ISSN:1018-8665    

DOI:10.1159/000246979

出版商:S. Karger AG    

年代:1994

数据来源: Karger

ISSN:1018-8665    

DOI:10.1159/000246980

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

Primary cutaneous lymphomas represent a heterogeneous group of malignant B- and T-cell lymphomas. In the last two decades, the diagnostic criteria, classification and terminology of these disorders have changed enormously. A historical overview of the subsequent concepts on the classification of this group of disorders is presented. Disadvantages of currently available classification schemes are discussed, and a proposal for a new classification for this group of disorders is formulated.

ISSN:1018-8665    

DOI:10.1159/000246981

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

Recent advances in molecular and cell biology of the cutaneous basement membrane zone have drastically increased our knowledge of the pathomechanisms underlying skin blistering disorders. Defects in genes coding for the structural proteins of the basement membrane zone have been defined in epidermolysis bullosa (EB), and abnormal expression of the structural proteins in some of its subtypes. The underlying cause for several subtypes of dystrophic EB are mutations in the gene for collagen VII, COL7A1. In vitro studies utilizing cells derived from the skin of such patients have helped understanding the pathogenetic pathways that lead from the mutation to the symptom, skin blistering. The data accumulated from analyses of the genetic disorders will also yield indirect information on the normal physiology of the skin and be highly relevant for the clarification of the etiopathogenesis of acquired blistering diseases.

ISSN:1018-8665    

DOI:10.1159/000246983

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

The clinical, biochemical and molecular characteristics of Ehlers-Danlos syndrome type IV are reviewed. The numerous clinical manifestations result from the generalized and excessive tissue fragility characteristic for this disorder. The variability in cutaneous involvement may hamper recognition of the condition and requires careful clinical examination. Clinical diagnosis can be confirmed by demonstration of a collagen type III defect. The severity of the phenotype appears to be related mainly to the intrinsic stability of mutant type III collagen protein and its disturbing effect on normal collagen fibrillogenesis in the extracellular matrix.

ISSN:1018-8665    

DOI:10.1159/000246984

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

Pemphigus remains the archetype of the autoimmune bullous diseases with circulating and skin antibodies in patients as well as in animal models. Pemphigus antigens are desmosomal-associated glycoproteins of 130 kD in pemphigus vulgaris (desmoglein 3) and of 160 kD (desmoglein 1) in pemphigus foliaceus (fogo selvagem in Brazil, pemphigus seborrheicus in other countries). Pemphigus may be associated to drugs and/or immune system related tumors (e.g. thymoma). Two new forms of pemphigus have been described: IgA pemphigus and paraneoplastic pemphigus. Targets of the autoantibodies are various desmosomal and cellular adhesion molecules. Even if each main pemphigus variant corresponds to specific antibodies, association of various antibodies may be found, leading to the concept of dysimmunoreactivity.

ISSN:1018-8665    

DOI:10.1159/000246985

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

The palmoplantar skin reacts monotonously to acute causes with vesicles or pustules and to chronic or chronically recurrent noxae with hyperkeratosis. Vesicular reactions are mostly triggered by eczematogens and fungi. Among the primary pustuloses, pustular psoriasis of the palms and soles is the dominating one, while the secondary pustuloses stem from infections of vesicular reactions with pyogens. Hyperkeratotic reactions may be caused directly by mechanical irritations or dehydration of the skin but also secondarily by chronic or chronically recurrent vesicular and pustular reactions.

ISSN:1018-8665    

DOI:10.1159/000246986

出版商:S. Karger AG    

年代:1994

数据来源: Karger

摘要:

Psoriatic arthritis is an inflammatory arthropathy associated with psoriasis and characterized by absence of rheumatoid factor. Any form of psoriasis can be complicated by joint inflammation. Four groups have been identified: pauciarticular psoriatic arthritis, psoriatic spondylitis, symmetrical polyarticular psoriatic arthritis and arthritis mutilans. The etiology of psoriatic arthritis remains unclear. Genetic, immunologic and environmental factors are thought to be important in the development of the disease. The role of cytokines and adhesion molecules in the mechanism of joint destruction is highlighted, and recent therapeutic strategies are discussed.

ISSN:1018-8665    

DOI:10.1159/000246987

出版商:S. Karger AG    

年代:1994

数据来源: Karger

ISSN:1018-8665    

DOI:10.1159/000246988

出版商:S. Karger AG    

年代:1994

数据来源: Karger

ISSN:1018-8665    

DOI:10.1159/000246989

出版商:S. Karger AG    

年代:1994

数据来源: Karger