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Biomedical subjects

E Thomine

Publications and source records attributed to E Thomine.

At least 55 records · Page 3Linked to original sources

[Melkersson-Rosenthal syndrome treated by thalidomide. 2 cases].

INTRODUCTION: Treatment of Melkersson-Rosenthal syndrome (MRS) remains uncertain. Two cases of MRS treated with thalidomide are described. CASE REPORT: Two patients with cheilitis granulomatosa and recurrent facial edema have been treated with thalidomide (100 mg daily) for 3 and 6 months respectively. The efficacy of thalidomide was complete clinically and histologically in one case. In the second one, thalidomide suppressed attacks of facial edema and reduced lip swelling. COMMENTS: These cases suggest a therapeutic effect of thalidomide in MRS.

Adult↗

Coexistent cutaneous T-cell lymphoma and B-cell malignancy. French Study Group on Cutaneous Lymphomas.

BACKGROUND: The coexistence of cutaneous T-cell lymphoma (CTCL) and a B-cell malignancy (BCM) is rare. OBJECTIVE: Our aim was to assess the clinical and pathologic aspects of coexistent CTCL and BCM and to examine potential explanations for this association. METHODS: We report six cases of concurrent CTCL and BCM in which B- and T-cell lineages were demonstrated by immunologic studies. The literature includes 13 additional cases. All 19 CTCL-BCM cases are reviewed. RESULTS: CTCL either preceded or followed the BCM, which was a low-grade malignancy in most cases (16 of 19). Possible explanations for the association include a genetic predisposition, underlying viral infection, chemotherapy-induced carcinogenesis, stimulation of a B-cell clone by malignant helper T cells, and alterations in progenitor cells before determination of B- and T-cell lineage. CONCLUSION: An alteration in progenitor cells, with subsequent oncogenic activation of variable origin, might account for most cases of coexistent CTCL and BCM.

Adult↗

Morphometric analysis of melanocytic tumors. A study of nuclear area and density.

Because the differential diagnosis of melanocytic tumors is sometimes difficult, we performed a morphometric analysis of 20 benign nevi, 20 malignant melanomas, and 10 Spitz's nevi. The nuclear pleomorphism and the maturation to the depth of the tumor were quantified by measuring nuclear area and nuclear density at serial levels of the tumor (every 220 microns of thickness). Data obtained were analyzed statistically. Linear regression was used to represent the variation of nuclear area to the depth, and the standard deviation of nuclear areas reflected nuclear pleomorphism. We attempted to separate different tumors from each other using the Jacknife procedure and morphometric data. Using the Jacknife procedure rather than the usual histological procedures, we were able to distinguish benign nevi from Spitz's nevi and malignant melanoma in as many as 92% of cases, but we distinguished Spitz's nevi from malignant melanoma in only up to 60% of cases. In conclusion, we found it difficult to distinguish between Spitz nevi and melanoma using nuclear morphometric parameters. Other studies using new morphometric parameters are needed in order to improve our ability to make this discrimination.

Adolescent↗

Overlapping distribution of autoantibody specificities in paraneoplastic pemphigus and pemphigus vulgaris.

Paraneoplastic pemphigus is an autoimmune bullous skin disease in which autoantibodies immunoprecipitate a characteristic antigenic complex. The objective of this study was to analyze by immunoblotting and immunoelectron microscopy the autoimmune response in five patients with clinical and immunohistologic features typical of paraneoplastic pemphigus. In a first series of experiments, immunoblotting and immunoelectron microscopy were performed using anti-human whole Ig. Although immunoblotting results were consistent with the autoantibody specificities previously described in paraneoplastic pemphigus sera, immunoelectron microscopy demonstrated the presence of Ig deposits on desmosomal plaques, on hemidesmosomes and, surprisingly, on both the extracellular part of desmosomes and the keratinocyte plasma membrane. In a second series of experiments, immunoblotting and immunoelectron microscopy were carried out using antihuman IgG subclasses. The major observation was that two sera contained, in addition to the anti-desmoplakins I-II, anti-185-kD and anti-230-kD autoantibodies, autoantibodies that stained the desmoglea by indirect immunoelectron microscopy and bound to a 130-kD polypeptide by immunoblotting. One serum was particularly demonstrative: IgG1 bound to the 250- and 220-kD bands corresponding to desmoplakins I and II on immunoblots and to the desmosomal plaques of keratinocytes in immunoelectron microscopic preparations; IgG3 recognized a 185-kD immunoblotting band and hemidesmosomes and desmosomal plaques by immunoelectron microscopy; IgG4 bound to the 130-kD immunoblotting band of pemphigus vulgaris and labeled the desmoglea and the keratinocyte plasma membrane by immunoelectron microscopy. These results demonstrate that the paraneoplastic-pemphigus autoimmune response involves both intracellular and extracellular desmosomal antigens and suggest an overlapping distribution of autoantibody specificities among autoimmune bullous skin diseases.

Aged↗

Thiopronine-induced herpetiform pemphigus: report of a case studied by immunoelectron microscopy and immunoblot analysis.

