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Ziele Die Klassifikation okkludierender Vaskulopathien (auser Vaskulitis [1]) kann einige Schwierigkeiten bereiten. Erstens kann ihre Einteilung unterschiedlichen Prinzipien folgen, wie klinisch-pathologischen Befunden, Atiologie oder Pathomechanismus. Zweitens unterscheiden Autoren zuweilen nicht zwischen Vaskulitis und okkludierenden Vaskulopathien. Drittens handelt es sich bei okkludierenden Vaskulopathien um systemische Erkrankungen. Aufgrund organspezifischer Besonderheiten sind morphologische Befunde schwer zu vergleichen. Zudem konnen geringfugige Veranderungen zwar auf der Haut erkennbar sein, in anderen Organen jedoch verborgen bleiben. Unser Ziel war es, die Haut und die Subkutis als Modell und die klinisch-pathologische Korrelation als Grundlage fur die Klassifikation zu verwenden. Methoden und ergebnisse Im ersten Schritt differenzieren wir zwischen okkludierenden Vaskulopathien kleiner und mittelgroser Gefase in der Haut. Dabei konzentrieren wir uns im vorliegenden Teil auf die okkludierenden Vaskulopathien mittelgroser Gefase. Im zweiten Schritt differenzieren wir zwischen den Subtypen der Gefase. Im letzten Schritt differenzieren wir nach dem Zeitpunkt des Koagulations-/Reorganisationsprozesses sowie nach den beteiligten Entzundungszellen und Stromamerkmalen. Durch die Anwendung des gleichen Verfahrens bei den verschiedenen Entitaten und die Visualisierung der Befunde uber Barcodes konnen die Ahnlichkeiten und Unterschiede besser veranschaulicht werden, sowohl klinisch als auch histopathologisch. Schlussfolgerungen Okkludierende Vaskulopathien sind haufig keine eigenstandigen Entitaten, sondern Reaktionsmuster und Epiphanomene. Ihre Abgrenzung von den Vaskulitiden ist aufgrund der Unterschiede bei Pathogenese, therapeutischem Vorgehen und Prognose von entscheidender Bedeutung.

Aims Classifications of occluding vasculopathies (except vasculitis [1]) may exhibit some difficulties. Firstly, classifications may follow different principles, e.g. clinicopathologic findings, etiology or pathogenesis. Secondly, authors may not distinguish between vasculitis and occluding vasculopathies. Thirdly, occluding vasculopathies are systemic diseases. Organ-specific variations make morphologic findings difficult to compare. Moreover, subtle changes are recognized in the skin, but may be invisible in other organs. Our aim was to use the skin and subcutis as a tool and clinicopathological correlation as the basic process for classification. Methods and results We first differentiate in the skin between small and medium vessel occluding vasculopathies. Here we focus on medium vessel-occluding vasculopathies. In the second step we differentiate the vessel subtypes. In the final step, we differentiate according to the time point of the coagulation/reorganization process and the involved inflammatory cells/stromal features. By applying the same procedure to the various entities and visualizing the findings in the style of bar codes, the overlaps and differences in the clinical picture as well as the histopathology become more apparent. Conclusions Occluding vasculopathies are often not separate entities, but reaction patterns and epiphenomena. Distinguishing them from vasculitides is crucial because of the differences in pathogenesis, therapeutic approach and prognosis.

