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E Dippel

Publications and source records attributed to E Dippel.

At least 37 records · Page 2Linked to original sources
PubMed1999

Clonal T-cell receptor gamma-chain gene rearrangement by PCR-based GeneScan analysis in advanced cutaneous T-cell lymphoma: a critical evaluation.

Detection of clonal T-cell receptor gamma (TCRgamma)-chain gene rearrangement is a promising approach to distinguish between cutaneous T-cell lymphomas (CTCLs) and reactive T-cell infiltrates. Despite the improved sensitivity by using the polymerase chain reaction (PCR) rather than Southern blot analysis, monoclonality could be demonstrated in only 53-90 per cent of CTCL biopsies in recent studies. In the present study, formalin-fixed, paraffin-embedded specimens of 21 selected patients with clear-cut advanced-stage CTCL were analysed using a semi-nested TCRgamma PCR with newly developed consensus primer pairs. Detection of PCR products was done by GeneScan analysis (GSA); this technique is advantageous due to its sensitivity and accuracy in the detection and size determination of PCR products and it is easier to interpret than direct read-outs from TGGE or DGGE gels. In serial dilution experiments, TCRgamma-PCR-GSA allowed the detection of clonal, rearranged T-cells with a high in vitro sensitivity against a polyclonal background (1-6 per cent). Despite the selection of clear-cut, advanced-stage CTCL cases, however, dominant clonal TCRgamma-chain gene rearrangement was found in only 16 of the 21 patients analysed, indicating an overall clinical sensitivity of 76 per cent. Specificity was evaluated using biopsy specimens from 21 control patients suffering from long-standing psoriasis (n=13) and eczema (n=8). Surprisingly, GeneScan profiles showing apparently single dominant peaks were detected in 14 per cent of these skin lesions, but these profiles turned out to be pseudo-monoclonal by repeated determinations. In conclusion, TCRgamma-PCR-GSA does not suffice reliably to exclude malignancy, due to its limited clinical sensitivity, but with precautions taken to detect pseudo-monoclonality and to secure specificity, TCRgamma-PCR-GSA is a valuable instrument in the diagnosis of CTCL.

Diagnosis, Differential↗
PubMed1999

[Manifestation of epidermolysis bullosa acquisita (EBA) in the ENT area].

Epidermolysis bullosa acquisita (EBA) is a rare, chronic, acquired bullous autoimmune dermatosis. It is characterized by the formation of IgG autoantibodies against type VII procollagen of anchoring fibrils with subepidermal formation of bullous lesions and consequent scarring. The epidemiology of this disease and its characteristic clinical findings cannot be completely surveyed at present due to the limited number of available publications. In general, bullous lesions form on the entire integument and can also involve mucosa. The development of scar-related adhesions on the mucosa of the upper airways and esophagus can lead to serious complications that are difficult to treat. We report our experience in managing a 44-year-old male patient in whom the diagnosis of EBA was established in 1993 on the basis of multiple persistent bullous lesions involving the eye, nose, skin and oral, pharyngeal and laryngeal mucosa. After failing previous medical and surgical therapies, interdisciplinary management resulted in the control of active lesions with extracorporal phototherapy and cyclosporin A.

Adult↗
PubMed1999

Follicular mycosis fungoides.

We describe a patient with follicular mycosis fungoides (MF), a rare folliculotropic variant of cutaneous T-cell lymphoma (CTCL). Follicular involvement in CTCL usually presents clinically as alopecia mucinosa associated histologically with follicular mucinosis. Follicular MF differs from alopecia mucinosa/follicular mucinosis associated with MF with regard to its clinical presentation, histology and, presumably, prognosis. Our patient presented with the characteristic findings of follicular MF, i.e. infiltrated plaques showing numerous enlarged, comedo-like follicular infundibula; histology was dominated by exclusive folliculotropism of atypical lymphocytes sometimes forming follicular Pautrier's microabscesses, and by lack of epidermotropism and follicular mucinosis. Despite photochemotherapy and treatment with oral retinoids and interferon alpha, the patient's follicular MF rapidly developed into a progressive CTCL with large tumorous lesions, but responded to electron beam therapy. The course of our patient's disease confirms the notion that follicular MF may be associated with a worse prognosis than classical MF. However, electron beam irradiation induced remission of follicular MF that was maintained by a combination therapy consisting of extracorporeal photopheresis and interferon alfa.

