[Cutaneous metastatic Crohn disease. Apropos of a case]. / Maladie de Crohn cutanée métastatique. A propos d'une observation.

The authors report the case of a 54-year old man suffering from facial papulonodular eruption. He has Crohn's disease which was diagnosed 36 years ago. There were no clinical symptoms of the disease when the cutaneous manifestations occurred. The biopsy of a cutaneous lesion showed cutaneous granuloma at skin's level. Having no other etiology, we shall diagnose it as a cutaneous metastatic Crohn's disease. Derived sulfasalazine (Pentasa) healed all lesions in 1 month. This observation reminded us to that it is essential to biopsy all atypical cutaneous lesions occurring in a Crohn's disease case.

[Capillary leak syndrome disclosing Ofuji's papuloerythroderma]. / Syndrome de fuite capillaire révélant une papulo-erythrodermie d'Ofuji.

INTRODUCTION: Capillary leak syndrome is a specific entity among syndromes with capillary hyperpermeability. Endothelial cell activation is related to the higt level of adhesion molecules (sICAM-1, sVCAM-&, sCD62E) possibly due to several cytokines (IL-2, TNF ...). CASE REPORT: An 84-year-old woman was hospitalized for erythroderma. Ofujui papuloerythroderma was diagnosed and edema was attributed to capillary leak. A kinetic study of several cytokines and adhesion molecules sCD62E, sVCAM-1 and sICAM-1 was done. Outcome was favorable with corticopuvatherapy. DISCUSSION: The capillary leak syndrome reported here is simlar to that described in other erythrodermas with or without lymphoma. The keratinocyte would be activated by the CD4 T lymphocyte via the gamma-interferon mediator. The T cell secretes cytokines (interleukin-1, tumor necrosis factor ...) which activates the endothelium and increases vascular permeability. The level of adhesion molecules and changes observed during the episode of edema demonstrated the endothelial activation.

[Bullous localization of a lupus erythematosus induced by radiotherapy]. / Lupus érythémateux bulleux: rôle localisateur de la radiothérapie.

INTRODUCTION: Connective diseases induced or exacerbated by radiotherapy are not frequent. We report a particular erosive erythematosus lupus with lesions in the precise distribution of radiation therapy given for a breast cancer. OBSERVATION: An 80 year-old woman presented with painful erosive skin lesions of her breast which had been treated by irradiation 9 years before. The patient also had rheumatoid arthritis. A skin biopsy showed keratinocyte necrosis, acantholysis and a dermal lymphocytic infiltrate under the basal cell layer. Direct immunofluorescence showed granular deposition of IgG, IgM and C3 along the basal cell layer. Antinuclear antibodies were positive at a titre of 1: 1,000. Erythematosus lupus diagnosis was established and annular lesions secondarily appeared on her neck, back, and arms. A treatment with hydroxychloroquine and topical corticosteroïds was effective in 3 months. DISCUSSION: The unusual erosive lesions and their localization on a previously irradiated site suggest the role of X-rays in our observation, despite their late appearance after radiotherapy. Radiotherapy and erythematosus lupus affect the same target structures (basal cells and small dermal capillaries) and could have additive effects. The presence of rheumatoid arthritis in the past medical history may have exaggerated this complication.

[Chronic lymphocytic leukemia revealed by atypic pemphigus]. / Pemphigus atypique révélateur d'une leucémie lymphoïde chronique.

BACKGROUND: Bullous diseases are occasionally reported during chronic lymphocytic leukemia. We report the case of a woman in which a bullous disease of difficult nosologic classification has revealed chronic lymphocytic leukemia. CASE REPORT: A 57-year-old woman, well-known for a rheumatoid arthritis, developed a bullous eruption. It was associated with voluminous lymphadenopathies, and laboratory investigations revealed a chronic lymphocytic leukemia. Clinicopathological aspect and immunofluorescence studies argued for a paraneoplastic pemphigus but immunoblot showed only antibodies against desmoglein 1. DISCUSSION: Paraneoplastic pemphigus is an autoimmune bullous disease defined by the criteria of Anhalt, including autoantibodies mostly directed against cytoplasmic proteins of the plakin family. Our case is unusual for the absence of these antibodies and for the presence of antibodies directed against a cell surface target, desmoglein 1. Such a case confirms the overlapping auto-immune responses in pemphigus.

[Panniculitis-fasciitis syndrome disclosing systemic erythematous lupus]. / Syndrome panniculite-fasciite révélant un lupus érythémateux systémique.

