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  • 1
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Science Ltd
    Journal of the European Academy of Dermatology and Venereology 17 (2003), S. 0 
    ISSN: 1468-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Science Ltd
    Journal of the European Academy of Dermatology and Venereology 15 (2001), S. 0 
    ISSN: 1468-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Linear IgA dermatosis presented with erythema annulare centrifugum lesions in three elderly women. Search for underlying malignancy revealed low-grade B-cell lymphoma in one case. In addition to subepidermal blistering, histology showed a typical mixed infiltrate of granulocytes and eosinophils and, occasionally, papillar microabsesses in one case. In the two other subjects, characteristic subepidermal lining with granulocytes was observed. Immunofluorescence studies confirmed the diagnosis, while autoantibodies characteristic for dermatitis herpetiformis were absent. To our knowledge this is the second report of adult linear IgA dermatosis in association with erythema annulare centrifugum lesions. Our observations concord with several other reports of figurate erythema associated with autoimmune blistering disease and other immune disorders. Common antibody-related immunological mechanisms indicate that the two distinct clinical pictures are probably stages of the same pathogenic entity.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Journal of the European Academy of Dermatology and Venereology 12 (1999), S. 0 
    ISSN: 1468-3083
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: In order to investigate possible alterations in c-kit protein expression on epidermal melanocytes in different hypopigmentary disorders, we have examined skin specimens from one patient with piebaldism, one patient with naevus depigmentosus, and five patients with vitiligo. Cryosections were examined by immunohistochemistry using monoclonal antibodies against the c-kit protein (YB5.B8) and melanosomes (TA99).In piebaldism, hypomelanotic epidermis contained only a few TA99-positive epidermal melanocytes and no detectable c-kit protein, whereas in naevus depigmentosus the expression of c-kit protein was strong, and TA99 immunoreactivity was faint. In vitiligo lesions, no epidermal immunoreactivity for melanosomes or c-kit protein was found. Normally pigmented skin of all patients showed immunoreactivity of epidermal melanocytes for both c-kit protein and melanosomes.Different hypomelanotic lesions can thus be differentiated by absent melanocyte c-kit protein and low or no expression of melanosomal marker in piebaldism, normal c-kit but low melanosome expression in naevus depigmentosus, and the absence of all melanocyte markers in vitiligo.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Science Ltd
    British journal of dermatology 153 (2005), S. 0 
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Background  Cutaneous T-cell lymphoma (CTCL) is a slowly progressive malignancy for which there is no cure. Therefore, accurate prediction of prognosis is important for the conduct of clinical trials and for counselling of individuals.Objectives  To improve prediction of survival in patients with CTCL.Methods  Prognostic factors including tumour–node–metastasis (TNM) criteria and the CTCL Severity Index (CTCL-SI) were analysed using a Weibull model for multivariate analysis in a sample of 62 patients with classical CTCL (mycosis fungoides and Sézary syndrome). The Brier score was used to quantify the quality of individual prediction.Results  Estimated 5-year survival rate (SR5) differed according to TNM stage: stage IA, 100% (95% confidence interval 70–100%); IB–III, 86% (73–100%); IVA, 54% (32–91%); IVB, 0% (0–52%). In a multivariate analysis, two independent prognostic factors were identified: lymph node (P = 0·036) and blood involvement (P = 0·015). A probability of survival model showed correlation of CTCL-SI with survival in patients with CTCL-SI 〉 20 according to the following formula: SR5 = 124–2 × (CTCL-SI)%. Calibration of SR5 against CTCL-SI-independent CTCL subsets revealed underestimation of Sézary syndrome. When CTCL-SI parameters were adjusted accordingly, the probability of survival model did not change significantly, while SR5 values became adequate. In addition, CTCL-SI was shown to be superior to TNM by 30% regarding individual predictive power.Conclusions  Probability of survival in CTCL can be accurately predicted by a CTCL-SI-based survival rate formula. Careful monitoring of lymph node and blood compartments and quantification by CTCL-SI are reliable tools for follow-up of patients with CTCL and allow progression-adjusted prediction of prognosis.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Science, Ltd
    British journal of dermatology 146 (2002), S. 0 
