S1-Leitlinie - Dermatosen bei dermaler Lymphostase.

Das Ziel dieser S1-Leitlinie ist es, aktuelles Wissen über dermatologisch relevante Krankheitsbilder bei lokal begrenzter dermaler Lymphostase an allen Lokalisationen des Hautorgans zu vermitteln, um diese frühzeitig zu erkennen, diagnostisch zu sichern und gezielt zu behandeln. Wann immer möglich, sollte diese Therapie anhand klar definierter Algorithmen stadiengerecht erfolgen. Die im klinischen Alltag häufig auftauchenden differenzialdiagnostischen und therapeutischen Fragen lassen eine aktuelle Leitlinie notwendig erscheinen.

S1 Guidelines - Dermatoses associated with dermal lymphostasis.

The objective of the present S1 guidelines is to present current knowledge about dermatologically relevant diseases associated with localized dermal lymphostasis, thus facilitating their early detection, diagnostic workup, and targeted treatment. Whenever possible, treatment should be based on stage-appropriate and clearly defined algorithms. The numerous issues regarding differential diagnosis and treatment clinicians are confronted with in everyday clinical practice seem to warrant the publication of up-to-date guidelines.

Localized cicatricial pemphigoid of the Brunsting-Perry type with transition into disseminated cicatricial pemphigoid. Report of a case proved by preembedding immunogold electron microscopy.

Background: In 1979, Provost described two patients with the clinical features of disseminated cicatricial pemphigoid for the first time. Until now, only four additional cases of disseminated cicatricial pemphigoid have been described. Existence of diagnosis of disseminated cicatricial pemphigoid has been discussed controversially because in four cases investigated by electron microscopy the blister formation was found below the lamina densa, which is indicative of an epidermolysis bullosa acquisita.

[Localized cicatricial bullous pemphigoid of the Brunsting-Perry type].

Localized cicatricial pemphigoid of the Brunsting-Perry type is a very rare bullous condition, which has so far been reported in 51 cases. It is characterized by scarring blisters confined to the head, scalp and neck. Diagnosis can be difficult because of the discrete skin lesions, often repeatedly false-negative direct immunofluorescence, and the absence of circulating antibodies.

Acquired epidermolysis bullosa with the clinical feature of Brunsting-Perry cicatricial bullous pemphigoid.

A 56-year-old woman with the typical clinical feature of cicatricial bullous pemphigoid of the Brunsting-Perry type was studied. Histologic examination of a lesion skin biopsy specimen demonstrated a subepidermal blister. Direct immunofluorescence microscopy revealed linear deposits of IgG, IgM, and C3 located on both the roof and the floor of the blister.

Clinical association of autoantibodies to fibrillarin with diffuse scleroderma and disseminated telangiectasia.

Circulating autoantibodies against a variety of nuclear and nucleolar antigens are characteristic serologic findings in systemic scleroderma. Some of these antibodies correlate with clinical subsets of the disease. We describe three patients with systemic scleroderma and high autoantibody titers against U3 ribonucleoprotein-associated fibrillarin, a recently identified 34 kD nucleolar protein.

[The basement membrane and its significance in congenital and acquired diseases of the skin].

Basal membranes are an ubiquitous component of all human organs and fulfil a large variety of functions. They separate epithelial from mesenchymal tissue and control the passage of substances, of inflammatory as well as tumor cells. They form the extracellular cytoskeleton, regulate growth processes, and play an important part in wound healing.