Aged↗
PubMed1999

Unilateral angiolymphoid hyperplasia with eosinophilia involving the left arm and hand.

A case report of recurrent angiolymphoid hyperplasia with eosinophilia (ALHE) in an otherwise healthy 20-year-old female with manifestation of the disease limited to the left arm and hand is presented together with brief evaluation of the literature as well as the features distinguishing ALHE and Kimura's disease. Immunohistochemical investigations support the hypothesis that ALHE represents a reactive inflammatory lesion rather than a benign vascular neoplasm. A viral cause of ALHE (e.g., HHV8 or Epstein-Barr virus (EBV)) could not be demonstrated. The recurrent nature of the disease is shown by this case, which also demonstrates the need for frequent medical and surgical management.

Adult↗
PubMed1999

Superimposed primary chancre in a patient with Adamantiades-Behçet's disease.

Adamantiades-Behçet's disease was diagnosed in a 42 year old Turkish patient with recurrent oral aphthae, genital ulcerations, papules, and sterile pustules, histologically presenting as cutaneous vasculitis, and intermittent arthritis with joint effusion particularly of the knees. Six months after initial improvement under treatment with colchicine 2 mg/day, a solitary genital ulcer with enlarged inguinal lymph nodes appeared and persisted for 7 weeks despite the continuation of colchicine treatment and the introduction of clindamycin 2 mg/day intravenously. The unusual persistence of the ulcer and the failure of clindamycin therapy led to further differential diagnostic considerations and the identification of primary syphilis. The genital lesion healed 4 weeks after initiation of treatment with tetracycline 2 mg/day by mouth for 15 days.

Adult↗
PubMed1999

Long-term extracorporeal photoimmunotherapy for treatment of chronic cutaneous graft-versus-host disease: observations in four patients.

BACKGROUND: Chronic cutaneous graft-versus-host disease (GvHD) can arise as a late complication after allogeneic bone marrow transplantation. Patients with extensive disease to date require intensive early and long-term immunosuppression; however, treatment is often insufficient. Since the beneficial effects of phototherapy for chronic cutaneous GvHD are well known, extracorporeal photoimmunotherapy (ECP) was also tried with some success for a few single patients with this disease. OBJECTIVE: The long-term effect of ECP was evaluated in 4 patients with therapy-resistant severe chronic cutaneous GvHD after allogeneic bone marrow transplantation. METHODS: Four patients were treated with monthly sessions of ECP over a period of 16-40 months. Disease severity was assessed by a semiquantitative score adapted from the literature including extent of skin area involved, rigidity of the skin, involvement of joints and immunosuppressive drug consumption. RESULTS: In all patients, a favorable response was observed after 6-12 treatment cycles. One patient had a complete response, 2 patients had a partial response, and 1 patient had a minor response after treatment. In 2 patients, immunosuppressive medication started before initiating ECP could be reduced or completely withdrawn under ECP. Peripheral blood lymphocyte immunophenotyping revealed reduction of CD3+ CD4+ T cells in 3 patients and of elevated CD3+ CD8+ and CD57+ CD8+ T cell subsets in 2 patients. CONCLUSION: ECP is effective in treating severe chronic cutaneous GvHD. ECP possibly exerts its effects by reducing the number of CD8+ suppressor/cytotoxic T cells, the presumptive effector cells of GvHD. ECP is well tolerated with essentially no side effects and allows reducing the dosage of immunosuppressive agents.

Adult↗
PubMed1999

Alternative versus classical activation of macrophages.

