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B Labeille

Publications and source records attributed to B Labeille.

At least 19 recordsLinked to original sources
PubMed2006

[Lupus profundus and myelopathy].

BACKGROUND: We report a case of myelopathy during the course of lupus profundus that is unique to our knowledge. CASE-REPORT: A 29-year-old woman had lupus profundus since 1999, initially associated with thrombopenia (28,000 platelets/mm3) treated with corticosteroids for 6 months. Several nodular eruptions occurred from 1999 to 2004. Antinuclear antibodies were positive at 1/320 to 1/640 and complement C4 fraction was low. While being treated with hydroxychloroquine for a recent flare-up of nodular lesions of lupus profundus, she complained of paraesthesia of the abdominal wall and of the upper and lower limbs, suggestive of a medullary lesion. MRI disclosed an image of acute myelitis at the level of the second cervical vertebra. High doses of corticosteroids were promptly administered intravenously (methylprednisolone bolus) followed by oral prednisone and hydroxychloroquine. Six months later the patient was free of neurologic or cutaneous symptoms. DISCUSSION: In the literature, systemic signs are rarely associated with lupus profundus and myelitis has never been reported. Lupus myelitis is a vascular and/or demyelinating and usually segmental lesion of the spinal cord. Neurologic symptoms are those of acute rather than chronic or recurrent transverse myelitis. The prognosis is poor with frequent and severe functional sequelae. Treatment is mainly based on high-dose systemic corticotherapy alone or combined with cyclophosphamide. Our patient responded favourably to early methylprednisolone bolus followed by oral corticosteroids and antimalarial treatment. CONCLUSION: Lupus profundus is classically of benign course with only cutaneous involvement, but it may sometimes be a sign of systemic lupus and in rare cases may be associated with severe complications.

Acute Disease↗
PubMed2004

[Relationship between clinical findings of patients with bullous pemphigoid and antigens recognized by their circulating antibasement membrane zone antibodies].

INTRODUCTION: Clinical features and extent of bullous pemphigoid lesions differed widely among patients. The pathogenic role of anti-BPAG2 antibodies has been recently demonstrated. The aim of this study was to analyze the relationship between clinical features of bullous pemphigoid patients and the antigens recognized by their serum. PATIENTS AND METHODS: One hundred and twelve bullous pemphigoid patients were included in this prospective multicenter study. Inclusion criteria were the following: 1) diagnosis of bullous pemphigoid established on the presence of 3 of the 4 clinical features of bullous pemphigoid, histological picture of bullous pemphigoid and positive direct immunofluorescence; 2) serum available for immunoblotting studies. The clinical and biological findings were prospectively recorded on standard forms. Sera were collected and analyzed using indirect immunofluorescence and immunoblotting on human epidermal extracts. RESULTS: Analysis of patient's clinical features depending on the antigens recognized by their serum showed that patients whose serum contained anti-BPAG1 antibodies had more frequently pruritus, blisters on the lower limbs and a positive indirect immunofluorescence. Patients whose serum contained anti-BPAG2 antibodies had blisters more frequently localized on the head, and a more frequently negative indirect immunofluorescence. Patients whose serum was negative by immunoblotting had less frequently urticarial and/or eczematous lesions, bullae less frequently localized on the lower part of the trunk, abdomen and lower limbs, lower eosinophilia and a more frequently negative indirect immunofluorescence. CONCLUSION: Patients with circulating anti-BPAG1 antibodies exhibited the most typical, clinical and biological features of bullous pemphigoid.

Aged↗
PubMed1998

Evaluation of clinical criteria for diagnosis of bullous pemphigoid. French Bullous Study Group.

