein is the major CP target-antigen that demonstrated immunologic cross-reactivities with the BP180 and the BP230 antigens.

Antigens↗
PubMed1992

Effects of a combination of evening primrose oil (gamma linolenic acid) and fish oil (eicosapentaenoic + docahexaenoic acid) versus magnesium, and versus placebo in preventing pre-eclampsia.

In a placebo controlled, partially double-blinded, clinical trial, a combination of evening primrose oil and fish oil was compared to Magnesium Oxide, and to a Placebo in preventing Pre-Eclampsia of Pregnancy. All were given as nutritional supplements for six months to a group of primiparous and multiparous pregnant women. Some of these women had personal or family histories of hypertension (21%). Only those patients who received prenatal care at the Central Maternity Hospital for Luanda were included in the study. Compared to the Placebo group (29%), the group receiving the mixture of evening primrose oil and fish oil containing Gamma-linolenic acid (GLA), Eicosapentaenoic acid (EPA), and Docosahexaenoic acid (DHA) had a significantly lower incidence of edema (13%, p = 0.004). The group receiving Magnesium Oxide had statistically significant fewer subjects who developed hypertension of pregnancy. There were 3 cases of eclampsia, all in the Placebo group.

Adolescent↗
PubMed1992

[Does bullous pemphigoid with negative direct immunofluorescence exist? Apropos of 3 cases].

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)

Aged↗
PubMed1991

Healing of full-thickness cutaneous wounds in the pig. I. Immunohistochemical study of epidermo-dermal junction regeneration.

In order to determine the kinetics of epidermo-dermal junction (EDJ) regeneration during would healing, we studied the regeneration of five EDJ components during reepidermization. Cutaneous wounds (50-mm length, 2-mm width, and 5-mm depth) were produced on the flank area of two pigs and left unsutured. Daily biopsies from day 1 to day 20 were studied by light microscopy on paraffin-embedded sections and by indirect immunofluorescence on cryostat sections using human sera to bullous pemphigoid antigen (BPA) with specificity previously confirmed by indirect immuno-electron microscopy, rabbit antisera to type IV collagen (Coll IV) and to fibronectin, and the monoclonal antibodies (MoAb) 4C 12-8 to laminin and NP-76 to type VII collagen (Coll VII). Histologically, reepidermization started from day 1 and progressed unidirectionally and exclusively from the wound edges. Up to day 9, the distal tips of the neo-epidermal tongues generally extended between the crust and the granulation tissue (GT). They fused on day 10, restoring epidermal continuity. For each EDJ component, the date of appearance (emergence), the spreading under the neo-epidermis tongue (expression), and the morphologic aspect of the labeling were studied. BPA and Coll IV were detected from day 1 to day 20 and found to be expressed all along the neo-EDJ. Fibronectin and laminin were detected from day 1, were present in the proximal and median zones of the neo-EDJ before day 7, up to the distal tip from day 7 to day 9 and were all along the neo-EDJ from day 10 to day 20. Coll VII was only detected from day 3. It was present in the proximal zone on day 3 and day 4, in the proximal and median zones on day 5 and day 6, than all along the neo-EDJ from day 7 to day 20. From day 10, all the labeling characteristics of the five components were found to be similar in the neo-EDJ and in the normal EDJ. With regard to the neo-epidermis progression, we found a synchronism of emergence and expression for BPA and Coll IV, a synchronism of emergence but a delay of expression for fibronectin and laminin and lastly, a delay of emergence and expression for Coll VII. We concluded that BPA and Coll IV could constitute the framework on which the neo-EDJ is progressively built by adjunction of the other components, restitution being obtained just after epidermal continuity is restored.

Animals↗
PubMed1991

Cicatricial pemphigoid antigen differs from bullous pemphigoid antigen by its exclusive extracellular localization: a study by indirect immunoelectronmicroscopy.

Several components of the dermal-epidermal junction (DEJ) bear the name of the autoimmune bullous disease in which they are involved. The epidermolysis bullosa acquisita (EBA) antigen, a component of anchoring fibrils, and the bullous pemphigoid (BP) antigen, a component of hemidesmosomes (HD) with a molecular weight of 220-240 kD, have been well characterized. In contrast, there is little data known about the cicatricial pemphigoid (CP) antigen. No differences between CP and BP have been reported when sera of patients were studied by Western immunoblotting. Findings of a study of sera from 8 patients with CP by indirect immunoelectron microscopy (IEM) on normal human skin are reported. Saponin (0.1% 10 mn) was used as a permeabilizing agent of cytomembranes and saponin-treated skin samples were compared to saponin-untreated skin samples. Four sera from patients with BP, one from a patient with EBA, and three from healthy donors served as controls. The CP sera produced a similar staining of DEJ on both saponin-treated and saponin-untreated skin samples: immune deposits were localized over the lamina densa and the lower part of the lamina lucida clearly separated from the cytoplasmic membrane of keratinocytes, in regularly spaced clumps. The BP sera produced an intense staining of DEJ only on saponin-treated skin samples: immune deposits were observed on the cytoplasmic attachment plaque of the HD; on saponin-untreated skin samples, BP sera produced only a faint staining of the extracellular part of HD. Finally, as expected the EBA serum appeared on the lower part of the lamina densa and anchoring fibrils, and no DAB deposits were observed with the serum of healthy donors. This data indicated that CP antigen is different than BP antigen by its exclusive extracellular localization. It may be a component of anchoring filaments.

Autoantigens↗
PubMed1991

The subclass distribution of IgG autoantibodies in cicatricial pemphigoid and epidermolysis bullosa acquisita.

To study the subclass distribution of autoantibodies and their complement-fixing capacity in cicatricial pemphigoid (CP) and epidermolysis bullosa acquisita (EBA) we studied the sera from 23 patients by both indirect immunofluorescence (IIF) on 4-microns cryostat sections of normal human skin and immunoblotting of epidermal or dermal extracts. Monoclonal antibodies of strict specificity for human IgG subclasses were used. Sera from 20 patients with BP served as controls. In addition, total IgG subclass levels were determined by indirect competitive ELISA in all sera. Complement binding capacity was studied by IIF using antibodies to C3 after incubation of skin section with autoantibodies and source of fresh complement. CP autoantibodies reacting with the 230-240 kD and/or the 180-kD epidermal bands showed an IgG4/IgG1 subclass restriction, with a predominance of IgG4 in 10 cases, of IgG1 in four. In BP sera, IgG4 and IgG1 autoantibodies were detected with a similar frequency (100% and 83%, respectively). In EBA sera, autoantibodies reacting with the 290 kD and 145 kD dermal bands also showed an IgG1/IgG4 restriction. Concordant results were obtained by IIF. However, the IIF method had a lower sensitivity for the detection of IgG4 CP antibodies and IgG1 EBA antibodies than immunoblotting. Finally, when CP antibodies were analyzed for their complement-binding activity, it was found that sera containing IgG4 autoantibodies alone never fixed complement whereas all complement-fixing CP sera had IgG1 autoantibodies, suggesting that only this subclass of antibodies is capable of fixing complement.

Aged↗