In parallel to the Th1/Th2 paradigm, antigen-presenting cells (APC) are divided into classically activated APC (dendritic cells/effector macrophages) and alternatively activated APC (IL-4-induced, alternatively activated macrophages/IL-10-induced, immature dendritic cells). Alternatively activated APC share a special molecular repertoire including receptors of innate immunity with broad specificity for foreign antigen and anti-inflammatory cytokines such as IL-1Ra and alternative macrophage activation-associated CC-chemokine-1. Alternatively activated APC mediated tolerance and downregulated inflammation. Abuse of alternatively activated APC in support of infectious susceptibility or tumor immune escape is counteracted by the classical pathway. Thus, classically and alternatively activated APC secure the balance between proinflammatory and anti-inflammatory immune reactions.

Animals↗
PubMed1999

Beyond inflammation: tolerance, immunotherapy and more.

Inflammatory skin diseases such as allergic contact dermatitis, atopic dermatitis, and psoriasis are together the most frequent dermatoses and are of great medical and economic significance. Despite impressive progress in recent years, the immune pathogenesis of these diseases is not yet fully understood. In particular, it remains an open question which anti-inflammatory mechanisms can be activated to prevent development of such abnormal immune reactions in the still healthy organism, and how these anti-inflammatory processes may be enhanced and instrumentalized for treatment of these disease states.

Dermatitis, Allergic Contact↗
PubMed1998

[Zosteriform lichen aureus].

Lichen aureus is a special localized variant of purpura pigmentosa chronica. A patient presented with an unusual striate and segmental or zosteriform distribution of lichen aureus involving the left leg and lower abdominal area. Histology showed the characteristic dense band-like infiltrate consisting mainly of small lymphocytes and numerous hemosiderin-containing macrophages. No underlying infection or medication could be identified as a trigger. The lesions did not respond well to topical corticosteroid therapy.

Biopsy↗
PubMed1998

[Granulomatosis disciformis Miescher].

We describe granulomatosis disciformis Miescher in a 57-year old female patient with typical clinical manifestation: disc-shaped plaques with slight central sclerosis and lesions resembling necrobiosis lipoidica and granuloma anulare. Histology revealed an infiltrate mainly composed of macrophages and lymphocytes and small foci of necrobiotic collagen and of new collagen formation. We review the literature on granulomatosis disciformis and nosology, concluding--as do most authors--that granulomatosis disciformis is a characteristic variant within the spectrum of cutaneous noninfectious granulomatous disease lying in between generalized granuloma anulare and necrobiosis lipoidica.

Adult↗
PubMed1998

[Exfoliatio areata linguae et mucosae oris: a mucous membrane manifestation of psoriasis pustulosa?].

Lesions of the oral mucosa are frequently described in association with psoriasis, particularly in the pustular type. Controversy surrounds the question whether mucosal lesions can be considered as oral manifestation of psoriasis. Two patients presented with concurrent pustular psoriasis and mucosal lesions with the characteristic picture of geographic tongue. Histopathology of the mucosa showed typical features of psoriasis such as marked acanthosis, clubbing of the rete ridges, focal parakeratosis and neutrophilic infiltrates. There was parallel improvement of the skin and the mucosal lesions with systemic retinoid treatment. On the basis of the histopathological features and the clinical course we favour the hypothesis that geographic tongue is an oral manifestation of pustular psoriasis.

Aged↗
PubMed1998

Induction of human leukaemic mast cell differentiation by fibroblast supernatants, but not by stem cell factor.

