[LSD score. A new classification system for peristomal skin lesions]. / LSD-Score. Ein neues Klassifikationssystem für peristomale Hautläsionen.

BACKGROUND: Peristomal skin lesions are frequent complications of ostomy; however, there is no generally accepted nomenclature and classification system. OBJECTIVE: An interdisciplinary German expert panel (GESS) composed of ten members, developed an innovative semiquantitative classification system for peristomal skin lesions for further stratification of ostomy therapy. This score is based on criteria which can be assessed by stomal therapists and treating physicians. RESULTS: The new peristomal skin lesion score grades three categories: lesion (L), status of ostomy (S) and disease (D). The L category describes the integrity of the skin as normal (L0), lesion with sustained integrity of skin (L1), integrity destroyed (L2) and local infection (L3). The S category rates the complexity of ostomy therapy as normal (S0), increased (S1) and high but not sufficiently effective (S2). The additional letters for categorization O. R. P. H. E. US describe anatomical pathologies of the stoma itself: ostomy stenosis (O), retraction (R), prolapse (P), hernia (H), edema (E) and unfavorable site (US). A systemic disorder is either absent (D0), irrelevant (D1) or relevant (D2). The LSD score is the basis for a management algorithm. CONCLUSION: The LSD score is comprehensive, standardized and holistic. Its straightforward use by health professionals can improve the consistency of the description of skin lesions and enhance the quality of ostomy therapy.

Optical surface profiling of orb-web spider capture silks.

Much spider silk research to date has focused on its mechanical properties. However, the webs of many orb-web spiders have evolved for over 136 million years to evade visual detection by insect prey. It is therefore a photonic device in addition to being a mechanical device. Herein we use optical surface profiling of capture silks from the webs of adult female St Andrews cross spiders (Argiope keyserlingi) to successfully measure the geometry of adhesive silk droplets and to show a bowing in the aqueous layer on the spider capture silk between adhesive droplets. Optical surface profiling shows geometric features of the capture silk that have not been previously measured and contributes to understanding the links between the physical form and biological function. The research also demonstrates non-standard use of an optical surface profiler to measure the maximum width of a transparent micro-sized droplet (microlens).

[Allergic contact dermatitis to common ivy (Hedera helix L.)]. / Allergische Kontaktdermatitis auf Efeu (Hedera helix L.).

Common ivy (Hedera helix L.) is a ubiquitous plant in Europe whose major allergen falcarinol has moderate allergic potential. It is not related to poison ivy (Toxicodendron spp.). There are no cross reactions between the allergens of common ivy (falcarinol) and poison ivy (urushiol). Contact with common ivy or falcarinol may lead to sensitization and then a delayed hypersensitivity reaction. There are only few cases described in the literature. We report on a male hobby gardener with appropriate clinical history and positive patch test. The pathogenic mechanism is a type IV reaction following a sensitization exposure. Gardeners and landscape architects with frequent exposure to common ivy and thus a high risk of sensitization should wear appropriate protective clothing.

Subcutaneous T-cell lymphoma.

We describe a patient with severe fatal histiocytic phagocytic panniculitis caused by a pleomorphic T-cell lymphoma. Analysis by polymerase chain reaction revealed clonality for both the T-cell receptor gamma-chain gene and the immunoglobulin heavy-chain gene. We also review 44 reported cases of primary or secondary lymphoma affecting the subcutaneous fat.

The transformation of pityriasis lichenoides chronica into parakeratosis variegata in an 11-year-old girl.

Parakeratosis variegata is a rare disorder with unknown aetiology. In a few cases it arises from benign skin diseases such as pityriasis lichenoides et varioliformis acuta (Mucha Habermann disease) or pityriasis lichenoides chronica. However, transformation into malignant diseases such as cutaneous T-cell lymphoma has been observed. We report an 11-year-old girl with a 10-year history of pityriasis lichenoides chronica now presenting with parakeratosis variegata. Analysis of skin infiltrating T cells showed clonally rearranged T-cell receptor gamma chains occurring with a frequency of more than 2%. This finding is compatible with the clinical observation of parakeratosis variegata transforming into a malignant T-cell disorder. We therefore suggest that patients suffering from parakeratosis variegata and other diseases such as pityriasis lichenoides et varioliformis acuta or pityriasis lichenoides chronica should be continuously monitored.

Evidence for a paracrine pathway of B-cell stimulation in hairy cell leukaemia.

It is a well-known phenomenon that the growth of malignant B-lymphocytes, i.e. hairy cells, is regulated by cytokines. Several investigators have suggested that the stimulating cytokines are produced by the malignant B cells themselves, indicating an autocrine growth regulation. In this paper we demonstrate that T-lymphocyte clones produce soluble mediators which stimulate the growth of malignant B lymphocytes. The incidence of the growth-stimulating T-cell clones derived from peripheral blood is identical in patients with hairy cell leukaemia (HCL) and healthy controls. About 50% of the clones stimulate the growth of hairy cells, but not the growth of purified B lymphocytes of healthy donors. The stimulating activity of a single clone varies when tested on different hairy cells. Interferon alpha (IFN alpha), but not antibodies against tumour necrosis factor alpha (TNF alpha) or interleukin-2 (IL-2), completely inhibit the growth-stimulating activity. Our results indicate that a paracrine growth regulation has to be considered in addition to the postulated autocrine loop in the growth regulation of malignant B cells.

