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Claus-Detlev Klemke

Publications and source records attributed to Claus-Detlev Klemke.

13 recordsLinked to original sources
PubMed2007

Multiple juvenile xanthogranulomas successfully treated with CO laser.

Juvenile xanthogranulomas are a rare disease entity within the spectrum of cutaneous non-Langerhans cell histiocytoses characterized by single or multiple cutaneous xanthogranulomas without further organ involvement. A 16-year-old girl developed multiple xanthogranulomas in various skin regions, especially the trunk and the flexural areas, over a period of 6 months. The clinical diagnosis was confirmed by H&E histology, showing a histiocytic infiltrate admixed with foam cells and Touton giant cells, and by immunohistochemistry using anti-bodies to stabilin-1 whose expression is highly specific for cutaneous non-Langerhans cell histiocytoses. No diabetes insipidus, pituitary hyperplasia or paraproteinemia were observed. Skin lesions were treated with a CO(2) laser and did not reappear during a 5 years follow-up period.

Adolescent↗
PubMed2006

Functional characterization of the epidermal cholinergic system in vitro.

The aim of this study was to analyze the influence of cholinergic and anticholinergic drugs on epidermal physiology using organotypic cocultures (OTCs). Blocking of all acetylcholine receptors (AChRs) by combined treatment with mecamylamine and atropine or treatment with strychnine (blocking alpha9nAChR) for 7-14 days resulted in a complete inhibition of epidermal differentiation and proliferation. Blockage of nicotinic (n)AChR with mecamylamine led to a less pronounced delay in epidermal differentiation and proliferation than blockage of muscarinic (m)AChR with atropine, evidenced by reduced epithelial thickness and expression of terminal differentiation markers like cytokeratin 2e or filaggrin. In OTCs treated with atropine, mecamylamine, or strychnine, we could demonstrate intracellular lipid accumulation in the lower epidermal layers, indicating a severely disturbed epidermal barrier. In addition, we observed prominent acantholysis in the basal and lower suprabasal layers in mecamylamine-, atropine-, and strychnine-treated cultures, accompanied by a decreased expression of cell adhesion proteins. This globally reduced cell adhesion led to cell death via intrinsic activation of apoptosis. In contrast, stimulation of nAChR and mAChR with cholinergic drugs resulted in a significantly thickened epithelium, accompanied by an improved epithelial maturation. In summary, we show that epidermal AChR are crucially involved in the regulation of epidermal homeostasis.

Acetylcholine↗
PubMed2006

New insights into the molecular biology and targeted therapy of cutaneous T-cell lymphomas.

Cutaneous T-cell lymphoma is an extra-nodal non-Hodgkin lymphoma of mature T cells. These tumor cells home to and persist in the skin,producing a broad spectrum of clinical entities. Recent results of basic research on tumor biology and tumor immunology as well as molecular genetics of cutaneous T-cell lymphoma have fostered the development of new therapeutic approaches. Several clinical trials testing these targeted therapies have shown encouraging results. This article provides an overview of recent research developments and therapeutic strategies for cutaneous T-cell lymphoma.

Animals↗
PubMed2005

Additional Her 2/neu gene copies in patients with Sézary syndrome.

BACKGROUND: Her 2/neu gene amplification has been reported in several types of cancer. Monoclonal antibodies against the Her 2/neu receptor are used as a treatment in e.g. metastatic breast cancer. AIM: The aim of this study was to determine the frequency of additional Her 2/neu gene copies in relations to the number of chromosome 17 centromeres of patients with Sézary syndrome. METHODS: Fluorescence in situ hybridization (FISH) with probes specific for the Her 2/neu gene locus and the centromere of chromosome 17 was performed on nuclei from peripheral blood cells of 9 patients with Sézary syndrome. For analysis of Her 2/neu protein expression immunostaining was performed. In addition, FISH was used to analyze distribution of typical lymphocytes on cryo-cut sections of affected skin of two patients. RESULTS: 7/9 cases showed additional Her 2/neu gene copies in relation to the number of chromosome 17 centromeres. 4/5 cases with additional Her 2/neu gene copies in which immunostaining was performed expressed Her 2/neu protein. On cryo-cut sections atypical lymphocytes with additional Her 2/neu gene copies were detected in the dermis as well as in the epidermis of affected skin. DISCUSSION: These data suggest that Her 2/neu might be involved in the pathogenesis of Sézary syndrome and that Her 2/neu might be a promising target for antitumor therapy in a subgroup of patients.

Aged↗
PubMed2005

[Ulceration of the scalp: a unique manifestation of pyoderma gangrenosum].

Pyoderma gangrenosum is a chronic, noninfectious, ulcerating cutaneous disease of unknown etiology usually presenting with multiple lesions on the legs and trunk. Solitary lesions in other locations are rare and therefore hard to diagnose. A 78-year-old and a 81-year-old woman with extensive scalp necrosis were both diagnosed as pyoderma gangrenosum after exclusion of other differential diagnostic possibilities. The separate diagnosis of malignant pyoderma for this disease state no longer seems justified. Scalp necrosis is infrequently seen in dermatological patients; most previous reports describe it in giant cell arteritis. Other differential diagnoses include viral, bacterial and mycotic infections as well as erosive pustular dermatosis of the scalp. In conclusion, we again draw attention to unilesional pyoderma gangrenosum of the scalp as a rare disease entity. Immunosuppressive drugs are an effective treatment modality.

