Atypical fibroxanthoma in a renal graft recipient.
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Biomedical subjects
Publications and source records attributed to A Claudy.
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The human dermis contains a heterogeneous network of cells with a dendritic morphology, including factor XIIIa+ dermal dendrocytes and CD34+ dendritic cells located around epidermal adnexae. Whereas dermal dendrocytes have been immunohistochemically studied, CD34+ dermal cells have not yet been well characterized. We studied by simple and double immunolabeling techniques on tissue sections of normal human skin the phenotype of these cells and found them to express vimentin and Te7 but none of the remaining markers sought (factor XIIIa, von Willebrand factor, CD1a, CD3, CD4, CD8, CD14, CD25, CD36, CD45, CD54, CD56, LFA-1, EGF-R, S-100 protein, Mac 387, and muscle-specific actin). Rare CD34+ cells of the interstitial dermis expressed human leukocyte antigen (HLA)-DR antigens, but this was not the case for periadnexal CD34+ cells. These results show that CD34+ dendritic cells of human dermis are mesenchymal cells bearing a unique immunophenotype different from that of (myo)fibroblasts, monocytes-macrophages, Langerhans cells, and factor XIIIa+ dermal dendrocytes. Whereas the involvement of CD34+ cells in some cutaneous tumors is well known, their physiologic role in normal skin remains to be established. On the basis of our results, we speculate that these cells could represent uncommitted mesenchymal cells, unique by virtue of CD34 antigen expression.
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BACKGROUND: Kaposi's sarcoma (KS), is a known complication following kidney transplantation. It has been reported more rarely following liver transplantation. OBJECTIVE: To assess the clinico-epidemiologic data of KS after liver transplantation. METHODS: 150 liver graft recipients were examined; those presenting with KS were studied clinically, histologically and virologically. RESULTS: Three cases of KS were observed. The three patients had been treated with OKT3 antiserum in addition to the standard regimen. The delay of appearance varied from 5 to 36 months. Two patients had a few cutaneous lesions and 1 had more extensive involvement; none of them had visceral localizations. In 2 cases, herpesvirus-like DNA sequences were detected within the lesions. Therapy consisted in decreasing the immunosuppressive treatment, in association with alpha-interferon or vindesine in 2 cases, respectively. All patients were alive after a follow-up of 19-45 months. CONCLUSION: KS seems relatively frequent (2%) and appears within a short delay after liver transplantation; the prognosis may be more favourable than previously reported.
Bombesin-related peptides are expressed in the skin of batrachians and mammals. As gastrin-releasing peptide belongs to this family, we searched for the presence and distribution of gastrin-releasing peptide receptors (GRPr) in the skin of healthy human adults by immunohistochemistry, flow cytometry and electron microscopy. The results indicated that GRPr are expressed on nerves and vessels in the dermis, on eccrine sweat glands, sebaceous glands and erector pili muscle. Within epidermis, staining was localized only on basal and suprabasal layer cells, or in the whole epidermis, according to the samples studied. Interestingly, suprabasal epidermal dendritic cells occasionally showed a strong labelling. Some of these epidermal dendritic cells were identified as Langerhans' cells by immunoelectron microscopy studies. Flow cytometry analysis of crude epidermal cell suspensions resulted in the expression of GRPr on about 43% of the cells. Therefore, we investigated whether human GRPr could modulate Langerhans' cells antigen-presenting functions. For this purpose, we added increasing concentrations of GRP (10(-12) to 10(-5) M) to mixed epidermal cell lymphocyte reactions. Allogeneic T-cell proliferation was not significantly modified when added to GRP-pretreated epidermal cells. In conclusion, we demonstrated the presence of GRPr in human skin, suggesting that GRP may modulate epidermal cell functions but does not modify antigenic presentation.
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The differential diagnosis between Kaposi's sarcoma and the so-called 'pseudo-Kaposi's sarcoma' or acroangiodermatitis of the feet is often fraught with difficulty, not only on clinical but also on histological grounds. The aim of this study was to assess whether immunolabelling for the CD34 antigen, a marker of Kaposi's sarcoma cells, could be of value in the distinction between these two angioproliferative disorders. We comparatively examined 16 biopsy specimens from cases of Kaposi's sarcoma and seven biopsies from patients with pseudo-Kaposi's sarcoma, by a streptavidin-biotin-peroxidase method, using a monoclonal antibody to the CD34 antigen. All cases of Kaposi's sarcoma showed CD34 labelling both on endothelial cells and on the characteristic spindle-shaped, perivascular cells. Biopsies of pseudo-Kaposi's sarcoma showed a strong labelling of endothelial cells of hyperplastic vessels. However, in sharp contrast with Kaposi's sarcoma, a complete absence of perivascular CD34 expression was noted. It seems therefore that immunolabelling for the CD34 antigen appears to be a valuable tool in the differential diagnosis between Kaposi's sarcoma and pseudo-Kaposi's sarcoma.