INTRODUCTION: Panniculitis is a common manifestation of lupus, but is rarely the first manifestation in systemic lupus erythematosus. We report a case associated with fasciitis. CASE REPORT: A 67-year-old man was hospitalized for fever, pleuropericarditis, subcutaneous diffuse infiltration of the two thighs and the abdomen due to panniculitis and lupic fasciitis. Antiphospholipid antibody test was positive. There was non antiphospholipid syndrome. COMMENTS: Panniculitis can occur simultaneously or a few months or years before systemic lupus erythematosus. An association with fasciitis should be entertained whenever the cutaneous involvement is extensive and firm. In such a situation, deep biopsies are indicated. Observations of further cases will help identify the role of general steroid therapy in fasciitis-panniculitis and systemic lupus erythematosus.

[Hepatic amebiasis in France. Apropos of 152 case reports]. / L'amibiase hépatique en France. A propos de 152 observations.

The authors report upon 152 cases of hepatic amebiasis (H.A.) observed in France between 1969 and 1983, among young european men (average age 29,2) who were hospitalised in the initial phase of their illness. H.A. was clinically revealed through a painful and febrile hepatomegaly in 3/4 cases and in 6 cases through complications. No chronic form was observed. The amebic serology (I.F.I. and/or H.A.P.) was always positive. The research of an intestinal portage of the parasite was generally negative. The hepatic functions were impaired in the third of the cases. Whichever technique was employed, the anatomic diagnosis has always been made in a phase of intra hepatic collection. The superiority of echotomography over other methods is confirmed (especially for diagnosis of multiple abscess: 47% of success). All patients recovered, most frequently through medical treatment (117 cases). The nitro-imidazoles (1,5 to 2 g per day during 7 to 14 days) remain the prefered treatment, but they are responsible for 5 failures which were overcome by emetine. On the other hand, 4 patients had, after being cured, showed middle and long term relapses without patient recontamination. The evolution of the treated H.A. is significantly correlated to the importance and/or the multiplicity of the liver collection: the voluminous abscess of the right lobe being the most potentially dangerous. The pathogenesis of the H.A. remains imperfectly known. The different clinical aspects which have been found, depend upon the preexistant immunity of the patient and could also be equally associated with a pathology of complex immunity systems.

Thermal exchanges during sleep in anhidrotic ectodermal dysplasia.

Anhidrotic ectodermal dysplasia is a congenital syndrome characterized by the absence of sweat glands. A sweating test was performed on such a patient and proved his inability to sweat. Thermal exchanges during night sleep were then measured in this patient and compared with data obtained from a healthy control subject. Ambient conditions were as follows: dry bulb temperature 32.2 degrees C, relative humidity 30%-40%, wind speed 0.7 m.s-1. Polysomnographic recordings showed normal sleep patterns in both subjects, but a "first night effect" in the patient. Rectal (Tre) and mean skin (Tsk) temperatures and loss of mass were monitored continuously throughout the 8-h sleep recording. Loss of mass averaged 34.1 g.h-1 in the patient vs 78.1 g.h-1 in the control subject. No relationship with sleep stages was observed in the patient, in contrast to the control subject who experienced a decrease in evaporation during rapid eye movement sleep. Body temperatures varied little in the patient, but decreased until the 6th h of sleep in the control subject. On two occasions there was a 0.3 degrees C fall in the Tre of the patient during two slow wave sleep (SWS) phases, while Tsk and loss of mass did not change. As thermolytic processes had not varied on these two occasions, it was concluded that the fall in Tre indicated a concomitant decrease in metabolic heat production, in agreement with the assumption that SWS represented a state of energy conservation.

Comparative field evaluation of HIV rapid diagnostic assays using serum, urine, and oral mucosal transudate specimens.

BACKGROUND: Comparative field utility of selected HIV-1 assays using homologous collections of serum, urine and oral mucosal transudate (OMT) was determined in adult populations from a tuberculosis hospital and STD clinic in Djibouti, East Africa. STUDY DESIGN: Enzyme immunoassay with confirmatory Western blot was performed on all serum specimens for comparison with rapid, instrument-free assays (SUDS HIV-1, Murex: TestPack HIV-1/2. Abbott; and COMBAIDS HIV 1 + 2, SPAN Diagnostics) using various specimen sources. Delayed (48 h post-collection) testing was also performed on urine. Sensitivity and specificity for the rapid assays, in descending order, were as follows: serum SUDS HIV-1 assay (100%, 98.3%), serum COMBAIDS HIV-1/2 assay (98.4%, 99.6%), and OMT SUDS HIV-1 assay (98.4%, 94.5%). RESULTS: The OMT EIA optical density cutoff value was modified resulting in an improved specificity from 89.1 to 99.6%, however, sensitivity decreased from 100 to 98.5%. Urine EIA and rapid assays demonstrated unacceptable test performance for use as a screening test.