Biomedical subjects

S Dommann

Publications and source records attributed to S Dommann.

6 recordsLinked to original sources

PubMed1998

Histopathologic and ultrastructural study of lupus-like skin lesions in a patient with Bloom syndrome.

The histopathology of the lupus-like skin lesions associated with Bloom syndrome has been sporadically described. Skin biopsies from a 2-year-old boy with the classical features of Bloom syndrome, including lupus-like skin lesions, demonstrated marked interface changes with basal liquefaction degeneration, a moderate superficial mononuclear infiltrate, pigmentary incontinence, and capillary dilation in the papillary dermis. Immunophenotyping of the dermal infiltrate revealed predominance of T-cells. Basement membrane thickening on periodic acid-Schiff examination was not seen. Direct immunofluorescence failed to demonstrate deposits of immunoglobulin other than nonspecific IgM deposition along the basement membrane zone of lesional skin. Ultrastructurally, the most striking findings were disintegration of basal cell cytoplasm and tubuloreticular inclusions in vascular endothelia. Taken together, the histologic and ultrastructural features of lupus-like lesions associated with Bloom syndrome mimic those of cutaneous lupus erythematosus, with the exception of paucity of immune deposits at the dermoepidermal junction.

Basement Membrane↗

PubMed1995

New perspectives in experimental and clinical research for cutaneous T cell lymphomas.

Cutaneous T cell lymphomas (CTCL) are lymphoproliferative disorders, which can be classified by an adaptation of the Kiel classification. The most common CTCL, Mycosis fungoides (MF) and Sézary syndrome (SS), are monoclonal T helper memory lymphomas. They belong to the group of peripheral T cell lymphomas. However, CTCL includes other disease entities such as granulomatous slack skin, pagetoid reticulosis, and lipotropic CTCL. Polymerase chain reaction of the T cell receptor (TCR)-gamma and screening of the PCR products for sequence-specific mobility in acrylamide gels has increased the detection limit for clonal T cells in the skin. This method will help to define the relationship of CTCL to other lymphoproliferative disorders and may contribute to the early diagnosis of CTCL. In SS, the malignant CTCL clone secretes a T helper-2 cytokine pattern, which might be responsible for the systemic immunosuppression in CTCL patients. New treatment modalities (IL-12) might correct these immunologic abnormalities. Cutaneous B cell lymphomas mostly are follicular center cell derived and usually have a good prognosis. Therapy of cutaneous lymphomas should take into account prognostic factors, the most reliable of which in CTCL is the tumor mass expressed by the tumor burden index.

Cytokines↗

PubMed1995

Necrotizing herpes zoster mimicking relapse of vasculitis in angioimmunoblastic lymphadenopathy with dysproteinaemia.

An 81-year-old man presented with a generalized maculopapular rash, lymphadenopathy, conjunctivitis and arthritis. Vasculitis was confirmed by skin biopsy and by direct immunofluorescence, which showed perivascular C3 and granular IgM accumulation. Histology of an inguinal lymph node was diagnostic for angioimmunoblastic lymphadenopathy with dysproteinaemia (AILD), and this was confirmed by the finding of hypergammaglobulinaemia and elevated IgE levels. Immunohistology on a lymph node biopsy showed a T-helper cell (CD4) infiltrate expressing the interleukin (IL)-2 receptor alpha and beta chains. While receiving prednisone 100 mg/day, the patient developed new lesions, mimicking a relapse of vasculitis, which were subsequently shown to be necrotizing herpes zoster. Serum IL-2 and IL-6 levels were elevated. To our knowledge, this is the first report of simultaneous elevation of IL-2 and IL-6 in AILD: IL-2 may be involved in proliferation of the malignant cell clone, and IL-6 in the pathogenesis of both the vasculitis (via endothelial cell activation) and the hypergammaglobulinaemia.

Aged↗

PubMed1995

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↗

PubMed1994

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↗

PubMed1993

[Malignant lymphoma of the skin].

Extranodal non-Hodgkin lymphomas preferentially involve the skin. Among them, 65% are T-cell lymphomas, 25% B-cell lymphomas and about 10% rare variants or nonclassifiable lymphomas. Mycosis fungoides is the most common form of low-grade malignant peripheral cutaneous T-cell lymphoma (CTCL): Lymphomas originating from follicular center cells are the most common types of cutaneous B-cell lymphomas (CBCL). Only 15 to 25% of cutaneous lymphomas show extracutaneous manifestations at time of diagnosis. The prognosis is relatively good, since the average survival time from diagnosis is 12 to 14 years. For making the diagnosis, clinical, histo- and cytomorphological parameters often have to be supplemented by phenotypic, genotypic and molecular biologic techniques in order to establish the correct diagnosis. An important problem still is the early diagnosis and early evaluation of prognostic parameters in CTCL. The therapeutic approach is control of the disease according to stage and prognostic parameters.

Combined Modality Therapy↗