INTRODUCTION: Multinucleate cell angiohistiocytoma is a rare entity. We report two unusual cases of this lesion. CASE REPORT: A 39 year-old man had presented since two years an arciform papular eruption of the forehead. A 40 year-old woman had a disseminated maculo-papular eruption since three years. Histological examination of skin samples of the two patients showed a dermal small vessel proliferation, associated with multinucleate cells, and confirmed the diagnosis of multinucleate cell angiohistiocytoma. DISCUSSION: Our cases, typical histologically, have unusual clinical presentation (first case observed in a man, and the second in a disseminated form). Multinucleate cell angiohistiocytoma is a rare benign vascular proliferation individualized in 1985. Clinically, the lesion consists in small circumscribed papules. Acral sites and notably of the dorsum of the hands are the more frequent topography. It generally occurs in women aged over 50 years. Histologically, there is a dermal proliferation of capillaries and venules, and a fibrosis associated with the interstitial mononucleate cells and multinucleate cells.

Adult↗

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[Value of biopsy of accessory salivary glands for the diagnosis of amyloidosis].

There are few reports of amyloidosis diagnosed by deliberate biopsy of accessory salivary glands. Usually, a biopsy performed for dry mouth syndrome reveals an unsuspected amyloidosis. We report the case of 2 patients with lambda-type light chain monoclonal gammapathy complicated by generalized amyloidosis and in whom biopsy of the accessory salivary glands showed signs of amyloidosis. In the first patient accessory salivary gland biopsy was performed because these glands were enlarged, and the monoclonal dysglobulinaemia was subsequently diagnosed by serum immunoelectrophoresis. In the second patient with nephrotic syndrome, renal biopsy could not be carried out owing to the presence of a renal malformation; amyloidosis was confirmed by periumbilical fat aspiration, and a systematic biopsy of accessory salivary glands also showed evidence of amyloidosis. Biopsy of accessory salivary glands seems to be a particularly simple and safe method to detect generalized amyloidosis in patients with chronic inflammatory disease or monoclonal dysglobulinaemia.

Aged↗

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[Double heart valve replacement disclosing antiphospholipid syndrome].

In patients with systemic lupus erythematosus (SLE) heart valve lesions are usually discovered at echocardiography; their haemodynamic repercussions are uncommon, and valve replacement is exceptional. We report the case of a woman who had undergone aortic and mitral valve replacement before antiphospholipid antibodies were found associated with 4 ARA criteria of SLE. Histopathological examination confirmed the diagnosis of Libman-Sachs specific endocarditis. The presence of antiphospholipid antibodies leads to a discussion of their role in the physiopathology of the heart valve lesions and vascular accidents that occurred in this patient. The overlap observed between the diagnostic criteria of SLE and those of primary antiphospholipid syndrome is discussed. Heart valve lesions may be one of the modes of access to the antiphospholipid syndrome.

Adult↗

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Characterization of the secreted form of endothelial-cell-specific molecule 1 by specific monoclonal antibodies.

Endothelial-cell-specific molecule 1 (ESM-1) is a recently identified endothelial cell molecule. As ESM-1 mRNA is preferentially expressed in human lung and kidney tissues, and as ESM-1 mRNA expression is regulated by inflammatory cytokines, ESM-1 is thought to play a role in the vascular contribution to organ-specific inflammation. In order to define its behavior, mouse anti-ESM-1 monoclonal antibodies were developed, and three distinct epitopes were mapped, which allowed development of a specific ELISA assay, immunohistological staining and immunoblot analysis. Here, we demonstrate that ESM-1 is present in cell lysates of human endothelial cells (human umbilical vein endothelial cells) with an apparent molecular weight of 20 kD. In contrast, the secreted form of ESM-1 is shifted to an apparent molecular weight of 50 kD, indicating that the secreted form of ESM-1 is posttranslationally modified. By ELISA, we show that the secretion of ESM-1 is significantly enhanced in the presence of TNFalpha. In contrast, the spontaneous as well as TNFalpha-induced secretion of ESM-1 is strongly inhibited by IFNgamma. Moreover, ESM-1 was detected in the serum of healthy subjects at an average concentration of 1.08 ng/ml, and we demonstrated that the serum level of ESM-1 is dramatically increased in patients presenting a septic shock. Analysis of ESM-1 expression in normal human tissues by immunohistochemistry showed that ESM-1 is localized in the vascular network, but also in the bronchial and renal epithelia. Our results demonstrate that ESM-1 is mainly expressed in the vascular endothelium both in vitro and in vivo, but also by different epithelia. ESM-1 may represent a new marker of endothelial cell activation, and may have a functional role in endothelium-dependent pathological disorders.

Adult↗

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Airway-like inflammation of minor salivary glands in bronchial asthma.

