Primär kutane Lymphome zeigen definitionsgemäß zum Zeitpunkt der Diagnosestellung keinen weiteren Organbefall [2]. Beim Auftreten kutaner Lymphome ist stets eine sekundäre Hautbeteiligung bei primär extrakutanen Lymphomen in Betracht zu ziehen. Wir stellen eine 85-jährige Patientin mit einem CD30+-intestinalen T-Zell- Lymphom vor, dessen Erstsymptom multiple, noduläre, zentral ulzerierende Läsionen an der Haut waren. Kutane Absiedelungen beim intestinalen T-Zell-Lymphom stellen eine Rarität dar und sind in der Literatur nur als Einzelfälle beschrieben. Im vorliegenden Fall konnten die kutanen Absiedelungen trotz initialer Durchuntersuchung erst verzögert im Verlauf gänzlich verstanden werden, nachdem es komplizierend zu einer Dünndarmperforation gekommen war und die histologische Diagnose eines T-Zell-Lymphoms des Jejunums gestellt wurde. Eine Chemotherapie nach dem CHOP-21-Schema (Cyclophosphamid, Doxorubicin, Vincristin und Prednisolon) zeigte im Re-Staging eine komplette intestinale sowie kutane Remission. Der vorliegende Fall zeigt besonders eindrücklich, dass auch bei kutanen T-Zell-Lymphomen stets an sekundäre Hautinfiltrate bei extrakutanen Lymphomen gedacht werden muss und eine konsequente Durchuntersuchung gegebenenfalls auch wiederholt veranlasst werden muss.

CD30-positive anaplastic large T-cell lymphomas are either primary cutaneous T-cell lymphomas or primary systemic T-cell lymphomas. Primary cutaneous CD30-positive anaplastic large T-cell lymphomas are usually ALK-negative whereas systemic CD30-positive T-cell lymphomas are more often ALK-positive (60 – 80 %). We present a rare case of a 67 year old patient with a systemic CD30-positive ALK-negative T-cell lymphoma and secondary cutaneous manifestation. This form of a CD30-positive anaplastic large T-cell lymphoma has a bad prognosis and must be treated more aggressively than a primary cutaneous form of CD30-positive ALK-negative anaplastic large T-cell lymphoma.

CD30-positive anaplastic large T-cell lymphomas are either primary cutaneous T-cell lymphomas or primary systemic T-cell lymphomas. Primary cutaneous CD30-positive anaplastic large T-cell lymphomas are usually ALK-negative whereas systemic CD30-positive T-cell lymphomas are more often ALK-positive (60-80%).We present a rare case of a 67 year old patient with a systemic CD30-positive ALK-negative T-cell lymphoma and secondary cutaneous manifestation. This form of a CD30-positive anaplastic large T-cell lymphoma has a bad prognosis and must be treated more aggressively than a primary cutaneous form of CD30-positive ALK-negative anaplastic large T-cell lymphoma.

Terminologie und Klassifikation Klinik, Histologie und Diagnosestellung Stadieneinteilung Therapie und Prognose

At the time of initial diagnosis, primary cutaneous lymphoma by definition don't show any spread to other organs of the body. On the other hand primary extracutaneous lymphoma may cause secondary skin lesions. We present a case of an 85 years old female patient with a CD30+ intestinal T-Cell lymphoma. The initial symptoms of the disease were multiple nodular skin lesions with central ulceration. Cutaneous lesions are rare in intestinal lymphoma and so far only singular cases have been described. The initial staging exam couldn't find any extracutaneous lymphoma. Two months later the patient suffered an intestinal perforation. The histological examination of the specimen from the jejunal resection detected an intestinal T-Cell lymphoma. Chemotherapy was initiated and a complete remission of the cutaneous und intestinal lesions was achieved after 6 cycles of CHOP-21. As shown in this particular case the presentation of a cutaneous T-cell lymphoma requires an accurate and repeated staging exam considering the possible presence of secondary skin lesions of an extracutaneous lymphoma.

Im Vergleich zu indolenten Formen primär kutaner B-Zell-Lymphome kommt es beim diffus-großzelligenTyp häufig zu einer extrakutanen Beteiligung. höheren Erwachsenenalters, können jedoch auch zu Beginn des Erwachsenenalters auftreten.

