Presence Of Skin Involvement Research Articles

Selection and Efficacy of Cytoreductive Agents in Patients with Mastocytosis Included in the Registry of the European Competence Network on Mastocytosis (ECNM)

Selection and Efficacy of Cytoreductive Agents in Patients with Mastocytosis Included in the Registry of the European Competence Network on Mastocytosis (ECNM)

Is immunosuppressive therapy the anchor treatment to achieve remission in systemic sclerosis?

Since activation of the immune system and a perivascular infiltrate of inflammatory cells are key features of SSc, immunosuppression has long been considered to be an anchor treatment. Non-selective immunosuppression remains central to the treatment of interstitial lung disease (ILD) and skin involvement, with CYC most widely used to obtain remission. The use of MTX as a first-line agent may be considered in the presence of skin involvement without ILD. More recently, MMF has shown encouraging results in observational studies, but still needs more formal evaluation to verify if it can be considered an alternative drug to CYC or a maintenance agent such as AZA. Rituximab has provided promising results in small open-label studies and other novel therapies targeting specific molecular and cellular targets are under evaluation. Patients with rapidly progressing diffuse cutaneous SSc should be evaluated for haematopoietic stem cell transplantation.

SAT0545 Comparing Health-Related Quality of Life across Rheumatoid Arthritis, Psoriatic Arthritis and Axial Spondyloarthritis: Analyses from Certolizumab Pegol Clinical Trial Baseline Data

BackgroundInflammatory diseases such as rheumatoid arthritis (RA), psoriatic arthritis (PsA) and axial spondyloarthritis (axSpA) are associated with significant burden on patients’ (pts) function and health-related quality of life (HRQoL). However,...

Mastocytosis and related disorders

Mastocytosis represents a heterogeneous group of disorders characterized by an abnormal accumulation of mast cells in one or more organ systems. Mastocytosis is further divided into different subtypes according to the sites of involvement, laboratory findings, and degree of organ impairment. Cutaneous mastocytosis is diagnosed in the presence of skin involvement and absence of extracutaneous disease, and is most commonly seen in the pediatric population. Systemic mastocytosis, the disease form most commonly seen in adults, is characterized by the presence of multifocal, compact (dense) mast cell aggregates in the bone marrow or other extracutaneous organs. The mast cells may display atypical, often spindle-shape morphology and/or aberrant CD2 and/or CD25 expression. Elevation of serum tryptase and/or presence of KIT D816V mutation are other common findings. Systemic mastocytosis is further divided into different subtypes based on a combination of clinical features and laboratory findings. Recent studies have indicated that CD30 is frequently expressed in aggressive systemic mastocytosis and mast cell leukemia but infrequently in indolent systemic mastocytosis, and may be a useful marker for distinguishing these subtypes of systemic mastocytosis from one another. A group of related myeloid disorders, collectively termed myelomastocytic overlap syndromes, may pose diagnostic difficulty because of their significant clinical and pathologic overlap with systemic mastocytosis, and these will also be discussed in this review.

Preesclerodermia

Scleroderma, or systemic sclerosis, is a multisystem autoimmune connective tissue disorder characterized by fibrosis of the skin and internal organs and vasculopathy. The diagnosis of scleroderma is often delayed, when the disease has produced a degree of irreversible fibrosis and vasculopathy. Early diagnosis is highly important in the preescleroderma phase, before fibrosis has become established. There appears to be a therapeutic window which would allow early and aggressive treatment to be started. New classification criteria have been proposed to identify scleroderma in the earliest stages, before skin involvement has developed. Prescleroderma must be suspected whenever there is Raynaud's phenomenon, swollen edematous fingers, specific autoantibodies, and a capillaroscopic pattern of scleroderma. The incorporation of capillaroscopy and autoantibodies allows clinical forms of scleroderma to be identified, even without the presence of skin involvement.

T4 breast cancer under closer inspection: A case for revision of the TNM classification

The presence of skin involvement in breast cancer results in the classification of the tumor into the highest tumor category, and accordingly into the highest non-metastatic disease stage (current TNM classification: T4/stage III). This traditional view is no longer justifiable, as tumors that show non-inflammatory skin involvement (T4b) make up a considerably heterogeneous group with a high percentage of small-sized tumors. Classifying all lesions demonstrating this feature together results in the combination of tumors with widely differing prognostic and therapeutic implications into a single group. This violates the basic principle of the TNM concept in that only tumors exhibiting similar extension and prognosis should be grouped into one category/stage. Furthermore, the currently valid definitions of non-inflammatory skin involvement are misconceived for the substantial group of small tumors which often have ambiguous morphologic findings: the clinical classification depends on the subjective perception of the individual observer, and the pathologic staging considers histologic criteria that are not justifiable from a functional-morphological point of view. For these reasons, we strongly feel that there is a need to revise the current T4 category. We recommend that breast carcinomas currently classified as T4a-c should be eliminated from the T4 category and classified simply according to their tumor size (T1-3). The prognostically very unfavorable inflammatory carcinoma (T4d) should be maintained as the only clinicopathologic entity in the T4 category. This proposal, which will also lead to a revision of the stage III group, adheres more closely to the goals and principles of the TNM classification than do the current classification guidelines. Through the revision of the T4 category, the definitions and guidelines of inflammatory breast carcinoma should be adapted to the internationally accepted nomenclature.