We report a case of herpetiform pemphigus induced by thiopronine. Direct immunoelectron microscopy performed on peribullous skin showed IgG deposits predominantly located in the extracellular portion of desmosomes. By immunoblot analysis using bovine tongue extracts as the antigen, the patient's serum recognized a 160-kDa polypeptide which comigrated with desmoglein I. This study underlines the contribution of immunoblot analysis to the diagnosis of atypical cases of pemphigus, and confirms that herpetiform pemphigus may be a clinical variant of pemphigus foliaceus.

Aged↗

Erythema gyratum repens. A case studied with immunofluorescence, immunoelectron microscopy and immunohistochemistry.

We report a patient with erythema gyratum repens (EGR), in whom a bronchial carcinoma was found. Direct immunofluorescence revealed granular deposits of immunoglobulins at the basement membrane zone (BMZ) in the skin, and in the lung tumour. Direct immunoelectron microscopy showed that the immune deposits were localized just beneath the lamina densa. Indirect immunofluorescence revealed circulating anti-BMZ antibodies. Immunohistochemical staining, using anti-transforming growth factor-beta, anti-epidermal growth factor receptor, anti-vimentin and anti-alpha-actin, was found to be more intense in the lesional skin and the lung tumour than in normal tissues. Possible mechanisms in the pathogenesis of EGR are discussed.

Autoantibodies↗

DNA flow-cytometric analysis of basal cell carcinomas and its relevance to their morphological differentiation: a retrospective study.

DNA flow cytometry (FCM) was performed on archived material of 82 basal cell carcinomas (BCC), using Hedley's technique. Sixty-five samples were analysed. Biopsy samples were classified in five groups depending on their morphological differentiation: solid, cystic, adenoid, keratotic and metatypical. Two parameters were studied: proliferative index and DNA index. Statistical analysis revealed that BCC with keratinizing or metatypical differentiation had a greater amount of aneuploidy when compared with BCC without particular differentiation (solid and cystic) or aneuploid differentiation (p < 0.001). The proliferative indices were significantly different in the four groups. DNA FCM of BCC confirms the value of morphological classifications and gives another example of aneuploidy in a tumour with low aggressiveness.

Aneuploidy↗

[Langerhans-cell histiocytosis in twin sisters].

BACKGROUND--Histiocytosis of Langerhans cells includes a range of clinical manifestations that have been described as bone eosinophilic granuloma, Hand-Schüller-Christian syndrome, Letterer-Siwe syndrome and Hashimoto-Pritzker histiocytosis. These syndromes represent a spectrum of severity and prognosis of the same underlying disorder which is usually sporadic. It has occurred in monozygotic twins and in a familial pattern. This report describes monozygotic twins who developed the disease a few months after their father was found to be suffering from Hodgkin's disease. Case n. 1.--A 4 month-old girl was admitted because of fever, disseminated lymphadenopathy and hepatomegaly. She also had interstitial pneumonia. Infiltrating abnormal histiocytes were demonstrated in lymph node and bone marrow biopsies. X-rays showed lytic areas in the skull. Serology for EBV infection was negative. Special studies with immune markers of lymph node histiocytes confirmed the diagnosis of Langerhans cell histiocytosis, and more precisely, Letterer-Siwe syndrome. The patient was given prednisolone followed by vinblastine without success. She was given etoposide 11 weeks later, which induced remission. This treatment was replaced by vinblastine when the patient was aged 2 years 9 months. Case n. 2.--The monozygotic twin of the case n. 1 was also admitted at 4 months of age because of the same manifestations. Laboratory findings were identical to those of her sister, as was her response to the same drugs. The father was diagnosed as having Hodgkin's disease 3 months before the first manifestation of Langerhans cell histiocytosis in his daughters. His maternal uncle had also been treated for Hodgkin's disease. Immunologic studies of the twin were negative. CONCLUSION--These cases of Langerhans cell histiocytosis in monozygotic twins have no apparent relationship with the Hodgkin's disease of their father. Etoposide seems to be useful for treating such severe forms of the disease.

Diseases in Twins↗

Brunsting-Perry cicatricial bullous pemphigoid: a clinical variant of localized acquired epidermolysis bullosa?

An 84-year-old man who had the typical clinical features of Brunsting-Perry cicatricial pemphigoid is described. Direct immunofluorescence microscopic examination of salt-split skin revealed linear deposits of IgG and C3 on the floor of the artificial bullae. Direct immunoelectron microscopic examination of peribullous skin showed dermal cleavage level below the lamina densa and granular deposits of IgG and C3 attached to and below the lamina densa in a pattern identical to epidermolysis bullosa acquisita. These findings suggest that Brunsting-Perry cicatricial pemphigoid may represent a clinical variant of epidermolysis bullosa acquisita.

Aged↗

New electron microscopic findings in a case of multicentric reticulohistiocytosis. Long spacing collagen inclusions.

