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The skin is one of the organs most frequently involved in vasculitides. Cutaneous vasculitis may present (1) part of a systemic vasculitis (e.g., IgA vasculitis), (2) a skin-restricted or skin-dominant variant of the corresponding systemic vasculitis without clinically apparent visceral involvement (e.g., cutaneous IgA vasculitis), or (3) a vasculitis occurring exclusively in the skin (e.g., erythema elevatun diutinum). The clinical symptoms of vasculitides are markedly determined by the size of the predominantly affected blood vessels. Systemic polyarteritis nodosa is regarded as a medium vessel vasculitis and is associated with multiple skin symptoms: (1) vasculitis of digital arteries with ensuing digital infarction, (2) livedo racemosa and subcutaneous nodules, and (3) in some patients even purpura and hemorrhagic macules due to additional small vessel vasculitis. In contrast, in its skin-restricted entity (i.e., cutaneous polyarteritis nodosa), the predominant symptoms are subcutaneous nodules surrounded by livedo racemosa, often on the lower legs. Among small-vessel vasculitides palpable purpura with predilection for the legs is a nearly pathognomonic feature of immune complex vasculitis. Variations in clinical symptoms indicate additional pathophysiological mechanisms or different vascultides: (1) ANCA-associated vasculitides often also entail nodules or sometimes livedo, (2) cryoglobulinemic vasculitis additionally may present with necrosis at cold exposed areas and involvement of vessels of various size, (3) small vessel vasculitis associated with systemic lupus erythematosus or rheumatoid arthritis shows predilection for additional sites (e.g., nailfolds) and also involvement of vessels beyond postcapillary venules, (4) recurrent macular vasculitis in hypergammaglobulinemia also occurs on dependent parts, but shows numerous small hemorrhagic macules instead of palpable purpura, (5) erythema elevatum diutinum begins with brightly red to violaceous plaques at extensor sites, followed by fibrotic nodules. Consequently, cutaneous symptoms provide pivotal clues for further diagnosis and ensuing management of vasculitides.

As a rare antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis, eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome) is characterized by asthma, severe peripheral eosinophilia and the presence of extravascular granulomas. Cutaneous involvement usually includes palpable purpura or cutaneous to subcutaneous nodes. We present the case of a 43-year-old woman with EPGA and the unusual cutaneous manifestation of livedo racemosa.

Cutaneous polyarteritis nodosa, a special form of polyarteritis nodosa (PAN) without systemic involvement, is classified as one of the ANCA-negative vasculitides of small and medium-sized vessels. It is a very rare disease with unknown etiology and occurs more commonly in women over the age of 40. Typical skin lesions are subcutaneous nodules, livedo racemosa, and ulcerations. We report the case of a 46-year-old woman presenting to our outpatient department who reported having very painful ulcerations of the lower legs with unknown origin for 6 months.

Das Cholesterinemboliesyndrom (CES) ist eine den Dermatologen kaum bekannte Multiorganerkrankung, die eine haufige und schwerwiegende Komplikation nach angiographischen und gefaschirurgischen Interventionen, aber auch nach Lysetherapien darstellt. Die Diagnose einer Cholesterinembolie wird nur in knapp einem Drittel der Falle zu Lebzeiten der Patienten korrekt gestellt und die Haufigkeit dieses Krankheitsbildes in der Regel unterschatzt. Pathognomonisch fur die Erkrankung ist die Konstellation akraler Schmerzen, schlecht heilender Ulzerationen und Nekrosen, einer Livedo racemosa bei intakten peripheren Pulsen und dem Neuauftreten oder einer Verschlechterung einer bestehenden Niereninsuffizienz und arteriellen Hypertonie. Beweisend fur die Diagnosestellung ist letztlich der Nachweis von Cholesterinkristallen durch Biopsien der betroffenen Organe. Es wird uber einen 66-jahrigen Patienten berichtet, der im Anschluss an eine Koronarangiographie mit PTCA und Stentung der A. coronaria dextra (RCA) akrale Nekrosen, Livedo racemosa und eine dialysepflichtige Niereninsuffizienz entwickelte.

Wir beschreiben den Fall einer 43-jahrigen Patientin mit einer seit Kindheit bestehenden intermittierend schmerzhaften Livedo racemosa am Rucken, die im Krankheitsverlauf zu Ulzerationen fuhrte. Die umfangreichen Untersuchungen konnten keinen Hinweis auf eine zugrunde liegende Erkrankung erbringen, wobei initial und im Verlauf insbesondere kein Anhalt fur ein Sneddon-Syndrom bestand. Mittels konserativer und chirurgischer Masnahmen konnte zunachst eine Abheilung der Ulzerationen erreicht werden, diese rezidivierten jedoch in der Folgezeit. Eine zyklische Therapie mit Iloprost-Infusionen fuhrte im vorliegenden Fall zu einer langfristigen Abheilung der Ulzerationen und zum Sistieren der Schmerzsymptomatik. Eine derartige Behandlung ist bisher fur diese Erkrankung in der wissenschaftlichen Literatur noch nicht beschrieben.

