Small plaque (digitate) parapsoriasis is an 'abortive cutaneous T-cell lymphoma' and is not mycosis fungoides.
Explore the source record for details and available documents.
Diagnosis, Differential↗
Biomedical subjects
G Burg
Publications and source records attributed to G Burg.
Pathology of cutaneous T-cell lymphoma.
Clinical features as well as histomorphology and cytomorphology are the golden standard for the differentiation of the various nosologic entities of cutaneous lymphomas, consisting of pre-lymphomas and pseudolymphomas, abortive lymphoma, latent lymphoma, definite low grade malignant lymphoma, and high grade malignant lymphoma. Histomorphologic and cytomorphologic criteria allow additional subtyping of lymphoproliferative T-cell infiltrates of the skin.
Humans↗
Distinct subtypes of subcutaneous T-cell lymphoma.
Explore the source record for details and available documents.
Humans↗
Molecular defects in erythropoietic protoporphyria with terminal liver failure.
We identified two additional mutations in the ferrochelatase gene in two Swiss patients with erythropoietic protoporphyria (EPP). Ferrochelatase cDNA from patients was amplified by the polymerase chain reaction (PCR) and subjected to mutation analysis by sequencing PCR products either directly or after subcloning. The first patient, who underwent liver transplantation because of terminal liver failure, was identified as having a single point mutation (C to T) at nucleotide 175 that resulted in a Gln to stop codon conversion in one allele of the gene. In the second case, in which the patient has so far no liver involvement, a two-base deletion (T899G900) was found in one allele. Frameshift as a result of the deletion creates a stop codon. This study presents two new genotypes of EPP, including one with liver failure, a rare and fatal form of EPP.
Adult↗
Influence of various cytokines on the interleukin-2-dependent lysis of melanoma cells in vitro.
To obtain information about useful combinations of various cytokines in melanoma therapy, we studied the influence of interleukin-2 (IL-2) in combination with interferon-alpha (IFN-alpha), IFN-gamma and tumour necrosis factor-alpha (TNF-alpha) on the lytic activity of IL-2-stimulated cells in vitro. Peripheral mononuclear cells (PMC) were incubated for 4 days with various combinations of cytokines and used as effector cells. Two different melanoma cell lines (M19 and M26) were used as target cells. The lytic activity of stimulated PMC was determined using a modified hexosaminidase assay. IL-2 was mainly responsible for the lytic activity of the effector cells in a dose-dependent manner. IFN-alpha, IFN-gamma and TNF-alpha did not enhance lytic activity with an optimal IL-2 dose (50 IU/ml IL-2). Using a suboptimal IL-2 dose (5 IU/ml), they increased cytotoxicity. The specific lysis of M19 cells was significantly increased by pretreatment of the cells with 5 IU/ml IFN-alpha together with 50 IU/ml TNF-alpha (t-test, P < or = 0.001), while the specific lysis of M26 cells was increased by pretreatment with 5 IU/ml IFN-gamma. We conclude that the lysis of melanoma cells by cytotoxic cells in vitro can be enhanced by various cytokines. The optimal cytokine combination differed for the two melanoma cell lines tested.
Cytokines↗
[Pili torti et canaliculi in ectodermal dysplasia].
Uncombable hair is a heterogeneous symptom with a partially genetic background. In order to make an exact diagnosis, it is mandatory to recognize associated abnormalities, do pedigree analyses, and perform scanning electron microscopic studies of the hair. In a case of ectodermal dysplasia, ectrodactyly, cleft lip/palate (EEC) syndrome and in a patient with familial tricho-odonto-onychial ectodermal dysplasia with syndactyly, scanning electron microscopy demonstrated pili torti et canaliculi, helicotrichia and cuticular dystrophy. Congenital pili torti et canaliculi must be differentiated from pili torti and from pili trianguli et canaliculi ("cheveux incoiffables"), inasmuch as they may present in hypotrichosis congenita hereditaria of Marie-Unna or as part of complex ectodermal dysplasia syndromes with clefting of the lip/palate and/or limb defects. It is noteworthy that some of those patients show a dysmorphic facies and an atopic constitution in addition to the aforementioned abnormalities. We discuss the possible relationship of these syndromes to each other, with special respect to the Hay-Wells or ankyloblepharon, ectodermal dysplasia, cleft lip/palate (AEC) and the Rapp-Hodgkin (ectodermal dysplasia, midfacial hypoplasia, cleft lip/palate) syndromes.
Abnormalities, Multiple↗
[Actinobacillus actinomycetemcomitans isolated from a mycetoma (of the forearm)].
Mycetomas are subacute or chronic infections usually localized on unprotected skin areas and caused by fungi or Actinomyces. We report the case of a patient who suffered a splinter injury on his wrist after a motor accident. Six months later a cutaneous abscess with Actinobacillus actinomycetemcomitans developed on his wrist. Complete healing was obtained within 1 month after administration of ceftriaxon and doxycycline. The therapeutic management will be discussed.
Abscess↗
[The tumor suppressor gene p53 and its significance for dermatology].