OBJECTIVE: To check the potential usefulness of clinical criteria for the diagnosis of bullous pemphigoid when state-of-the-art techniques such as Western immunoblotting, immunoprecipitation, and indirect immunofluorescence on salt-split skin or direct immunoelectron microscopy are not available. DESIGN: Comparison of the clinical criteria between 2 groups (with and without bullous pemphigoid) as defined by immunoelectron microscopy used as standard criterion, in a prospective study. Multivariate logistic regression analysis was carried out by including all items that were statistically significant (at P < .05 level) in univariate analysis. SETTING: Five dermatology departments in teaching hospitals. PATIENTS: The 231 patients studied had subepidermal autoimmune bullous diseases with linear IgG or C3 deposits in the basement membrane zone (157 with bullous pemphigoid, 33 with cicatricial pemphigoid, 30 with epidermolysis bullous acquisita, 5 with lupus erythematosus, and 6 others). A second set of patients was used to calculate predictive values. RESULTS: The multivariate logistic stepwise analysis resulted in a final set of predictors that included only 4 items: absence of atrophic scars, absence of head and neck involvement, absence of mucosal involvement, and age greater than 70 years. No additional variables met the .05 significance level to enter into the model. If 3 of these 4 characteristics were present, a diagnosis of bullous pemphigoid could be made with a sensitivity of 90% and a specificity of 83%; these predictive values were calculated on a sample of 70 new cases. CONCLUSIONS: With and estimated incidence of bullous pemphigoid among subepidermal autoimmune bullous diseases of 80%, the presence of 3 of the 4 significant criteria allows the diagnosis of bullous pemphigoid, with a positive predictive value of 95%. Our set of clinical criteria thus allows the diagnosis of bullous pemphigoid with good validity for both clinical practice and therapeutic trials.

Aged↗
PubMed1998

[Acquired tufted angioma in an adult].

INTRODUCTION: Acquired vascular tumors have been raising new interest since Kaposi's illness was discovered in human immunodeficiency virus infection. We herein report a case of a rare different entity of acquired vascular tumor, described by E. Wilson-Jones for the first time in 1976. CASE REPORT: A 69-year-old woman had had for several months an asymptomatic erythematous lesion on the face gradually increasing in size. Histopathologic findings were pathognomonic of "tufted" angioma. The patient did not receive any treatment because of uncertain efficiency and because her lesion was well tolerated. DISCUSSION: "Acquired tufted angioma" is a benign angioma of the skin mainly occurring in children and young adults of both sexes. Histopathologic findings are pathognomonic. Typically, tufted angioma enlarges for a few years and then ceases growing and remains stable. Spontaneous regression may occur. The principal differential diagnoses are Kaposi's illness and low grade malignant angiosarcoma. Pulsed dye laser in the most efficient treatment.

Aged↗
PubMed1995

Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Bullous Diseases French Study Group.

BACKGROUND AND DESIGN: The incidence and distribution of autoimmune subepidermal bullous diseases were estimated from prospective data (including immunoelectron microscopy) obtained from 100 cases during a mean period of 35 months in three university dermatologic centers in Amiens, Limoges, and Tours, France, that correspond to a cumulative reference population of 3.55 x 10(6). RESULTS: Using data from these regions, we found a mean annual incidence of autoimmune subepidermal bullous diseases to be 10.4 per million people and, therefore, estimated the overall number of new cases of these disorders in France to be about 590 cases per year. According to clinical and immunoelectron microscopic criteria, a precise diagnosis was established in 94 cases, distributed as follows: bullous pemphigoid, 69 cases; cicatricial pemphigoid, 12 cases; linear IgA dermatosis, five cases; herpes gestationis, four cases; epidermolysis bullosa acquisita, two cases; and vesiculobullous systemic lupus erythematosus, two cases. CONCLUSION: Our prospective study is the first assessing the incidence and distribution of autoimmune subepidermal bullous disorders that systematically included immunoelectron microscopic data. Our estimated incidence of bullous pemphigoid (seven new cases per million people per year) is large enough to establish bullous pemphigoid as the major autoimmune subepidermal bullous disease for the purpose of therapeutic trials. On the contrary, all other disorders, particularly epidermolysis bullosa acquisita (estimated annual incidence, 0.17 to 0.26 per million people), were very rare and reflect the paucity of patients available for short-term clinical studies in France.

Adult↗
PubMed1994

[Acquired cutaneous osteomatosis. A primary monomelic form].

Eight cases of sporadic acquired primary osteoma cutis have been reported in the literature. Age at onset varies from 16 to 55 years with a mean of about 35 years. The sex ratio is 1 and a wide range of localizations have been reported. There is no known treatment. We report a new case of primary osteoma cutis observed in an adult. The monomelic feature of this case has not been reported previously. The patient was 76 years old and had multiple painless, stone-like formations at several sites on the left thigh and leg since the age of 40. Histological examination of skin biopsies showed a perfectly differentiated bone tissue in the dermal layer. There was no similar family history nor abnormal morphotype. Likewise, the absence of laboratory signs of pseudohypoparathyroidism, together with the late and spontaneous onset allowed us to eliminate hereditary Albright's osteodystropy or secondary osteomatosis due to a local pathological process. Despite the late onset, the monomelic character of the osteomas observed and the association of hemicorporeal hypertrophy and linear basocellular naevi reported in the literature would suggest a hamartomatous origin rather than a metaplasic process in this patient.

