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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. These guidelines focus on patients of all age groups and genders exhibiting skin lesions caused by dermal lymphostasis. Specific recommendations are provided with respect to the diagnosis and differential diagnosis of the various clinical manifestations. In this context, comorbid skin diseases such as atopic dermatitis, psoriasis, hidradenitis suppurativa, urticaria, and contact dermatitis will be highlighted, including their treatment and associated specific risks. Several other relevant current guidelines are referenced as regards the distinction from and treatment of common cofactors and comorbid conditions.

Das Ziel dieser S1-Leitlinie ist es, aktuelles Wissen uber dermatologisch relevante Krankheitsbilder bei lokal begrenzter dermaler Lymphostase an allen Lokalisationen des Hautorgans zu vermitteln, um diese fruhzeitig zu erkennen, diagnostisch zu sichern und gezielt zu behandeln. Wann immer moglich, sollte diese Therapie anhand klar definierter Algorithmen stadiengerecht erfolgen. Die im klinischen Alltag haufig auftauchenden differenzialdiagnostischen und therapeutischen Fragen lassen eine aktuelle Leitlinie notwendig erscheinen. Diese Leitlinie fokussiert auf Patienten jeglichen Alters und Geschlechts mit Hautveranderungen, die im kausalen Zusammenhang mit dermaler Lymphostase stehen. In konkreten Handlungsempfehlungen werden die Diagnostik und Differenzialdiagnostik der verschiedenen Manifestationsformen bei gleichzeitig auftretender dermatologischer Komorbiditat wie beispielsweise atopische Dermatitis, Psoriasis vulgaris, Acne inversa, Urtikaria, Kontaktekzeme sowie ihre Therapien unter Berucksichtigung besonderer Risiken veranschaulicht. Fur die Abgrenzung und Behandlung haufiger therapierelevanter Kofaktoren und Komorbiditat wird auf mehrere andere hierfur relevante aktuelle Leitlinien verwiesen.

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. These guidelines focus on patients of all age groups and genders exhibiting skin lesions caused by dermal lymphostasis. Specific recommendations are provided with respect to the diagnosis and differential diagnosis of the various clinical manifestations. In this context, comorbid skin diseases such as atopic dermatitis, psoriasis, hidradenitis suppurativa, urticaria, and contact dermatitis will be highlighted, including their treatment and associated specific risks. Several other relevant current guidelines are referenced as regards the distinction from and treatment of common cofactors and comorbid conditions.

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. Diese Leitlinie fokussiert auf Patienten jeglichen Alters und Geschlechts mit Hautveränderungen, die im kausalen Zusammenhang mit dermaler Lymphostase stehen. In konkreten Handlungsempfehlungen werden die Diagnostik und Differenzialdiagnostik der verschiedenen Manifestationsformen bei gleichzeitig auftretender dermatologischer Komorbidität wie beispielsweise atopische Dermatitis, Psoriasis vulgaris, Acne inversa, Urtikaria, Kontaktekzeme sowie ihre Therapien unter Berücksichtigung besonderer Risiken veranschaulicht. Für die Abgrenzung und Behandlung häufiger therapierelevanter Kofaktoren und Komorbidität wird auf mehrere andere hierfür relevante aktuelle Leitlinien verwiesen.

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. Observation: A 78-year-old woman is presented with a generalized eruption of blisters leaving behind scars that developed after a 7-year-long history of mild circumscribed recurrent blisters and scarring eruptions that had been diagnosed previously as Brunsting-Perry type of cicatricial pemphigoid. Immunofluorescence antigen mapping disclosed the blister formation above the lamina densa. Electron and immunoelectron microscopy using a preembedding immunogold technique revealed blister formation and antibody binding within the lamina lucida, predominantly below the subbasal dense plate. Conclusions: The clinical features of disseminated blistering followed by scarring, the immunofluorescence antigen mapping, and the electron and immunoelectron microscopic findings in our case for the first time clearly prove the existence of a disseminated cicatricial pemphigoid. (Arch Dermatol. 1995;131:580-585)

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. These patients showed a progressive course with multiple organ and diffuse skin involvement with disseminated telangiectasia.

• 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 lesional 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. Immunofluorescence antigen mapping using cryostat sections of a spontaneous blister and antisera against defined basement membrane components localized the bullous pemphigoid antigen and type IV collagen in the roof of the blister. This dermal type of blister formation was confirmed by electron microscopy, which showed the cleavage level below the lamina densa. In direct immunoelectron microscopy, granular deposits of C3 and IgG were found attached to and just beneath the lamina densa in a pattern identical to the distribution of anchoring fibrils. These findings are diagnostic of acquired epidermolysis bullosa, a blistering disease that has much more clinical heterogeneity than previously suggested. ( Arch Dermatol. 1991;127:391-395)