Der vorliegende Fall zeigt die seltene Assoziation eines Basalzellkarzinoms mit Dermatofibromen auf eindrückliche Weise. Das Augenmerk soll hierbei auf die möglichen histopathologischen Veränderungen der Epidermis über Dermatofibromen, welche von leichter Akanthose bis zum Basalzellkarzinom reichen kann, gelenkt werden. Ein 43-jähriger Patient berichtet über seit 26 Jahren bestehende, gruppierte Hautveränderungen am rechten Knie, welche histologisch als Dermatofibrome eingeordnet werden konnten. Im Zentrum der Gruppe von Dermatofibromen kam es in den letzten Monaten zunehmend zu einer Plaquebildung und schließlich Ulzeration. Histologisch zeigte sich ein ausgedehntes sklerodermiform wachsendes Basalzellkarzinom, randlich angrenzend an umgebende Dermatofibrome. Es erfolgte eine Exzision im Gesunden mit anschließender Spalthauttransplantation und Vakuumversiegelung.

We present a case in which the rare association between basal cell carcinoma and dermatofibroma is demonstrated. Dermatofibromas are benign dermal tumors that frequently exhibit a spectrum of epidermal changes. Most frequent mild acanthosis is present and very rarely the diagnosis of a dermatofibroma and a basal cell carcinomawas made. A 43-year old man presented with a 26-year history of dermatofibromas on his right knee. The lesion had enlarged over time and an ulceration was observed in recent months. Surgical excision of the lesion was performed and hematoxylin-eosin staining showed a basal cell carcinoma adjoining to dermatofibroma. After micrographic surgery a split-thickness skin graft was performed.

Wir berichten über einen 74-jährigen asiatischen Patienten mit seit 3 Jahren bestehenden, generalisierten, juckenden, rauen Papeln und Plaques. Die Laboranalyse zeigte eine periphere Eosinophilie, Lymphozytopenie und einen erhöhten Serum-IgE-Wert. Die Körperfalten waren ausgespart und die Histologie zeigte eine spongiotische Dermatitis. In Zusammenschau der Befunde konnte die Diagnose Papuloerythrodermie Ofuji gestellt werden. Eine UVA-1-Lichttherapie in Kombination mit topischen Glukokortikosteroiden und Antihistaminika wurde durchgeführt.

Korrespondenzadresse Dr. med. Hermann Kneitz Klinik für Dermatologie, Venerologie und Allergologie Julius-Maximilians-Universität Josef-Schneider-Str. 2, Gebäude D8 97080 Würzburg kneitz_h@klinik.uni-wuerzburg.de Anamnese ! Bei einem 58-jährigen Patienten bestand seit 8 Jahren ein kutaner, nicht schmerzhafter Knoten mit nur geringer, aber kontinuierlicher Größenprogredienz am rechten Nasenabhang (●" Abb. 1). Anamnestisch ist der Knoten nie entzündet gewesen, intermittierend bestand ein geringer Juckreiz.

We report on a 74 year old asian patient with a 3-year history of generalized pruritic rash with scaly papules coalesced into plaques. Blood tests showed peripheral eosinophilia, lymphocytopenia and increased serum IgE. The lesions spared the skin folds and histology showed features of spongiotic dermatitis. The diagnosis of Papuloerythroderma of Ofuji was made and the patient was treated with UVA1-therapy, topical corticosteroids and antihistamines.

Journal Article Simvastatin‐induced amyopathic dermatomyositis Get access O. Inhoff, O. Inhoff Department of Dermatology, University Hospital Mannheim, University of Heidelberg, 68135 Mannheim, Germany Search for other works by this author on: Oxford Academic Google Scholar W.K. Peitsch, W.K. Peitsch Department of Dermatology, University Hospital Mannheim, University of Heidelberg, 68135 Mannheim, Germany Search for other works by this author on: Oxford Academic Google Scholar B.E. Paredes, B.E. Paredes Dermatopathologische Gemeinschaftspraxis, Friedrichshafen, Germany Search for other works by this author on: Oxford Academic Google Scholar S. Goerdt, S. Goerdt Department of Dermatology, University Hospital Mannheim, University of Heidelberg, 68135 Mannheim, Germany Search for other works by this author on: Oxford Academic Google Scholar M. Goebeler M. Goebeler Department of Dermatology, University Hospital Mannheim, University of Heidelberg, 68135 Mannheim, Germany Correspondence: Matthias Goebeler (Current address: Department of Dermatology, University Hospital Giessen, University of Giessen, 35385 Giessen, Germany) E‐mail: matthias.goebeler@derma.med.uni‐giessen.de Search for other works by this author on: Oxford Academic Google Scholar British Journal of Dermatology, Volume 161, Issue 1, 1 July 2009, Pages 206–208, https://doi.org/10.1111/j.1365-2133.2009.09205.x Published: 01 July 2009