Aged↗
PubMed2003

Atypical generalized eruptive histiocytosis associated with acute monocytic leukemia.

Histiocytoses are diseases caused by proliferation of either dendritic cells/Langerhans cells or of monocytes/macrophages. Generalized eruptive histiocytosis belongs to the cutaneous non-Langerhans cell histiocytoses and is a rare monocyte-macrophage proliferative disorder that usually follows a benign clinical course. We present the case of a 59-year-old man who presented with a 7-month history of progressively developing erythematous macules and slightly elevated papules widely distributed over the trunk, neck, face, and thighs. Ultrastructurally, no Birbeck granules were observed, and immunochemistry did not reveal any S-100 protein or CD1a antigen in any of the lesional cells, excluding Langerhans cell histiocytosis. In addition, the histiocytic infiltrate in the skin of our patient was shown to strongly express MS-1 high molecular weight protein, a marker described as highly characteristic for cutaneous non-Langerhans cell histiocytoses. Bone-marrow smear examination and flow cytometric analysis revealed monocytic leukemia. This is the second report of generalized eruptive histiocytosis associated with acute monocytic leukemia. We discuss the differential diagnoses of the clinical picture and stress that this benign cutaneous disorder may indicate an underlying hematologic malignancy.

Back↗
PubMed2003

Common clonal T-cell origin in a patient with T-prolymphocytic leukaemia and associated cutaneous T-cell lymphomas.

An unusual course was observed in a patient with indolent T-prolymphocytic leukaemia (T-PLL) who subsequently developed mycosis fungoides (Mf), lymphomatoid papulosis (LyP) and cutaneous CD30+ anaplastic large cell lymphoma (ALCL). Polymerase chain reaction analysis demonstrated identical monoclonal T-cell receptor-beta and -gamma gene rearrangements in all the different clinical entities. Furthermore, cytogenetic studies revealed the same aberrant clone with trisomy of chromosome 8 in T-PLL and ALCL cells. This unique observation suggests that in T-PLL, the leukaemic cells might undergo secondary transformation, subsequently resulting in different phenotypes of cutaneous T-cell lymphoma.

Aged↗
PubMed2003

Predominant telangiectatic erythema in linear atrophoderma of Moulin: novel variant or separate entity?

Linear atrophoderma of Moulin is a distinctive disease originally described in 1992 and characterized by acquired, mildly atrophic, non-sclerotic, slightly hyperpigmented lesions following the lines of Blaschko. Here, we describe a 15-year-old girl with a 13-year history and a 29-year-old male with a 6-year history of prominent linear telangiectatic erythema and mild atrophoderma following the lines of Blaschko that involved the right leg and hip, and both legs, the trunk and both arms, respectively. As pronounced telangiectatic erythema within lesions of atrophoderma of Moulin has not hitherto been described, we propose that the disease in our patients represents a novel variant of linear atrophoderma of Moulin. Due to considerable overlap, we do not favour the notion that our cases constitute an entity entirely separate from linear atrophoderma of Moulin.

Adolescent↗
PubMed2002

Clonal T cell receptor gamma-chain gene rearrangement by PCR-based GeneScan analysis in the skin and blood of patients with parapsoriasis and early-stage mycosis fungoides.

Cutaneous T cell lymphoma (CTCL) and reactive T cell skin diseases represent opposite ends of a spectrum of diseases ranging from overtly malignant to persistently benign. Within this spectrum, the parapsoriasis group is not clearly defined regarding malignant potential. In contrast to consistent findings in advanced-stage CTCL, clonality analysis of parapsoriasis has produced conflicting results in previous studies. As T cell receptor gamma-chain polymerase chain reaction GeneScan analysis (TCR-gamma-PCR-GSA) stands out by its sensitivity, its accuracy in size determination of PCR products, its capacity to identify false positives by repeated analysis and its easy applicability, this approach was used to analyse the clonality status of 41 patients with borderline T cell lymphoproliferative skin diseases, including parapsoriasis (n=27) and early-stage mycosis fungoides (MF) (n=14). A monoclonal T cell infiltrate was demonstrated by repeated TCR-gamma-PCR-GSA in lesional skin specimens in 19.2% of parapsoriasis patients and in 66.6% of early-stage MF cases (p=0.013). In peripheral blood, a monoclonal T cell population was found in a similar percentage of parapsoriasis and of early-stage MF patients (26.7% versus 12.5%; p=0.611). A detailed analysis of parapsoriasis subentities, namely small and large plaque parapsoriasis, and parapsoriasis lichenoides, revealed monoclonality in 2(6)/2(5), 3(14)/2(8) and 0(6)/0/(3) of the skin and peripheral blood specimens, respectively. The high detection rate of false positive cases by repeated analysis (20-37.5%) provides a corrected perspective for the high rates of dominant T cell clones found by others in the peripheral blood of such patients. From the results obtained, three major conclusions can be drawn: firstly, CTCL is clearly associated with detection of monoclonality, even in its early stages; secondly, monoclonality is not a prerequisite for potential CTCL precursor entities; and thirdly, recirculating malignant T cells identical to the skin clone are not readily detected in parapsoriasis or early-stage MF, but may rather indicate disease progression.

Adult↗