[Modern molecular biology diagnosis of malignant cutaneous lymphomas]. / Moderne molekular-biologische Diagnostik kutaner maligner Lymphome.

The diagnosis of cutaneous malignant lymphoma is based on clinical, histo-morphological and immunochemical findings and, now a days, on molecular biology analyses of the genotype in the lymphocytic infiltrate. By using the polymerase chain reaction (PCR) with specific primers for the Ig heavy chain gene and the T-cell receptor gamma chain gene, the detection of monoclonal cell populations in the skin infiltrate is possible. Since this method produces results within 3 days, since paraffin-embedded skin and lymph node biopsies and heparinized peripheral blood can be used and since no radioactivity is necessary, this technique has important advantages over traditional techniques such as Southern blot analyses. In addition, specific PCR analyses may allow a patient-specific monitoring during therapy and also may detect early relapses of the lymphoproliferative malignant disease.

Use of polymerase chain reaction in the detection of clones in lymphoproliferative diseases of the skin.

Early-stage mycosis fungoides shows similar clinical symptoms and histological and immunophenotypical features to several benign lymphoproliferative skin disorders. We analyzed T cell receptor gamma gene rearrangement by polymerase chain reaction in the search for monoclonal lymphoid subpopulations in skin infiltrates. Totally, 283 skin biopsies (paraffin-embedded and frozen material) from patients with different malignant and reactive skin diseases were investigated. Using primers for the T cell receptor gamma chain gene, monoclonality was detected in 59 out of 66 (89%) cases of pleomorphic cutaneous lymphoma, in 60 out of 78 (77%) patients with mycosis fungoides, in 11 out of 22 (50%) cases of parapsoriasis en plaques, in five out of 35 (14%) cases of pseudolymphoma, in six out of 15 (40%) patients with lymphomatoid papulosis, and in none out of 64 patients with inflammatory skin diseases. The results show that clonal T cell population can be detected in the majority of patients with cutaneous T cell lymphoma, but the findings have to be correlated with the histological and morphological features.

Systemic treatment for cutaneous lymphomas.

Primary malignant T cell lymphomas of the skin form a heterogenous group. Relevant classifications were recently made to separate different entities by various criteria. This is of great importance, because one should only rely on those therapeutical trials in which patients were included according such classifications. In this paper, we will mainly focus on therapeutic modalities for mycosis fungoides, which is the most frequent cutaneous T cell lymphoma and which may serve as a model disease. In principle, local (e.g., psoralens and ultraviolet A, PUVA) and systemic therapies (e.g., interferon-alpha 2a) can be applied. Very recently, we were able to demonstrate that even in initial stages of mycosis fungoides, the T cell clone is not restricted to the skin, but rather is present in low amounts in the peripheral blood. Therefore, systemic therapeutic modalities alone or in combination with local strategies (interferon-alpha 2a and acitretin/PUVA and interferon-alpha 2a) should be more effective, which will be proven by currently running clinical trials.

["Granulomatous slack skin"--cutaneous elastolytic lymphoma]. / "Granulomatous slack skin"--kutanes elastolytisches Lymphom.

The term "granulomatous slack skin" (GSS) was introduced by Ackerman for a disease first described by Convit et al. in 1973. GSS represents a rare cutaneous lymphoma characterized by localized elastolytic lesions with a granulomatous infiltrate. We recently observed two male patients with the characteristic features of this disease. Both patients responded well to therapy with interferon-alpha 2b. In one patient clinical remission was stable under long-term treatment with clofazimine. We report on common features of these two patients and give a review of the cases published in the literature.

Clonal disease in early cutaneous T-cell lymphoma.

Our experience with 185 cases of T-cell dominated skin infiltrates demonstrates that the PCR-based method for detection of TCR gamma chain gene rearrangement in combination with temperature gradient electrophoresis can routinely be used for the demonstration of clonal T cells in formalin-fixed and paraffin-embedded biopsies of lesional skin. In contrast to Southern blot analysis, the amplification by PCR is nonradioactive, is not time consuming (approximately 3 days), can be performed using frozen or paraffin-embedded tissue, and allows additional molecular biologic analyses, such as sequencing. Furthermore, it offers the possibility to design patient-specific primers for monitoring of the disease activity. It also has to be concluded from our study that all available clinical, histologic, cytologic, immunophenotypical, and rearrangement studies have to be considered in order to establish the correct diagnosis.

Paracrine regulation of B-cell growth in hairy cell leukemia.

There is evidence that the growth of malignant B lymphocytes e.g. hairy cells is regulated by cytokines. Several investigators suggested that the stimulating cytokines are produced by the malignant B cells indicating an autocrine growth regulation. Here we demonstrate that T lymphocyte clones produce soluble mediators which stimulate the growth of malignant B lymphocytes. The incidence of the growth stimulating T cell clones derived from peripheral blood is identical in patients with hairy cell leukemia (HCL) and healthy controls. About 50% of the clones stimulate the growth of hairy cells, but not the growth of purified B-lymphocytes of healthy donors. The stimulating activity of a single clone varies when tested on different hairy cells. Interferon alfa but not antibodies against tumor necrosis factor alfa or interleukin-2 inhibit completely the growth stimulating activity. We propose that interferon alpha inhibits the production of soluble mediators produced by normal T-cells. Our results indicate that a paracrine growth regulation has to be considered in addition to the postulated autocrine loop in the growth regulation of malignant B cells.