In an approach to discover new inhibitors of trypanothione reductase from Trypanosoma cruzi, the causative agent of Chagas' disease, a virtual high-throughput screening was performed. Two structurally new types of inhibitors emerged, the antimicrobial chlorhexidine {1,1'-hexamethylenebis[5-(4-chlorophenyl)biguanide]}, a linear competitive inhibitor (K(i) = 2 +/- 1 microM), and a piperidine derivative acting as mixed inhibitor (K(i) = 6.2 +/- 2 microM and K(i)' = 8.5 +/- 2 microM). Neither compound interferes with human glutathione reductase. Based on chlorhexidine, different series of compounds were synthesized and studied as inhibitors of T. cruzi trypanothione reductase. Most efficient derivatives were three bis(amidines) showing mixed type inhibition with K(i,slope) and K(i,int) values of 2-5 microM and 16-47 microM, respectively. Although these compounds did not exert an improved inhibitory potency compared to chlorhexidine, the change from competitive to mixed-type inhibition is advantageous, since substrate accumulation does not overcome inhibition. Remarkably, all three derivatives carried two copies of an identical 2-methoxy-4-methyl-1-(phenylmethoxy)benzene substituent.

Mastocytoses are a heterogeneous group of diseases with a clinical spectrum ranging from localized mastocytoma to generalized forms such as mast cell leukemia. A pathological increase of mast cells in the tissue involved is common to all forms of mastocytosis. The most frequent clinical manifestation of mastocytosis is urticaria pigmentosa, which may occur confined to the skin or represent a symptom of systemic mastocytosis. The frequency of urticaria pigmentosa in systemic mastocytosis is inversely related to increasing malignancy. Recent studies suggest that in more than 50% of adult patients, urticaria pigmentosa is associated with systemic involvement, mainly bone marrow infiltration. Urticaria pigmentosa is usually easy to diagnose due to the typical clinical and histological findings. Here we discuss the importance of a correct diagnosis and classification according to current WHO standards as well as the therapeutic options and the prognosis of the disease. It is important that the c-kit receptor, which is present in its mutated form with high frequency in adult mastocytosis, is resistant against the c-kit inhibitor imatinib. The systemic adult mastocytoses with hypereosinophilia and FIP1L1-PDGFRA rearrangement are the only imatinib sensitive forms.

9-Aminoacridines and (terpyridine)platinum(II) complexes are competitive and irreversible inhibitors, respectively, of trypanothione reductase from Trypanosoma cruzi, the causative agent of Chagas' disease. Four chimeric compounds in which 2-methoxy-6-chloro-9-aminoacridine was covalently linked to the (2-hydroxyethanethiolate)(2,2':6',2' '-terpyridine)platinum(II) complex were synthesized and studied as inhibitors of the parasite enzyme. The derivatives differed by the nature and/or the length of the spacer connecting the two aromatic systems. All four compounds were effective mixed type inhibitors of trypanothione reductase with K(i) and K(i)' values of 0.3-4 and 2-11 microM, respectively. The most potent inhibitor had an ethylthioether linkage between the two aromatic ring systems, and the other compounds contained an alkyl ether group with 4-6 methylene groups. In contrast to the parasite enzyme, human glutathione reductase, the closest related host enzyme was not inhibited by these compounds. The finding that the conjugation of a competitive and an irreversible inhibitor can give rise to reversible mixed type inhibitors underlines the difficulties associated with inhibitor design based on the three-dimensional structure of trypanothione reductase.