The product of the p53 tumour suppressor gene is a sequence-specific DNA-binding protein that acts as a transcription factor and can inhibit transformation in vitro. Mutational inactivation of p53 is the most frequent genetic alteration found in human cancer. Point mutations of the p53 gene have been detected in about 50% of squamous cell carcinomas, basaliomas and cases of Bowen's disease. A significant portion of these mutations were CC-->TT or C-->T transitions suggestive of UV involvement in mutagenesis. Increased concentrations of p53 protein were immunohistochemically detected in cutaneous malignant melanomas, but p53 mutations are rare in this tumour.
Bowen's Disease↗
Ectodermal dysplasia with corkscrew hairs: observation of probable autosomal dominant tricho-odonto-onychodysplasia with syndactyly.
Explore the source record for details and available documents.
Ectodermal Dysplasia↗
Absence of p53 gene mutations in cutaneous melanoma.
Mutations in the p53 tumor suppressor gene are a common finding in many human malignancies. These mutations have been shown to inactivate the p53 protein and sometimes confer an oncogenic potential to the mutated gene. Type and pattern of p53 mutations may give clues to the tumor etiology, for example, ultraviolet-induced CC-->TT and C-->T transitions. Genomic DNA of 16 primary cutaneous melanomas of the superficial and nodular subtype and six melanoma metastases were screened for the presence of mutations in exons 5 to 8 of the p53 tumor suppressor gene, using the polymerase chain reaction and single-strand conformation polymorphism analysis, followed by direct DNA sequencing. We detected no mutations in any of the primary and metastatic melanomas in exons 5 to 8 of the p53 tumor suppressor gene. This indicates that, in contrast to skin carcinomas, p53 mutations are not operative in the evolution of human melanoma.
Base Sequence↗
Skin irritant reactivity following experimental cumulative irritant contact dermatitis.
Despite the frequency of irritant contact dermatitis, very little is known about the duration of barrier function impairment following cumulative irritant contact dermatitis. We studied post-irritation irritant reactivity by assessing the response to SLS irritation in previously irritated sites. Cumulative irritant contact dermatitis was induced on the forearms of 15 volunteers aged 18 to 50 years by repeated occluded application of 0.5% SLS 1 h per day over 3 weeks. 3, 6 and 9 weeks later, previously irritated and unirritated control sites were challenged with 2% SLS under occlusion for 23 h. Irritation was assessed by visual scoring, transepidermal water loss (TEWL) as an indicator of epidermal barrier function, and capacitance as a parameter of epidermal water content. While no difference in irritant reactivity between pre-irritated and unirritated sites was observed 3 weeks following irritant contact dermatitis, there was a significant hyporeactivity of previously irritated skin as expressed by clinical scores, TEWL and capacitance at 6 and 9 weeks. Our results indicate that epidermal barrier function remains altered even 9 weeks after cumulative irritant contact dermatitis. With regard to patch testing, post-irritation hyporeactivity might be a cause of false-negative tests on previously irritated sites.
Adolescent↗
Various cytokines modulate ICAM-1 shedding on melanoma- and CTCL-derived cell lines: inverse regulation of ICAM-1 shedding in a Sézary cell line by interferon-gamma.
BACKGROUND: Since soluble intercellular adhesion molecules 1 (sICAM-1) retain their ability to bind to their ligand, they can interfere in cell-mediated immunosurveillance. OBJECTIVE: We studied the impact of various cytokines on ICAM-1 release of several tumor cell lines. METHODS: Two melanoma cell lines (M19, M26), 1 B lymphoblastoid cell line (Daudi), 1 erythroleukemia cell line (K562), 1 Sézary-cell-derived cell line (SeAx), 1 cell line derived from a mycosis fungoides lesion (MyLa) and normal peripheral blood mononuclear cells (PBMC) were grown in the presence of interferon gamma (IFN-gamma), IFN-alpha, interleukin-2 (IL-2), IL-6 and tumor necrosis factor alpha (TNF-alpha) in various concentrations. ICAM-1 release into the supernatant was measured after 24 and 48 h using an enzyme-linked immunosorbent assay (ELISA). RESULTS: PBMC and the B lymphoblastoid cell line did not shed detectable amounts of sICAM-1. In all other cell lines, ICAM-1 shedding was found with and without cytokine stimulation. In all cell cell lines except the CTCL-derived one, ICAM-1 shedding was marginally affected by IFN-alpha, IL-2 and IL-6. TNF-alpha and IFN-gamma enhanced the shedding in a time- and dose-dependent manner. In the SeAx line, IL-2 and IFN-gamma inhibited ICAM-1 release. By contrast, TNF-alpha and IL-6 enhanced it. The CTCL-derived MyLa responded to IFN-alpha with a dose-dependent increase in ICAM-1 shedding. All other cytokines had marginal influence. CONCLUSION: Cytokine-modulated ICAM-1 shedding shows quantitative and qualitative differences in the investigated cell lines. This might have implications for the pathophysiology of cutaneous malignancies and their susceptibility to immunotherapies.
Antigens, CD↗
Eruptive hamartomatous clear-cell acanthomas.
