Single-organ cutaneous small-vessel vasculitis according to the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides: a study of 60 patients from a series of 766 cutaneous vasculitis cases.

OBJECTIVE: Cutaneous vasculitis (CV) encompasses a wide group of entities characterized by inflammation of skin blood vessels. The term single-organ vasculitis was recently coined by the 2012 Chapel Hill Consensus Conference (CHCC) to define vasculitis affecting a single organ. To our knowledge there are no published reports on single-organ cutaneous small vessel vasculitis (SoCSVV). Our aim was to characterize this entity from a wide series of patients with CV. METHODS: We analysed cases of SoCSVV from a series of 766 patients with CV from a single university referral centre. According to 2012 CHCC, the following conditions were required to define SoCSVV: (i) skin biopsy showing characteristic leucocytoclastic vasculitis and (ii) vasculitis limited to skin. RESULTS: We included 60 patients (26 women and 34 men) with a mean age of 56 years. The main precipitating factors for SoCSVV were drugs [26 patients (52%)] and previous infection [17 patients (34%)]. The main clinical manifestations were palpable purpura (81.7%) and fever (18.3%). The most frequent laboratory findings were leucocytosis and elevated ESR. Nearly one-quarter of patients with SoCSVV required pharmacological therapy. Corticosteroids (15%) and NSAIDs (13.3%) were the main agents prescribed. After a median follow-up of 4 months, complete recovery was observed in all the patients, although relapses occurred in 8% of patients. CONCLUSION: SoCSVV defined according to the 2012 CHCC may be considered a benign disease usually associated with drugs and/or a previous infection.

Vasculitis in childhood ­ a dermatological approach.

Vasculitis, an inflammatory condition affecting the blood vessels, may be restricted to a single organ or involve several organ systems. The size of the involved vessels is an important criterion for categorization of vasculitides, which is a prerequisite for rapid diagnosis and initiation of treatment. In pediatric patients, this particularly applies to Kawasaki disease. However, making the diagnosis can be challenging for dermatologists as skin involvement may be variable and non-specific. In contrast, Henoch-Schönlein purpura (IgA vasculitis) presents with the classic picture of palpable purpura. It predominantly affects postcapillary venules frequently following upper respiratory tract infections. Severe organ involvement is relatively rare in children and the prognosis is good. As renal involvement may occur during the course of disease, continuous monitoring is required. Acute hemorrhagic edema of infancy is considered as a distinct type of immune complex vasculitis and is characterized by a triad of fever, edema and rosette-shaped purpura. The clinical course of this rare disease is usually benign and self-limited. Due to the variability of clinical symptoms and manifestations, management of childhood vasculitides represents a special challenge requiring interdisciplinary collaboration. Dermatologists should be aware of their important role especially for making an early diagnosis.

Acute hemorrhagic edema of infancy with abdominal pain and elevated transaminases.

Acute hemorrhagic edema of infancy is a rare type of leukocytoclastic vasculitis characterized by a triad of fever, edema, and rosette-shaped purpura, mainly over the face, auricles, and extremities in a nontoxic infant. Visceral involvement is infrequent in acute hemorrhagic edema of infancy. We report a case of acute hemorrhagic edema of infancy with abnormal liver function tests and abdominal pain.

Clinical study on single-organ cutaneous small vessels vasculitis (SoCSVV).

Leukocytoclastic vasculitis (LCV) is a heterogenous group of disorders that may manifest as a mild disease isolated to the skin or be a part of life-threatening systemic vasculitis. According to the 2012 Chapel Hill Consensus Conference nomenclature, patients presenting symptoms of LCV confined only to the skin should be defined as suffering from a single-organ cutaneous small vessel vasculitis (SoCSVV). SoCSVV is a benign disease with a good clinical outcome but with a significant risk of relapse and skin ulcer formation.The aim of the current study was to characterize SoCSVV and to identify factors that may be associated with the risk of recurrence and skin ulcers.Medical records of patients with LCV hospitalized at the Department of Dermatology at University Hospital in Cracow in the years 2010 to 2015 were analyzed.A total of 24 patients fulfilled criteria of SoCSVV. Drugs and preceding infections were identified as precipitating factors in 40% and 20% of cases, respectively. Skin lesions other than palpable purpura (i.e., macules, urticarial vasculitis, or ulcers) were identified in almost half of the patients. Interestingly, the presence of macules independently increased the risk of skin ulcer formation (odds ratio = 16; 95% confidence interval: 1.5-176.6; P = 0.0075) in the multivariate logistic regression analysis. One-quarter of patients with SoCSVV experienced relapse during the 6-month follow-up. The greater number of affected skin areas was an independent risk factor of recurrence (odds ratio = 5; 95% confidence interval: 2-45; P = 0.02).SoCSVV was usually associated with drugs and preceding infections. The disease relapses in approximately one-quarter of the patients. The more severe the skin involvement in the course of SoCSVV, the higher is the risk of recurrence.

