Medium-dose ultraviolet A1 phototherapy in transient acantholytic dermatosis (Grover's disease).
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Acantholysis↗
Biomedical subjects
Frank Breuckmann
Publications and source records attributed to Frank Breuckmann.
Fibromatosis of the hand associated with EMO syndrome: a case report.
BACKGROUND: EMO syndrome, defined as a triad including exophthalmus, pretibial myxedema and osteoarthropathia, is a rare condition in patients suffering from hyperthyreosis. CASE PRESENTATION: We here describe an interesting case of EMO syndrome associated with unilateral fibromatosis of the hand and an initial stage of generalized myxedema of the skin. To our knowledge a similar case has not yet been described in literature though reports about associated fibromatosis, e.g. located retroperitoneally, already exist. Familiar explanations include its initiation by autoimmune processes or aberrant T-cell cytokine stimulation leading to an overwhelming production of glycosaminoglycans. CONCLUSION: Interpreting our case in context with previous reports we conclude that associated fibromatosis induced by autoimmune processes may affect a variety of different localizations and therefore requires careful monitoring. A therapeutical attempt by using UVA1 irridation for pretibial myxedema remained without a satisfying regression.
Exophthalmos↗
Modulation of endothelial dysfunction and apoptosis: UVA1-mediated skin improvement in systemic sclerosis.
UVA1-mediated effects regarding vascular dysregulation as a primary pathogenetic factor of systemic sclerosis skin lesions have so far not been investigated. Pre- and posttherapy skin biopsies of four patients were evaluated immunohistochemically for angiostatic, angiogenic and angioapoptotic features. Immunohistochemistry revealed a partial pretherapy loss of endothelial CD31 and CD34 expression accompanied by a posttherapy increase of CD34(+) cells. Simultaneously, VEGF and M30 CytoDEATH immunolabeling demonstrated UVA1-induced neovascularization and decreased endothelial apoptosis. Our results suggest that UVA1 irradiation exerts its positive effects by a modulation of endothelial regulation/transformation beside the proposed induction of T cell apoptosis and collagenases.
Antibodies, Monoclonal↗
Immunohistochemical investigations and introduction of new therapeutic strategies in scleromyxoedema: case report.
BACKGROUND: Scleromyxoedema is a rare chronic skin disease of obscure origin, which may often be associated with severe internal co-morbidity. Even though different casuistic treatment modalities have been described, to date, curing still seems to be impossible. CASE PRESENTATION: We report a 44-year-old Caucasian female presenting with remarkable circumscribed, erythematous to skin-coloured, indurated skin eruptions at the forehead, arms, shoulders, legs and the gluteal region. Routine histology and Alcian blue labelling confirmed a massive deposition of acid mucopolysaccharides. Immunohistochemical investigations revealed proliferating fibroblasts and a discrete lymphocytic infiltration as well as increased dermal expression of MIB-1+ and anti-mastcell-tryptase+ cells. Bone marrow biopsies confirmed a monoclonal gammopathy of undetermined significance without morphological characteristics of plasmocytoma; immunofixation unveiled the presence of IgG-kappa paraproteins. CONCLUSIONS: Taking all data into account, our patient exhibited a complex form of lichen mxyoedematosus, which could most likely be linked a variant of scleromyxoedema. Experimental treatment with methotrexate resulted in a stabilisation of clinical symptoms but no improvement after five months of therapy. A subsequent therapeutic attempt by the use of medium-dose ultraviolet A1 cold-light photomonotherapy led to a further stabilisation of clinical symptoms, but could not induce a sustained amelioration of skin condition.
Adult↗
UVA/UVA1 phototherapy and PUVA photochemotherapy in connective tissue diseases and related disorders: a research based review.
BACKGROUND: Broad-band UVA, long-wave UVA1 and PUVA treatment have been described as an alternative/adjunct therapeutic option in a number of inflammatory and malignant skin diseases. Nevertheless, controlled studies investigating the efficacy of UVA irradiation in connective tissue diseases and related disorders are rare. METHODS: Searching the PubMed database the current article systematically reviews established and innovative therapeutic approaches of broad-band UVA irradiation, UVA1 phototherapy and PUVA photochemotherapy in a variety of different connective tissue disorders. RESULTS: Potential pathways include immunomodulation of inflammation, induction of collagenases and initiation of apoptosis. Even though holding the risk of carcinogenesis, photoaging or UV-induced exacerbation, UVA phototherapy seems to exhibit a tolerable risk/benefit ratio at least in systemic sclerosis, localized scleroderma, extragenital lichen sclerosus et atrophicus, sclerodermoid graft-versus-host disease, lupus erythematosus and a number of sclerotic rarities. CONCLUSIONS: Based on the data retrieved from the literature, therapeutic UVA exposure seems to be effective in connective tissue diseases and related disorders. However, more controlled investigations are needed in order to establish a clear-cut catalogue of indications.
Adult↗
UVA1-induced decrease in dermal neuron-specific enolase (NSE) in acrosclerosis.