The case of a 68-year-old woman with polyarthropathy and skin nodules is presented. Histologic findings of histiocytes with ground-glass cytoplasm favored the diagnosis of multicentric reticulohistiocytosis. In this article we report new electron microscopic findings of intra- and extracytoplasmic long spacing collagen (type VI). Type VI collagen inclusions are usually found in lymphohistiocytic neoplasms. We believe our findings support the concept of a proliferative rather than inflammatory etiology for multicentric reticulohistiocytosis. It may also give support for the argument of production of collagen by the histiocytic cells of multicentric reticulohistiocytosis.

Aged↗

Immunofluorescence and immunoelectron microscopy analyses of a human monoclonal anti-epithelial cell surface antibody that recognizes a 185-kD polypeptide: a component of the paraneoplastic pemphigus antigen complex?

We recently reported the production of a human monoclonal antibody (MoAb) derived from a patient with pemphigus vulgaris (PV) that binds to the keratinocyte membrane and reacts with a 185-kD polypeptide by immunoblot analysis. We have since examined the tissue specificity of that MoAb, F12. By indirect immunofluorescence (IIF), F12 stained both the cell membrane and the basement membrane zone of stratified squamous epithelia. Moreover, MoAb F12 stained other epithelial tissues, such as urinary bladder, small bowel, thymus, and liver, and non-epithelial tissues, such as myocardium. Indirect immunoelectron microscopy (IIEM) analysis showed that MoAb F12 bound to a component common to desmosomal and hemidesmosomal plaques and to zona adherens-type junctions between hepatocytes and bile duct cells. Inhibition experiments were then performed with sera from patients with pemphigus vulgaris, pemphigus foliaceus, paraneoplastic pemphigus, or bullous pemphigoid. Three sera blocked F12 reactivity; two were from paraneoplastic pemphigus patients and the other was from the pemphigus vulgaris patient whose peripheral blood lymphocytes were used to make F12. All these sera recognized a 185-kD band that co-migrated with the polypeptide labeled by MoAb F12 on immunoblots. In addition, the IIF and IIEM staining patterns of MoAb F12 were similar to those observed with sera from two patients with paraneoplastic pemphigus. These observations suggest a relationship between MoAb F12 and the autoimmune response characterizing paraneoplastic pemphigus patients' sera.

Antibodies, Monoclonal↗

Subcutaneous changes in dermatomyositis.

We report the case of a 42-year-old woman with concomitant panniculitis and dermatomyositis. Painful, indurated lesions on the buttocks, thighs, arms, abdomen and breasts were associated with proximal muscle weakness. Skin biopsy revealed lobular panniculitis, and vacuolar degeneration of epidermal basal cells. Direct immunofluorescence was negative. Serum muscle enzyme (creatinine-phosphokinase) levels were elevated, and electromyography demonstrated a myositic process. Muscle biopsy showed an inflammatory myositis. These results were consistent with dermatomyositis associated with panniculitis. Only five cases of this association have been reported previously. The relationship between these two conditions is discussed.

Adult↗

Local and systemic activation of the whole complement cascade in human leukocytoclastic cutaneous vasculitis; C3d,g and terminal complement complex as sensitive markers.

We have studied complement activation both in plasma samples and in lesional skin from patients with leukocytoclastic cutaneous vasculitis (LCV). Enzyme immunoassay (EIA) quantification of the complement activation markers, C3d,g and the terminal complement complex (TCC) in plasma, showed that their levels were significantly increased in 66% and 55% of the patients, respectively (n = 29) compared with healthy controls, whereas the standard measurements of C3, factor B, C1q, C4 and C2 were generally within normal range. Elevations of C3d,g and TCC levels in plasma were significantly correlated. Importantly, a significant correlation was found between the severity of the vasculitis and both C3d,g and TCC plasma levels. Immunofluorescence studies of skin biopsy specimens demonstrated simultaneous presence of perivascular dermal deposits of C3d,g and TCC in lesional skin from 96% and 80% respectively of the patients (n = 25). There was a significant correlation between the intensity of the deposits of both markers. Clusterin, a TCC inhibitory protein, was always found at the same sites of perivascular TCC deposits. Immunofluorescence studies at the epidermal basement membrane zone (BMZ) revealed in each case deposits of C3d,g which were accompanied by TCC deposits in 52% of the biopsy specimens. These data demonstrate that there is a local and systemic activation of the whole complement cascade in human LCV. The presence of both C3d,g and clusterin-associated TCC perivascular deposits suggests an intervention of a regulatory mechanism of local complement activation in LCV. Finally, measurement of plasma C3d,g and TCC appears to be a sensitive indicator of systemic complement activation and disease severity in LCV.

Aged↗

[Necrotizing sialometaplasia: an ignored disease].

Necrotizing sialometaplasia is an ignored pathology. Differential diagnosis has to be made with a neoplasm. The presented case is characterized by an important bone defect, which, to our knowledge, has never been reported.

Biopsy↗

[Metastatic basal cell carcinoma].

A case of basal cell carcinoma in a 17-year old male patient complicated, 5 years later, by inguinal and pulmonary metastases is reported. This clinical case raises two problems: the reality of the entity and the long-term follow-up of this type of tumours.

Adolescent↗