Classic variants of cutaneous borreliosis are erythema chronicum migrans (ECM), lymphadenosis benigna cutis (LBC) and acrodermatitis chronica atrophicans (ACA). Other dermatoses have been reported in the literature as possibly linked to borreliosis. A 59-year old female patient was seen in the late phases of cutaneous borreliosis with histologically confirmed ACA. In addition, prominent livedo racemosa was seen on the legs, also showing tissue changes similar to those of ACA. Borrelia burgdorferi infection was serologically confirmed by the presence of anti-IgM and anti-IgG antibodies. The clinical spectrum of late cutaneous borreliosis should be enlarged to include livedo racemosa.

Es wird uber einen Fall von Kalziphylaxie bei einer 40jahrigen Patientin mit terminaler Niereninsuffizienz berichtet. Nach 22 Jahren Hamodialyse entwickelte die Patientin multiple Herde von Livedo racemosa sowie schmerzhafte Nekrosen und Ulzera an der Hufte und den unteren Extremitaten. Als Ursache lies sich radiologisch eine Kalzinose peripherer Arterien, insbesondere der Becken- und Oberschenkelarterien und der Interdigitalarterien der Hande, sowie histologisch eine Fibrose und Kalzinose kleiner subkutaner Arterien aufzeigen. Mittels transkutaner Sauerstoffpartialdruckmessungen konnte eine generalisierte kutane Mikroangiopathie nachgewiesen werden. Laborchemisch bestand ein moderat ausgepragter sekundarer Hyperparathyreoidismus mit masiger Erhohung des Kalzium-Phosphat-Produkts. Neben der Hamodialysetherapie wurde der Versuch einer medikamentosen Verbesserung der Mikrozirkulation unternommen. Der Verlauf war durch ein protrahiertes Abheilen der Ulzera und das Auftreten neuer kutaner Nekrosen gekennzeichnet. Die Kalziphylaxie ist eine seltene Spatkomplikation bei Patienten mit fortgeschrittener, meist terminaler Niereninsuffizienz, die durch die Histologie gesichert wird und oft, aber nicht notwendigerweise, mit einem gestorten Kalzium- und Phosphatstoffwechsel sowie mit leicht erhohten Parathormonspiegeln assoziiert ist. Die Kalziphylaxie wird als Sonderform der metastatischen Kalzinose eingestuft.

A 64-year-old male patient presented with painful ulcerations and livedo racemosa of both lower limbs. He had a history of cerebral and myocardial infarctions. Dermatohistologic findings and laboratory tests of the patient's coagulation system revealed the diagnosis of livedoid vasculopathy with heterozygous factor V Leiden mutation and sticky platelet syndrome type II. Systemic treatment with acetylsalicylic acid and heparin as well as topical therapy with disinfectant and granulation-inducing agents resulted in improvement of the skin lesions.

A case report is presented to illustrate the cutaneous manifestations in essential thrombocythaemia. Knowledge of the related skin lesions may lead to earlier detection of this myeloproliferative disorder. Treatment may prevent severe haemorrhagic or thrombotic complications. Erythromelalgia is causally related to thrombocythaemia; prompt relief of painful symptoms after treatment with aspirin is typical.

A 61-year-old patient with life-threatening angioneurotic oedema was found to have an acquired C1-inhibitor (C1-INH) deficiency. In addition to lowered serum levels of C1-INH (both protein concentration and enzymatic activity), C2, C4 and CH50, which are characteristic for the hereditary form of angioneurotic oedema, markedly lowered C1q was found, which is typical for the acquired form. There were no antibodies against C1-INH. Repeated thorough examinations disclosed no neoplasm, though the presence of neoplasm has often been reported to be associated with the acquired C1-INH deficiency. However, the patient showed persistent paraproteinaemia and paraproteinuria and developed livedo reticularis. Treatment with danazol resulted in a rise in the complement fraction levels and cessation of angioneurotic oedema. Paraproteinaemia and livedo reticularis persisted unchanged.

In anticardiolin syndrome (ACS) a typical antibody constellation is associated with thrombotic and hematologic disorders. Furthermore, recurrent abortion, cerebral ischemia and different skin disorders occur. We report the case of a 29-year-old female suffering for the first time from painful, necrotic deep ulcers on the upper and lower legs and livedo racemosa on the arms as a rare example of a merely cutaneous manifestation of ACS with no demonstrable underlying disease. After systemic treatment with high-dose methylprednisolone, azathioprin and hydrocolloid dressings, healing of the scar tissue occurred. Simultaneously, a maintenance dose of acetylsalicylic acid (100 mg/day) was administered. So far, neither cutaneous relapse nor other signs of ACS have occurred.