Besides its role in small-cell carcinoma of the lung, elevated serum levels of neuron-specific enolase (NSE) have recently been reported to be associated with autoimmune rheumatic disorders such as systemic sclerosis. Serum NSE seems to correlate with disease activity as well as Rodnan skin score. The aim of the study was to assess the neuromodulatory effects of conventional UVA1 phototherapy on acrosclerosis as an additional mechanism besides an assumed T cell apoptosis, collagenase induction and angiogenesis. Punch skin biopsies of acrosclerotic skin lesions taken before and after treatment from four patients were evaluated immunohistochemically for the presence of NSE, S100 and neurofilament. Immunolabeling revealed a UVA-induced decrease in dermal NSE expression. In contrast, no alteration in neurofilament+ cells could be detected. In line with the findings of a previous investigation, a high number of S100+ cells were detected in most specimens. We demonstrated a UVA1-induced reduction in dermal NSE levels correlating with a softening of former sclerotic lesions. Even though the origin and the functional mechanisms remain obscure, NSE might be relevant directly within sclerotic skin lesions and may possibly be used as a diagnostic marker at least in SSc-associated acrosclerotic skin.
Humans↗
Low-dose UVA1 phototherapy in systemic sclerosis: effects on acrosclerosis.
BACKGROUND: Increased collagen synthesis, vascular damage, and T-lymphocytic infiltration contribute to the development of systemic sclerosis. Preliminary studies revealed the effectiveness of low-dose UVA1 phototherapy in acrosclerosis. OBJECTIVE: We sought to confirm data of a pilot study revealing the efficacy of low-dose UVA1 irradiation in acrosclerosis in a larger number of patients. METHODS: Symptoms of 18 patients receiving low-dose UVA1 phototherapy were evaluated clinically and biometrically in an open, nonrandomized study. A number of pretherapeutic and posttherapeutic biopsy specimens were tested immunohistochemically for matrix-metalloproteinase-1. RESULTS: UVA1 irradiation led to softening of former stiffness reflected by a significant decrease of the hand score, increase of total skin distension, and reduction of skin thickness. Posttherapeutically, matrix-metalloproteinase-1 immunolabeling revealed a significant dermal elevation of collagenase. CONCLUSION: Low-dose UVA1 phototherapy is a capable treatment option for acrosclerosis. Its beneficial effect may be mediated by the induction of collagenases and a reduction of collagen deposition and cellular infiltration.
Adult↗
Pimecrolimus 1% cream for cutaneous lupus erythematosus.
Topical treatment of cutaneous lupus erythematosus usually includes potent glucocorticosteroids. However, prolonged use causes adverse side effects including skin atrophy as the foremost concern. In contrast to glucocorticosteroids, the anti-inflammatory and immunosuppressive macrolactam pimecrolimus has no atrophogenic potential. Affected areas of 11 patients with different forms of lupus erythematosus were treated with pimecrolimus 1% cream under semiocclusive conditions twice daily for 3 weeks. Skin involvement before and after therapy was assessed by means of a clinical score. In all patients, significant regression of skin lesions was observed after therapy (P <.001). This was an open and uncontrolled study on a limited number of cases. We suggest that pimecrolimus 1% cream could be an efficacious and safe treatment option for cutaneous lupus erythematosus.
Administration, Cutaneous↗
Acquired perforating dermatosis: a report on 4 cases associated with scabies infection.
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Aged↗
Pulsed intravenous immunoglobulin therapy in livedoid vasculitis: an open trial evaluating 9 consecutive patients.
BACKGROUND: Livedoid vasculitis (LV) usually presents with painful, slowly healing ulcerations of the lower limbs. The precise pathophysiology of this relatively rare disease remains obscure. Therapeutic strategies usually include rheologic, anti-inflammatory, or immunosuppressive agents. However, no continuing benefit has been reported in any of these modalities. Recently, encouraging case reports about the successful use of intravenous immunoglobulin (IVIg) in LV have been published. METHODS: We initiated an open single center trial to investigate the efficacy and safety of IVIg in LV. Nine patients with LV, 7 of whom were refractory to other treatment modalities, were included. Therapy with IVIg at a dose of 0.5 g/kg body weight per day over 2 or 3 consecutive days was performed monthly. Skin involvement before and after therapy was assessed by means of a clinical score. RESULTS: In all patients, significant regression of skin lesions was observed after therapy resulting in a decrease of the clinical score (including differential semiquantitative assessment of erythema, ulceration, and pain) from 6.5 +/- 1.7 at the beginning to 1.3 +/- 1.2 after therapy (P <.001). IVIg was well tolerated and therapy was finished in all patients. CONCLUSION: In all patients clinical evaluation revealed a marked improvement of erythema, pain, and healing of areas of active ulceration. Although this was an open non-controlled study, we propose that IVIg is a promising therapeutic option in LV refractory to other treatment modalities.
Adult↗
Intravenous immune globulin in the treatment of persistent pemphigoid gestationis.
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Adult↗
Histomorphologic classification of recurrent saphenofemoral reflux.
