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B Labeille

Publications and source records attributed to B Labeille.

At least 37 records · Page 2Linked to original sources
PubMed1989

Diagnosis of adult linear IgA dermatosis by immunoelectronmicroscopy in 16 patients with linear IgA deposits.

Homogeneous linear IgA deposits at the dermo-epidermal junction (DEJ) shown by direct immunofluorescence are characteristic of what is termed linear IgA bullous dermatosis. However, it is not yet certain that this disease constitutes an entity distinct from other subepidermal blistering diseases, especially when IgG deposits are also present. Sixty-one cases of subepidermal blistering disease in adults were therefore investigated by immunoelectron microscopy (IEM), and the 16 patients observed to have homogeneous linear IgA deposits were compared with the 45 who had no IgA but had IgG and/or C3. In 11 of the 16 patients with IgA (four of whom also had IgG), the deposits were linear and formed a mirror image pattern on each side of the lamina densa from which they were separated by a clear space. In contrast to this monomorphic IEM pattern, clinical and other laboratory findings were very heterogeneous, making exact clinical diagnosis difficult. Of the remaining five patients in this group of sixteen, three (all with both IgA and IgG) had bullous pemphigoid, epidermolysis bullosa acquisita, and cicatricial pemphigoid, respectively, on IEM and clinical investigation. In the remaining two patients (one with both IgA and IgG, and one with IgA only) the deposits were located in the lamina lucida, making precise classification impossible. None of the 45 patients with isolated IgG and/or C3 deposition displayed the mirror image pattern. We conclude that this IEM pattern may constitute a specific diagnostic criterion of linear IgA dermatosis.

Adult↗
PubMed1989

[Acute generalized exanthematous pustulosis].

The authors report the case of a young patient who developed an eruption resembling an acute generalized pustular dermatitis after amoxicillin therapy for a tonsillitis. The nosological distinctions between this clinical entity and pustular psoriasis are discussed.

Adolescent↗
PubMed1989

[Marfan disease presenting in neonates with rapid cardio-vascular failure. Apropos of 2 cases].

We report two exceedingly unusual and severe cases of Marfan syndrome with neonatal manifestations. Clinical features were caricatural. The skin was of the cutis laxa type. Both infants died shortly after birth as a result of severe cardiovascular lesions. Histologic examination of the aortic media confirmed the diagnosis. Because this genetic connective tissue dysplasia is inherited on an autosomal dominant basis with strong penetrance, and because in both instances the two parents had a negative family history, a dominant mutation seems likely in our cases and prompted us to be optimistic when giving genetic counseling. We point out the characteristic features of these exceptional early forms and describe the results of a detailed histologic examination of the skin that completes data from the literature.

Aorta↗
PubMed1988

Epidermolysis bullosa acquisita and Crohn's disease. A case report with immunological and electron microscopic studies.

A review of the literature shows that the association of epidermolysis bullosa acquisita and Crohn's disease is rare. A 27-year-old man developed cutaneous blisters on the trauma-prone areas that were consistent with the diagnosis of epidermolysis bullosa acquisita. Immunological and electron-microscopic studies of the skin showed intense IgG deposits located beneath the basal lamina. Three years later, Crohn's colitis was diagnosed. Prednisolone and sulfasalazine treatment resulted in an improvement of the bowel disease but without appreciable effect on the skin lesions.

Adult↗
PubMed1987

Immunoelectron microscopy in subepidermal autoimmune bullous diseases: a prospective study of IgG and C3 bound in vivo in 32 patients.

Thirty-two patients suffering from subepidermal autoimmune bullous disease were studied prospectively by clinical examination and immunoelectron microscopy. Clinically, 1 patient had herpes gestationnis, 14 typical bullous pemphigoid (BP), 3 epidermolysis acquisita (EBA), 3 cicatricial pemphigoid (CP), and 11 patients overlapping clinical diseases. These 11 patients shared clinical features of BP, EBA, or CP and a clinical diagnosis could not be done safely. Immunoelectron microscopy revealed diaminobenzidine deposits in 20 patients on the epidermal side of dermo-epidermal junction in the lamina lucida as in BP. In 5 patients, deposits located mostly under the anchoring fibril zone, in the floor of a sublamina densa dermoepidermal separation for 2 of them, were consistent with a diagnosis of EBA. In 6 patients, deposits were located mostly in the lamina densa, in the floor of a dermoepidermal separation occurring in the lamina lucida for 3 of them. This suggests that some of these 6 patients had neither EBA or BP, but another autoimmune bullous disease again, an uncharacterized component of dermoepidermal junction located in the lamina densa. Finally, a correlation exists between the sites of IgG and/or C3 components on epidermal or dermal side of dermoepidermal junction and the presence or absence of characteristic clinical features such as scar, milia formation, or mucosal involvement.

Adult↗
PubMed1987

[Frank's sign and coronary disease].

The authors study the sensitivity, the specificity and the predicting value of Frank's sign (presence of a groove at the level of the earlobe) on a group of 172 patients undergoing a clinical examination, an EKG at rest and effort, and a selective coronary arteriogram for suspicion of coronary disease. The criteria retained for the diagnosis of coronary disease is the presence of stenosis superior or equal to 75 p. cent in one of the three main coronary vascular trunks. Statistical studies using the CHI 2 test reveal a highly significant association between Frank's sign and coronary disease (p less than 0.001). The sensitivity of Frank's sign reaches 75 p. cent, its specificity 57.5 p. cent and its positive predicting value 80.3 p. cent. The predicting value is a function of the sex: it is a great deal lower in women (50 p. cent) than in men (84.7 p. cent). The prevalence of Frank's sign increases progressively with age: 42 p. cent in the 30-39 age group and 75.8 p. cent in the 60-69 age group. The predicting value remains high however beyond 60 years: predicting value of 77 p. cent. Frank's sign is correlated neither with the gravity of the coronary disease, nor the duration of the angina, nor with any of the risk factors studied here: tobacco, hypercholesterolemia, arterial hypertension, diabetes, obesity. Frank's sign is therefore considered as a marker of the coronary disease, independent of risk factors but frequently associated with them. If its absence does not permit in any way to exclude the diagnosis of coronary disease, its presence corresponds in three quarters of the cases to an established coronary disease within a symptomatic population.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