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: We report on a 74-year-old female patient with a primary cutaneous CD20+, diffuse large cell B-cell lymphoma of the lower leg resembling a chronic non-healing leg ulcer. There was no evidence of systemic involvement on computed tomography (CT) scans of the chest, abdomen and pelvis; a slightly enlarged lymph node in the right groin showed dermatopathic lymphadenopathy on histology and immunohistochemistry. Involvement of the bone marrow and peripheral blood was ruled out by punch biopsy and fluorescent activated cell sorter (FACS) analysis of the blood, respectively. Therapeutic anti-CD20 monoclonal antibody rituximab was given at 375 mg m−2 i.v. once weekly for 7 weeks, without adverse effects, resulting in a minor improvement in the centre of the ulcerated tumour. Unfortunately, the response was not maintained, and after 7 weeks of treatment the patient started to develop new tumour lesions at the border of the ulcer. Local radiotherapy was started and combined photon and electron beam irradiation induced complete remission of the B-cell lymphoma.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: High variability of the clinical appearance of malignant melanoma (MM) and its metastases render the differential diagnosis of solid amelanotic tumours difficult. We report a 71-year-old woman with several unusual cutaneous tumours of cerebriform morphology, suggesting skin metastases from occult internal cancer. Histopathological findings and thorough investigations, however, revealed a late-stage metastatic MM. We discuss the differential diagnosis of skin metastases of various origin and underline the difficulties for early detection of MM.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Archives of dermatological research 287 (1994), S. 78-84 
    ISSN: 1432-069X
    Keywords: Stem cell factor ; Mast cells ; Melanocytes ; Growth factors
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Mechanisms affecting mast cell and melanocyte growth and function are still poorly understood. This report summarizes the current state of knowledge on a recently described growth factor for both these cell types and for primitive haematopoietic stem cells. Stem cell factor (SCF), also named mast cell growth factor or kit-ligand, has only recently been cloned and has been shown to be encoded on human chromosome 12. It may be of specific importance in cutaneous physiology and pathology since it is produced by several cell types in the skin (e.g. fibroblasts, keratinocytes, endothelial cells) and since it affects melanocyte and mast cell growth, survival, secretion and adhesion as well as migration into tissues. Defects in the genes encoding for the SCF receptor (c-kit-protein) have been shown to be responsible for human piebaldism. A pathogenetic role in mastocytosis has recently been proposed, but remains to be proven. SCF receptor expression is decreased on cells of some malignant cell lines compared to their physiological counterparts, making it unlikely that SCF is a key factor in malignant transformation and cellular hyperproliferation. In haematopoiesis, SCF acts primarily in concert with other growth factors, and we show here that alone in serum-free culture it has no effect on mast cell growth. Furthermore, there is evidence that besides SCF, additional mast cell growth factors are secreted by fibroblasts and keratinocytes, suggesting a complex orchestration of several growth factors in the regulation of cutaneous growth and differentiation in which SCF plays only one part.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1433-0458
    Keywords: Schlüsselwörter Epidermolysis bullosa acquisita ; Komplikationen im Kopf-Hals-Bereich ; Therapie ; Extrakorporale Photopherese ; Immunsuppression ; Key words Epidermolysis bullosa acquisita ; Head and neck complications ; Extracorporeal phototherapy ; Immunosuppression
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary 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.
    Notes: Zusammenfassung Die Epidermolysis bullosa acquisita (EBA) ist eine seltene, erworbene, bullöse Autoimmundermatose. Sie ist durch die Ablagerung von IgG-Autoantikörpern gegen das Typ- VII-Prokollagen der Verankerungsfibrille und subepidermale Blasenbildung mit Vernarbung charakterisiert. Die Epidemiologie dieser chronischen Erkrankung und ein charakteristisches klinisches Bild können zum gegenwärtigen Zeitpunkt aufgrund nur weniger Berichte im Schrifttum nicht genau eingeschätzt werden. Bullöse Effloreszenzen treten sowohl am Integument als auch an den Schleimhäuten auf. Die Entwicklung narbiger Verwachsungen der Schleimhäute der oberen Atemwege und des Ösophagus ist sehr selten und nur im Einzelfall beschrieben. Wir berichten über einen 44jährigen Patienten mit massiver Blasenbildung an der Augen-, Nasen-, Mund-, Pharynx- und Larynxschleimhaut und vereinzelten Hautläsionen mit anschließender Vernarbung, bei dem die Diagnose einer EBA gestellt wurde. Bei interdisziplinärer konservativer und operativer Zusammenarbeit war es nach verschiedenen frustranen Therapieansätzen möglich, die Entwicklung aktiver Läsionen mit darauffolgenden narbigen Verwachsungen und Stenosen u.a. mittels extrakorporaler Photopherese und Ciclosporin-A einzudämmen.
    Type of Medium: Electronic Resource
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