Bronchial asthma is characterized by a chronic inflammation with accumulation of eosinophils, mast cells and activated T lymphocytes that release a large panel of cytokines. As the mucosal immune system comprises a series of specialized lymphoid tissues with a well-identified lymphocyte traffic between different compartments, we initiated a study to evaluate the histological abnormalities of minor salivary glands (MSGs) in patients with bronchial asthma. 58 patients were studied (29 with allergic asthma, 29 with nonallergic asthma) and compared to 15 healthy controls and 15 patients with chronic obstructive pulmonary disease (COPD). MSGs were normal in all controls except one and in 14/15 COPD patients. 43/58 asthmatics (74%) exhibited MSG abnormalities with T lymphocyte infiltration (57%), mast cell infiltration (64%), basement membrane thickening (64%) and endothelial cell changes (26%). Histological abnormalities were predominantly observed in nonallergic asthmatics. We propose that activated T lymphocytes, present in the bronchial mucosal lymphoid tissue in chronic asthma might migrate, colonize other glandular and mucosal sites, and so trigger at a distance, an airway-like inflammation.

Adolescent↗

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Detection of the tax gene of HTLV-I in labial salivary glands from patients with Sjögren's syndrome and other diseases of the oral cavity.

OBJECTIVE: To confirm a possible association between Sjögren's syndrome (SS) and the tax gene of human T lymphotropic virus type I (HTLV-I). METHODS: We studied by PCR labial salivary glands (LSG) from 50 patients with definite SS and from 58 controls including 32 patients with LSG involved by other inflammatory processes and 26 normal LSG. Antibodies to HTLV-I and antibodies to the Tax protein were searched for in serum. RESULTS: We detected the tax gene of HTLV-I in LSG from 15/50 (30%) of patients with SS but also in specimens from 9/32 (28%) patients with LSG involved by other inflammatory processes (3/9 graft-versus-host disease, 5/19 extra-vasated cysts, 1/4 sarcoidosis) and from only 1/26 (4%) normal LSG. A 652 bp region, sequenced in 2 SS patients, was 98-98.5% homologous to the canonic sequence of tax HTLV-I. The HTLV-I gag, pol and env genes were never detected. The serum of the SS patients did not contain antibodies to HTLV-I. However, anti-Tax antibodies were detected in the serum of 18/25 (72%) SS patients, 10/10 (100%) patients positive for tax DNA in their LSG and 8/15 (53%) patients negative for tax DNA in their LSG. CONCLUSION: Our observations raise the possibility that a very low number of copies of the tax gene may be harbored innocuously in cells within the oral cavity in some healthy individuals, but that this gene may play a role as a co-factor in the development of SS or other diseases of oral cavity.

Adult↗

PubMedDate not supplied

An unusual cause of vascular purpura: recurrent cutaneous eosinophilic necrotizing vasculitis.

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Aged↗

PubMedDate not supplied

Tumor necrosis factor-alpha and receptors for it in labial salivary glands in Sjögren's syndrome.

OBJECTIVE: Modulation of TNF-alpha by neutralizing antibodies, soluble receptors and TNFR: Fc fusion proteins are being developed for the therapeutic modulation of immune inflammation. It is becoming increasingly important to understand the state and involvement of the TNF-alpha/TNFR system in various rheumatic diseases. Tumor necrosis factor-alpha (TNF-alpha) affects its target cells through binding to two different receptors, TNFR-p55 and TNFR-p75. Mitogenic, cytostatic and cytotoxic effects of TNF-alpha on various cells have been reported. In Sjögren's syndrome (SS) focal sialadenitis leads to salivary gland destruction and loss of function. Although TNF-alpha is one possible mediator in these processes, nothing is known about the spatial distribution of TNF-alpha in relation to its receptors/target cells in salivary gland tissue. METHODS: Labial salivary glands (LSG) were obtained from 16 SS patients and 13 healthy controls and stained using the immunohistochemical peroxidase-anti-peroxidase (PAP) method for TNF-alpha, TNFR-p55 and TNFR-p75. RESULTS: TNF-alpha, TNFR-p55 and TNFR-p75 staining was absent, weak or relatively inextensive in controls compared to SS patients. Infiltrating mononuclear inflammatory cells in SS patients displayed moderate to strong TNF-alpha and TNFR expression. In addition, resident vascular endothelial cells, ductal epithelial cells and fibroblasts co-expressed TNF-alpha and TNFR. In contrast, acinar end piece cells did not express TNF-alpha or TNFR-p75 although TNFR-p55 was expressed. CONCLUSION: The interrelated localization of TNF receptors and their ligand TNF-alpha in inflammatory and in endothelial cells suggests a proinflammatory role of TNF-alpha in SS. The expression of TNF-alpha and its receptors in fibroblasts and ductal cells may contribute to ductal hyperplasia and glandular fibrosis. However, in contrast to expectations, the cellular localization of the TNF-alpha/TNRF system argues against its role in acinar cell atrophy.

Antigens, CD↗