Adolescent↗
PubMed1993

[Acute febrile neutrophilic dermatitis (Sweet's syndrome) during therapeutic agranulocytosis in acute myeloblastic leukemia].

INTRODUCTION: the association of acute febrile neutrophilic dermatosis (Sweet's syndrome) with malignant haemopathies is well known and characterized by an usual lack of hyperleukocytosis: indeed, moderate neutropenia is often reported. However, cases of Sweet's syndrome in the agranulocytosis stage are exceptional (7 in the literature). CASE-REPORT: We report the case of a woman with acute myeloblastic leukaemia who had presented with Sweet's syndrome in the phase of therapeutic aplasia during induction of treatment, in the absence of white blood cells transfusion or treatment with haematopoietic growth factor (GM CSF, GCSF). COMMENTS: the physiopathology of Sweet's syndrome is unknown. Various mechanisms have been suggested, including immune reaction type III, increased interleukin-1 synthesis, increased chemotaxis of neutrophils, action of haematopoietic growth factors, iatrogenic effect of some drugs (e.g. cotrimoxazole, furosemide or minocycline). Yet none of these mechanisms involving circulating polymorphonuclears or their bone marrow precursors can explain the occurrence of Sweet's syndrome in the phase of agranulocytosis. CONCLUSION: the diagnosis of Sweet's syndrome must be considered in patients with agranulocytosis in order to avoid ineffective antibiotics and to initiate a corticosteroid therapy that will accelerate the cure of this benign dermatosis.

Agranulocytosis↗
PubMed1993

[Accidents caused by iontophoresis].

Ionophoresis is a well-established treatment for idiopathic hyperhidrosis. Modern apparatuses are reliable as long as the electrical equipment is used correctly. The authors present five cases of cutaneous incidents resulting in burns and secondary necrosis. Two of the cases could be explained by defective protection and the other three were apparently of the same type although non confirmation could be established. In comparison with uneventful incidences (pruritus, erythematous reactions, dysaesthesia) these spectacular burns should not exist. Machines must be regularly check and only electrodes with optimal protection should be used.

Adult↗
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↗
PubMed1992

[Panniculitis in children].

Panniculitis is an inflammation of subcutaneous fat responsible for fairly uniform clinical features including nodules, plaques or swelling located in the subcutaneous tissue, occasionally progressing to atrophy of subcutaneous fat. Some forms of panniculitis specific of childhood are responsible for readily recognizable clinical patterns. Examples include acute neonatal fat necrosis, sclerema neonatorum, cryopanniculitis, steroid-induced panniculitis, and toxoplasmosis-related multinodular fat necrosis of Royer syndrome. With the exception of erythema nodosum, the cause of panniculitis is identified upon histologic examination of a deep skin biopsy which ensures the differentiation of infectious nodules, vasculitis nodules, and apparently primary forms of nodular panniculitis fitting the description of Weber-Christian syndrome, Rothmann-Makaï syndrome, histiocytic cytophagic panniculitis or lupus panniculitis.

Biopsy↗
PubMed1991

Trichomegaly, pigmentary degeneration of the retina and growth disturbances. A probable autosomal recessive disorder.

Two brothers are described with trichomegaly, early pigmentary degeneration of the retina, growth retardation, anterior pituitary deficiencies and peripheral neuropathy. This syndrome, initially reported in a boy by Olivers and Mac Farlane in 1965 (6), and thereafter in six sporadic cases of both sexes, is not associated with a recognizable chromosomal defect. The present report of two brothers of healthy parents with negative familial history suggests an autosomal recessive mode of inheritance of this entity.

Adult↗
PubMed1991

[Leg ulcers in Werner's syndrome. Report of one case].

Werner's syndrome (adult progeria) is a rare autosomal recessive condition characterized mainly by a characteristic habitus (short stature, light body weight) scleroderma like changes of the limbs and premature aging. Chronic leg ulcers appears in about fifty per cent of the patients. These ulcers can be related to the combination of mechanical factors on atrophic subcutaneous tissue and skin of the feet and leg associated with early arteriosclerosis (20%) and diabetes mellitus (60%).

Adult↗