OBJECTIVE: The purpose of this study was to determine the apparent mechanisms of recurrent saphenofemoral junction (SFJ) reflux on the basis of the histologic features of intentionally sampled specimens from repeat operations in groins with symptomatic reflux after SFJ ligation and great saphenous vein (GSV) stripping. PATIENTS AND METHODS: Ninety-one samples obtained at repeat operation in patients with symptomatic recurrent groin reflux were evaluated histologically, including the number of venous lumens, the structure of the venous wall, and the surrounding tissue. All repeat operation specimens were obtained in our Department of Vascular Surgery; the original operations in our patients (age 56 +/- 11 years) had been performed in different centers and private practices 12.1 +/- 8.1 years previously. RESULTS: Single-channel recurrence was observed in 63 groins. In 62 the vein wall structure was complex; and 18 demonstrated venous valves. These vessels could be classified as residual stumps of the GSV. Just one single-channel recurrent vessel lined with endothelium and surrounded by scar tissue was detected, and was judged to be neovascular. Multichannel recurrent vessels were found in 28 groins. Twenty-three of these were thin-walled tortuous vessels embedded in scar tissue, and were interpreted as neovascular; 4 were multiple vessels surrounded by fatty tissue and were classified as venule hypertrophy; and 1 was a lymph node that contained numerous channels, and was attributed to lymphovenous connections. CONCLUSION: Original misidentification of the SFJ, as evidenced by an identifiable GSV stump with appropriate histologic features, characterized 68% (62 of 91) of these symptomatic groins. Neovascularity, marked by multiple irregular channels with an incomplete wall structure, was observed in 26% of the groins (24 of 91), accounting for 94% of the recurrent vessels. The findings at repeat operation, supported by analysis of histologic tissue sections, imply that better supervision and training or case concentration in centers of excellence should be more effective in decreasing the incidence of recurrent SFJ reflux after SFJ ligation and GSV stripping than putative operative maneuvers directed at reducing neovascularization.
Adult↗
Modulation of cathepsin G expression in severe atopic dermatitis following medium-dose UVA1 phototherapy.
BACKGROUND: During the last decade, medium-dose UVA1 phototherapy (50 J/cm2) has achieved great value within the treatment of severe atopic dermatitis (AD). The purpose of our study was to investigate to what extent UVA1 irradiation is able to modulate the status of protease activity by the use of a monoclonal antibody labeling cathepsin G. METHODS: In order to further elucidate the mechanisms by which medium-dose UVA1 irradiation leads to an improvement of skin status in patients with AD, biopsy specimens from 15 patients before and after treatment were analyzed immunohistochemically for proteolytic activation. RESULTS: Compared to lesional skin of patients with AD before UVA1 irradiation, the number of cells positive for cathepsin G within the dermal infiltrate decreased significantly after treatment. The decrease of cathepsin G+ cells was closely linked to a substantial clinical improvement in skin condition. CONCLUSIONS: In summary, our findings demonstrated that medium-dose UVA1 irradiation leads to a modulation of the expression of cathepsin G in the dermal inflammatory infiltrate in patients with severe AD. Cathepsin G may attack laminin, proteoglycans, collagen I and insoluble fibronectin, to provoke proinflammatory events, to degrade the basement membrane, to destroy the tissue inhibitor of metalloproteinases and to increase the endothelial permeability. Therefore, its down-regulation by UVA1 phototherapy may induce the reduction of skin inflammation as well as improvement of the skin condition.
Biopsy↗
Apoptosis of human dermal endothelial cells as a potential side effect following therapeutic administration of UVA1 irradiation: preliminary results.
Apoptosis is a highly selective form of cell suicide with characteristic morphological and biochemical features. UVA1 phototherapy has been introduced into the treatment of many T cell-derived skin diseases. The aim of our pilot study was to assess apoptosis of endothelial cells in relation to time after irradiation with medium-dose UVA1 using four different staining techniques. With in situ nick end labelling (ISEL) and Hoechst 33342 staining we investigated DNA degradation during apoptosis and used M30 CytoDEATH to selectively stain the cytoplasm of apoptotic cells. Additionally, the expression of the tumour suppressor gene p53 was determined. ISEL and Hoechst 33342 revealed only a few positive endothelial cells 3 h after UVA1 irradiation. After 6 h almost all vessels were positively stained. By 12 h after irradiation this peak concentration had lowered again. The first p53-positive endothelial cells were seen 6 h after UVA1 irradiation and reached a maximum at 12 h after irradiation. Fibroblasts of the lower dermis were positively stained after 6 and 12 h. M30-positive endothelial cells were found from 3 to 12 hours after irradiation. ISEL and Hoechst 33342 staining clearly revealed UVA1-induced apoptotic cell elimination predominantly restricted to endothelial cells as a possible side effect of UVA1 irradiation. The induction of apoptosis was specifically verified by M30 immunostaining of early caspase cleavage. Whereas the p53-positive endothelial cells underwent programmed cell death as demonstrated by M30, ISEL and Hoechst 33342, some fibroblasts seemed to accumulate the p53 antibody, but this did not induce apoptotic cascades.
Antibodies, Monoclonal↗