In order to explore the potential existence of human mast cell growth factors other than stem cell factor (SCF), we have compared SCF to L-cell fibroblast supernatants (LCS) during in vitro mast cell differentiation, using human leukaemic mast cells (HMC-1 cells) which contain a gain-of-function mutated SCF receptor (c-Kit) as model. At baseline, cells exhibited an immature phenotype, with <25% being metachromatic or chloroacetate esterase, tryptase and FcepsilonRIalpha positive. Intracellular levels of histamine, tryptase, TNF-alpha and chymase were low, whereas 83% of cells were c-Kit positive. During a 10 day culture with 30% LCS, a significant, time-dependent increase of all mast cell markers, except for chymase and c-Kit, was observed at the protein and for tryptase and FcepsilonRIalpha also at the mRNA level. Cytoplasmatic granulation and stimulated histamine and leukotriene C4 release were increased as well. In contrast to LCS, rhSCF induced none of these changes in HMC-1 cells. On Sephadex G100 fractionation of LCS, HMC-1 cells increased tryptase activity with fractions between 40 and 60, and below 10 kDa, away from the SCF peak. These data show that HMC-1 cells fail to differentiate in response to SCF and that in addition to SCF, LCS contains other human mast cell growth factors.

Animals↗
PubMed1998

Extracorporeal photopheresis of cutaneous T-cell lymphoma is associated with reduction of peripheral CD4+ T lymphocytes.

BACKGROUND: Extracorporeal photopheresis (ECP) has been successfully introduced for the treatment of cutaneous T-cell lymphoma; however, the mechanism(s) of its action is (are) unknown. OBJECTIVE: To investigate the effects of ECP on the immune system of patients with cutaneous T-cell lymphoma. METHODS: Clinical response and changes of lymphocyte subpopulations in 20 patients with cutaneous T-cell lymphoma under ECP monotherapy or combined regimens were evaluated and compared after 3, 6 and 12 ECP cycles. RESULTS: Thirteen of 20 patients showed a > or = 50% reduction of skin lesions after 6-12 ECP cycles. An overall T-lymphocyte reduction was assessed with a balanced CD4+ T-helper and CD8+ T-suppressor cell decrease in responders. In contrast, there was a trend of CD4+ T-helper cell increase in nonresponders which could result from the failure of treatment to control the natural course of the disease. The CD4+/CD8+ ratios were 1.6 at baseline and 1.4 after 12 cycles in responders, while they increased from 1.7 to 4.1 in nonresponders, respectively (p = 0.47). In addition, there was an overall decrease in the CD57+/CD8+ T-cell subpopulation mostly due to a reduction in the responder group. CONCLUSION: The marked differences detected in certain T-cell subpopulations suggest an effect of ECP on peripheral T lymphocytes and, especially, on CD4+ cells.

Adult↗
PubMed1998

Management of severe scleroderma with long-term extracorporeal photopheresis.

BACKGROUND: The management of systemic sclerosis remains unsatisfactory. Thus far, the action of extracorporeal photopheresis (ECP) in severe systemic scleroderma has been evaluated in short-term studies, and only limited experience has been obtained with long-term application. OBJECTIVE: The aim of the present study was to evaluate prospectively the long-term effect of ECP in a group of 16 patients suffering from severe scleroderma, showing visceral involvement and progressive clinical course. METHODS: Fourteen patients with systemic scleroderma involving several organs, 1 with CREST syndrome and another with scleroderma-myositis overlap syndrome were treated with ECP over a period of 6-45 months. In 3 cases, gamma-IFN was additionally administered. Skin and visceral involvement were assessed by evaluating a series of clinical criteria and results from laboratory, imaging and functional tests. RESULTS: Overall, clear improvement was encountered in 6 patients, mixed response in 2, stable disease in 3 and continuing progressive course in 5 patients. Four out of 6 patients with improvement were treated with ECP early after onset of scleroderma (< or = 2 years), whereas all patients with a progressive course under ECP had had scleroderma for longer than 2 years. Immunosuppressive drugs previously administered could be reduced or fully withdrawn under ECP treatment in 5 patients, but additional oral medication was introduced in 4 patients due to disease progression. Addition of gamma-IFN to ECP did not reveal further benefit. No side-effects were recorded under ECP treatment. CONCLUSIONS: Based on this observation, we believe that long-term ECP represents an effective treatment modality in severe scleroderma particularly when started early, with stabilization of the disease course and partial remission of the cutaneous findings, whereas visceral involvement, if present, may rarely improve.

Female↗