Mammakarzinome mit nichtinflammatorischer Hautbeteiligung: Neue Daten revidieren das traditionelle Bild einer «klassischen» kliniko-pathologischen Entität

Historically, the presence of skin involvement results in the classification of a breast carcinoma into the highest tumor category, or accordingly into the highest non-metastatic disease stage (current TNM classification: T4/stage III). However, probably the most important criterion of the TNM classification system is the basic rule that tumors exhibiting similar extension and prognosis should be grouped into one category. Newer studies indicate the need to revise the current TNM system, since grouping all tumors demonstrating "skin involvement" together results in the combination of tumors with widely differing prognostic and therapeutic implications into a single group. Thus, our recommendation is that breast carcinomas classified under the TNM categories T4a-c should be only grouped together according to tumor size. Only the prognostically very unfavorable inflammatory carcinomas (T4d) should be maintained in the T4 category.

Effective local control and long-term survival in patients with T4 locally advanced breast cancer treated with breast conservation therapy.

The presence of skin involvement has been accepted as a relative contraindication to breast preservation because it is believed to be associated with an increased local failure rate. This study was conducted to assess the outcome of a carefully selected group of patients who presented with breast cancer involving the skin and who had breast conservation therapy (BCT) following neoadjuvant chemotherapy. Between 1987 and 1999, 33 patients with stage IIIB or IIIC breast cancer completed treatment consisting of four cycles of neoadjuvant chemotherapy, lumpectomy, radiation therapy, and consolidative chemotherapy. Clinicopathologic factors were analyzed and patients were followed for locoregional and distant recurrence. Initial median tumor size was 7 cm. All patients had skin involvement, defined as erythema, skin edema, direct skin invasion, ulceration, or peau d'orange. Following chemotherapy, median pathologic tumor size was 2 cm. Complete resolution of skin changes occurred in 29 patients (88%). At median follow-up time of 91 months in surviving patients, 26 patients (79%) were alive without evidence of disease. The 5-year, disease-free survival rate was 70%, and the 5-year overall survival rate was 78%. The actuarial ipsilateral breast cancer recurrence rate was 6% at 5 years. Patients who present with T4 breast cancer who experience tumor shrinkage and resolution of skin changes with neoadjuvant chemotherapy represent a select group of patients who can have BCT. These patients have favorable rates of long-term local control and survival. Mastectomy is not mandatory for all patients with breast cancer who present with skin involvement.

Distinct phenotype of leukemic T cells with various tissue tropisms.

There is an emerging concept, the validity of which remains to be proven, that preferential expression of selective adhesion molecules on particular T cell subsets may result in tissue-specific migration. L-selectin, cutaneous lymphocyte-associated Ag (CLA), and integrin alpha4beta7 are proposed to be involved in selective migration of T cell subsets into peripheral lymph nodes, skin, and gastrointestinal mucosa, respectively. Adult T cell leukemia (ATL) is associated with lymphoid infiltration of tissues and secondary lymphoid organs. To clarify the role of these putative homing molecules in vivo, we assessed their expression on circulating ATL cells from patients with lymph node, skin, and gut involvement. L-selectin expression was significantly higher on peripheral ATL cells in patients with lymphadenopathy than in patients without it. CLA was highly expressed on peripheral ATL cells compared with normal T cells: its expression was also significantly higher on peripheral ATL cells from patients with skin involvement compared with cells from patients without it. beta7 was particularly highly expressed on peripheral ATL cells from patients with gastrointestinal involvement. In summary, the differential expression of beta7 and beta1 on peripheral ATL cells correlates with the presence of gastrointestinal involvement. Similarly, the presence of skin involvement is associated with the expression of CLA(high)beta7low on peripheral ATL cells. These results, which are consistent with the molecules CLA and alpha4beta7 mediating preferential T cell migration to the skin and gastrointestinal mucosa, respectively, may allow for a refinement of the classification of lymphoid neoplasms on the basis of differential expression of homing molecules.