The sera of 263 patients with bullous pemphigoid (BP) were tested by indirect immunofluorescence (IIF) on salt-split skin (SSS) and immunoblot (IB) assay, in order to assess the diagnostic sensitivity of these techniques. Among the 263 sera tested, 198 sera (75%) contained antibasement membrane zone antibodies demonstrable by IIF reacting to the epidermal (98%) or both the dermal and epidermal sides (2%) of SSS. One hundred and eighty-two of the 263 sera (69%) reacted by IB with BP antigens (Ag), most commonly the BPAg1 (93 cases, 51%), and a complex of BPAg1 and the 180 kDa minor BP antigen (BPAg2) (47 cases, 26%). BPAg2 alone was found in 42 cases (23%). A good correlation was found between the detection of autoantibodies by IIF and labelling of BPAg1 and/or BPAg2 by IB assay, in which 152 of 198 sera with an epidermal pattern in IIF identified a BP antigen. IB analysis of the 65 sera negative by IIF yielded positive results in 30 cases (46%). Thirty-one percent (13 of 42) of sera recognizing by IB BPAg2, were negative by IIF, as compared with 12% (11 of 93) of those recognizing BPAg1 (P < 0.01). Comparing the sensitivity of the two tests, IIF (75%) was found to be more sensitive than IB (69%). Thirty-five of the 263 sera (13%) remained negative by both techniques. It can be concluded from this study that IIF on SSS appears to be a sensitive and reliable assay for screening BP; IB should be performed for the sera that are negative by IIF as it may reveal circulating antibodies, particularly to BPAg2.
Over the past 8 years, we have followed a child born as a harlequin baby, who survived due to treatment with retinoids. His condition evolved clinically towards the erythrodermic form of lamellar ichthyosis (non-bullous congenital ichthyosiform erythroderma, NBCIE). According to ultrastructural and biochemical criteria, our patient originally presented with type II harlequin ichthyosis. Investigations showed an abnormal keratinosome structure and extrusion, a keratin pattern characteristic for epidermal hyperproliferation, and an absence of conversion of profilaggrin to filaggrin. Persisting keratinocyte hyperproliferation, associated with the presence of a dermal infiltrate, is in agreement with the present clinical picture of severe NBCIE. However, abnormal lamellar body production and defective filaggrin processing, which is not one of the diagnostic criteria of NBCIE, persist in the patient's skin. Further studies of the epidermal lipid composition, and of possible mutations of the keratinocyte transglutaminase gene performed on epidermal cell cultures of harlequin ichthyosis, will be necessary before type II harlequin ichthyosis can be accepted as an extremely severe form of NBCIE.
INTRODUCTION: The objective of this study was to evaluate the efficacy of thalidomide in the treatment of chronic erythema multiforme unresponsive to usual treatments. PATIENTS AND METHODS: Twenty-six patients with chronic erythema multiforme were given thalidomide 100 mg/day after other treatments had failed, particularly acyclovir and prednisone. RESULTS: Twenty patients had recurrent erythema multiforme and were given thalidomide at the beginning of an episode. The duration of the episodes was reduced by 11 days on the average. Six of the patients had subintrant erythema multiforme and were given continuous treatment. Lesions disappeared within 5 to 8 days and remission was maintained with low dose-treatment. DISCUSSION: The spectacular results obtained here should be verified in a controlled study.
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Neuropeptides are neurotransmitters and neurohormones that play a role in various cutaneous functions. Keratinocytes and dermal endothelial cells are able to synthesize neuropeptides which are transported by nerve fibers or immune cells. Specific receptors for neuropeptides are also present on cutaneous cells. Neuropeptides intervene as neurogenic modulators of inflammatory reactions and therefore participate in the pathogenesis of skin diseases. An increasing body of evidence supports the setting up of clinical trials using topically neuropeptide agonists and/or antagonists in the treatment of chronic inflammatory skin disorders such as post-herpetic neuralgia, prurigo nodularis, localized pruritus, psoriasis, atopic dermatitis, contact dermatitis, cold urticaria, nostalgia paresthetica, diabetic neuropathy, Raynaud's phenomenon. In the near future, neuropeptides will represent a new approach to skin therapy.
INTRODUCTION: Primary intestinal lymphangiectasias are often associated with lymphoedema. OBSERVATION: The diagnosis was performed at 4 months when Maxime presented with lymphoedema, diarrhea, hypoprotidemia and hypolipemia. Duodenum biopsies revealed intestinal lymphangiectasias. An hyperprotidic and low fat diet, medium chain triglyceride-supplemented and an elastic contention allowed a decline of the oedemas. DISCUSSION: We report one case of Waldman's disease. It shows very well the typical circumstances of diagnosis in this disease and the two types of oedema (lymphoedema and hypoprotidic oedema).
INTRODUCTION: Acrokeratoelastoidosis was first described by O. Costa in 1953. We report a new case with a unilateral localization. CASE REPORT: A 27-year-old woman had atypical acrokeratoelastoidosis lesions of the right hand and foot since adolescence. Diagnosis was confirmed by histology. DISCUSSION: Several cases of acrokeratoelastoidosis have been reported in the literature, but this case is novel because of the unilateral localization. We recall the characteristic features of this disease and emphasize the heterogeneous nature of the manifestations. Differential diagnosis is discussed for this disease which is one of the group of acral lenticular keratoses.
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