Zusammenfassung Hintergrund: Altere Klassifikationen fur Vaskulitiden weisen verschiedene Mangel auf. Erstens kann die Klassifikation unterschiedlichen Prinzipien folgen, wie klinisch-pathologischen Befunden, Atiologie, Pathogenese, Prognose oder therapeutischen Optionen. Zweitens unterscheiden die Autoren nicht zwischen Vaskulitis und Koagulopathie. Drittens sind Vaskulitiden systemische Erkrankungen. Aufgrund organspezifischer Variationen sind morphologische Befunde schwierig zu vergleichen. Viertens sind geringfugige Veranderungen zwar an der Haut erkennbar, verursachen jedoch an anderen Organen moglicherweise keine Symptome. Unser Ziel war es, die Haut und die Subkutis als Modell und die klinisch-pathologische Korrelation als Grundlage fur die Klassifikation zu verwenden. Methoden und Ergebnisse: Wir verwenden einen algorithmischen Ansatz mit Musteranalyse, der konsistente Angaben zu mikroskopischen Befunden ermoglicht. Zunachst differenzieren wir zwischen Vaskulitiden kleiner und mittelgroser Gefase. Im zweiten Schritt unterscheiden wir die Subtypen kleiner (Kapillaren versus postkapillare Venolen) und mittelgroser (Arteriolen/Arterien versus Venen) Gefase. Im letzten Schritt unterscheiden wir, je nach vorherrschendem Zelltyp, zwischen leukozytoklastischer und/oder granulomatoser Vaskulitis. Schlussfolgerungen: Ausgehend von der leukozytoklastischen Vaskulitis als zentralem Reaktionsmuster bei kutanen Vaskulitiden kleiner und mittelgroser Gefase konnen Zusammenhange oder Variationen darum herum angeordnet werden wie die Speichen eines Rades um die Nabe. Dies kann dazu beitragen, eine gewisse grundlegende Ordnung im vergleichsweise komplexen Bereich der kutanen Vaskulitiden einzufuhren, um zu einem besseren Verstandnis eines komplizierten Gebiets beizutragen. Summary Background: Previous classifications of vasculitides suffer from several defects. First, classifications may follow different principles including clinicopathologic findings, etiology, pathogenesis, prognosis, or therapeutic options. Second, authors fail to distinguish between vasculitis and coagulopathy. Third, vasculitides are systemic diseases. Organ-specific variations make morphologic findings difficult to compare. Fourth, subtle changes are recognized in the skin, but may be asymptomatic in other organs. Our aim was to use the skin and subcutis as a model and the clinicopathologic correlation as the basic process for classification. Methods and Results: We use an algorithmic approach with pattern analysis, which allows for consistent reporting of microscopic findings. We first differentiate between small and medium vessel vasculitis. In the second step, we differentiate the subtypes of small (capillaries versus postcapillary venules) and medium-sized (arterioles/arteries versus veins) vessels. In the final step, we differentiate, according to the predominant cell type, into leukocytoclastic and/or granulomatous vasculitis. Conclusions: Starting from leukocytoclastic vasculitis as a central reaction pattern of cutaneous small/medium vessel vasculitides, its relations or variations may be arranged around it like spokes of a wheel around the hub. This may help establish some basic order in this rather complex realm of cutaneous vasculitides, leading to a better understanding in a complicated field.

SummaryBackground Previous classifications of vasculitides suffer from several defects. First, classifications may follow different principles including clinicopathologic findings, etiology, pathogenesis, prognosis, or therapeutic options. Second, authors fail to distinguish between vasculitis and coagulopathy. Third, vasculitides are systemic diseases. Organ-specific variations make morphologic findings difficult to compare. Fourth, subtle changes are recognized in the skin, but may be asymptomatic in other organs. Our aim was to use the skin and subcutis as a model and the clinicopathologic correlation as the basic process for classification. Methods and Results We use an algorithmic approach with pattern analysis, which allows for consistent reporting of microscopic findings. We first differentiate between small and medium vessel vasculitis. In the second step, we differentiate the subtypes of small (capillaries versus postcapillary venules) and medium-sized (arterioles/arteries versus veins) vessels. In the final step, we differentiate, according to the predominant cell type, into leukocytoclastic and/or granulomatous vasculitis. Conclusions Starting from leukocytoclastic vasculitis as a central reaction pattern of cutaneous small/medium vessel vasculitides, its relations or variations may be arranged around it like spokes of a wheel around the hub. This may help establish some basic order in this rather complex realm of cutaneous vasculitides, leading to a better understanding in a complicated field.

Background Chondrodermatitis nodularis chronica helicis is a rather common condition which substantially affects quality of life causing pain and sleeping disturbances. Surgical treatment is connected with a tendency to recurrence. Mechanical pressure is probably the main etiological factor. Patients and methods A prospective study was performed with 18 patients that were offered a non-surgical treatment using a self-made bandage of foam plastic which they applied during the night. Of these, 12 performed the treatment including follow-up; 6 patients initially also consented, but they did not return after the initial visit. Results 11 patients reported substantial reduction of pain within the first month; after an average of 1.75 months they were free of pain. All 8 patients with initial sleeping problems caused by pain reported undisturbed sleep after only one month. In 11 patients the lesions receded satisfactorily, in 9 patients completely, in 2 patients leaving small asymptomatic residual lesions. Recurrences appeared in 3 patients with 2 of them having been surgically pretreated at the same location. Conclusions We recommend conservative treatment using protective padding as first line treatment for chondrodermatitis nodularis chronica helicis. The successful outcome achieved only by relief of pressure supports pressure as the main etiological factor in the development of the disease.