This is a report on a 38-year-old female, suffering from over 100 solid papules arising on her legs, arms and trunk during the past 10 years. The histology shows the typical feature of clear-cell acanthoma (CCA). Extensive investigations did not reveal any extracutaneous abnormalities. This case is exceptional because of the high number of hamartomatous CCA which exceeds the maximal number of about 20 seen in multiple CCA so far and therefore is referred to as eruptive hamartomatous CCA.
Adult↗
Classification of cutaneous lymphomas.
Despite the many distinct features of cutaneous lymphomas, they are best classified according to the Kiel classification and the Working Formulation for clinical usage. T-cell lymphomas predominate (65%); however, there are a significant number of primary B-cell lymphomas of the skin (25%). Moreover, there are a variety of unusual and rare manifestations and nosologic entities of cutaneous lymphomas that cannot be seen in the lymph node owing to the peculiarities of the skin as a distinct homing organ and vice versa (10%). It is important to note that some cutaneous lymphomas have a better prognosis when occurring primarily in the skin than their corresponding entity in the lymph node.
Humans↗
Interrater and intrarater variabilities in the evaluation of cutaneous lymphoproliferative T-cell infiltrates. EORTC-Cutaneous Lymphoma Project Group.
Histologic and cytologic evaluation of CTCL is one of the most difficult challenges in dermatopathology. It was the purpose of this study to evaluate the reliability and reproducibility of histologic criteria in CTCL and the interrater and intrarater variabilities. An overall of 2375 ratings of sections from 41 patients with CTCL were given by a panel of 22 dermatopathologists and pathologists familiar with lymphoma histology, some of whom were looking repeatedly at the same series of sections. The results clearly indicate that interrater as well as intrarater variabilities are significant and can be decreased by discussion preceding the evaluation as well as by repeated rating indicating a learning effect. Therefore, histologic evaluations of criteria have to be taken with caution, and statistical evaluation of level of agreement expressed by kappa coefficient is always mandatory in this type of study.
Humans↗
Interrater and intrarater reliability of histologic criteria in early cutaneous T-cell lymphoma. An EORTC Cutaneous Lymphoma Project Group study.
Researchers in many fields have become increasingly aware of the observer as an important source of measurement error. Consequently, reliability studies must be conducted in experimental or survey situations to assess the level of observer variability in the measurement procedures to be used in data acquisition. The present investigation demonstrated that interobserver and intraobserver variability is generally high, even between investigators internationally recognized for their experience in lymphomatous disorders and even when dealing with noninitial CTCL lesions. Similarly, sensitivity and specificity are generally low, the former astonishingly, without significant differences between early and noninitial CTCL lesions.
Humans↗
Semimalignant ("pseudolymphomatous") cutaneous B-cell lymphomas.
Almost 50% of cutaneous B-cell lymphoproliferative infiltrates are derived from follicular center cells. Among these, about 25% show a rapidly progressive course, whereas about 75% account for flattening of the survival curve after about 7 years. This group is referred to as semimalignant ("pseudolymphomatous") cutaneous B-cell lymphoma (SM-CBCL). Clinical, histologic, and phenotypical criteria for their differentiation from B-cell pseudolymphoma and from CBCL have been investigated in 60 patients (11 CBCL, 30 SM-CBCL, 19 PSL). Semimalignant CBCL are different from malignant CBCL because they do not tend to disseminate to extracutaneous sites or to transform into high-grade malignant blast-type lymphomas; follicular center cell formation with CD21 positive dendritic reticulum cells is usually present; and normal survival time is not affected. On the other hand, SM-CBCL differ from PSL in that complete cure of the usually multiple and disseminated skin manifestations is not possible and that follicular center formation and the network of CD21-positive cells in conjunction with a kappa or lambda light chain restriction of cellular surface immunoglobulins is seen. If these and other criteria are taken together, differentiation of these various nosologic entities demanding different therapeutic approaches can be achieved with significant reliability.
Adolescent↗
Therapeutic approaches in cutaneous lymphoma.
Standard therapy of the CTCL MF and SS consists of topical glucocorticosteroids, PUVA, topical chemotherapy, and total skin electron beam irradiation in stages Ia and IIa; local radiation or total skin electron beam irradiation in stage IIb; and systemic chemotherapy in stages III and IV. The experimental treatment modalities, interferon-alpha and retinoids, especially arotinoid, are most effective in early stages of CTCL; in advanced stages, the effectiveness can be increased by combination regimens. Up to now, the most promising results are obtained by combination therapy of interferon and PUVA. When serotherapy is considered as a therapeutic alternative for patients in stage IIb to IVb, the benefit/risk ratio must be carefully analyzed. Anti-thymocyte-globulin and monoclonal antibodies, either alone or conjugated to radioisotopes or toxin, have shown some therapeutic effect but are still under investigation. Extracorporal photopheresis is well established in erythrodermic patients. Initial reports also have shown encouraging results with this treatment for stage Ib and in combination with methotrexate or interferon. Hexadecylphosphocholine, a new, well-tolerated topical agent, induced a remission rate of 50%, with 25% complete remission in CTCL patients of stage Ia to IIb.
Antineoplastic Agents↗