Anti-neutrophil cytoplasm antibodies in patients with ACR criteria for polyarteritis nodosa: help for systemic vasculitis classification?

The american college of rheumatology (ACR) proposed in 1990 revised clinical criteria for systemic vasculitis classification to define homogeneous group of patients for clinical trials. However, microscopic polyarteritis (MPA) was not clearly identified from polyarteritis nodosa (PAN). Since anti-neutrophil cytoplasm antibodies (ANCA) are markers of disease activity of small vessel vasculitides including MPA, we tested the clinical significance of ANCA in 24 patients with PAN according to the ACR 1990 criteria. Two of 24 patients had ANCA, as defined by indirect immunofluorescence on normal human neutrophils, antigen-specific ELISA and Western blot analysis. However, they exhibited histologically proven small vessel but not medium vessel vasculitis. Furthermore, they had neither artery microaneurysms nor large organ injury consequent upon large vessel occlusion. Although they satisfied ACR criteria for PAN, they probably were misclassified and should be considered as MPA. We conclude that: (i) ANCA are not found in patients with classical PAN in the absence of MPA features; (ii) caution should be exercised when defining PAN according to the ACR 1990 criteria; (iii) ANCA may help systemic vasculitis classification.

Pulmonary angiitis and granulomatosis revisited.

Re-examination of the pathologic and clinical features of the entities traditionally classified under the heading "pulmonary angiitis and granulomatosis" indicates that there is little advantage in retaining this artificial category and that these entities should be considered variants of diseases to which they are actually related. Wegener's granulomatosis and allergic angiitis and granulomatosis appear to be examples of true systemic vasculitides in which the lung is a predominant but not the only or even the most important site of involvement. Wegener's granulomatosis may manifest with involvement limited to lung, a form that has been called limited Wegener's; however, many or most such cases progress to classic disease involving kidney and often upper respiratory tract. Similarly, Wegener's granulomatosis may present with disease limited initially to the upper respiratory tract (a form of midline granuloma); this process may also spread to involve lung and kidney. It seems unlikely that limited Wegener's is truly a separate disease category. Evaluation of the pathologic and clinical features of necrotizing sarcoid granulomatosis indicate that it very much resembles ordinary sarcoid in most histologic features, in the nature of extrapulmonary involvement, and in its clinical course and that it probably corresponds to the clinical--radiographic entity of nodular sarcoid. Lymphomatoid granulomatosis appears to have little relationship to the other members of the angiitis and granulomatosis group; its behavior and histologic features are those of a lymphoproliferative disorder that in most cases is or becomes histiocytic lymphoma. Some cases of so-called benign lymphocytic angiitis also fall into this category; the remainder appear to represent a variety of completely unrelated pathologic processes. Last, bronchocentric granulomatosis is most commonly one of the histologic manifestations of allergic bronchopulmonary aspergillosis, although it is likely that other agents or processes produce the same histologic pattern. Although the presence of a common set of pathologic features makes the concept of angiitis and granulomatosis attractive from a morphologic point of view, there is minimal clinical similarity among them, and these diseases appear to be totally separate entities.

The cutaneous neutrophilic vascular injury syndromes: a review.

The skin manifestations of vasculitis reflect injury by all of the classic immune reactions of Gell and Coombs. As the skin affords a window of opportunity for the clinician to obtain tissue for diagnostic purposes in patients with systemic vasculitic syndromes, a thorough understanding of the dermatopathologic manifestations of those systemic diseases is a considerable asset to the practicing pathologist. This review focuses on those systemic diseases that can provoke a small vessel neutrophilic injury pattern in the skin and provides clues by which these diseases can be separated from each other and from their innocuous mimics in which cutaneous vascular injury is the only significant consequence.

Cutaneous necrotizing venulitis.

The term vasculitis describes a variety of inflammatory changes in vessels. The most common type to affect the skin is a necrotizing venulitis associated with a neutrophilic polymorphonuclear leukocyte infiltrate. The most frequent presentation is palpable purpura on the lower extremities, but the lesions may include urticaria and ulcers. A diagnosis of cutaneous necrotizing venulitis obliges the physician to search for the cause, associated diseases, and the extent of the vascular involvement. Antigens, either in circulating immune complexes or as haptens bound to vessel proteins, are presumed to trigger immmune responses. The differentiation of large from small vessel disease and the prediction of systemic involvement may be difficult in a specific patient. Treatment, other than removal of the antigen, is theoretic or anecdotal.

[Infection and vascular purpura]. / Infection et purpura vasculaire.

Palpable purpura is the hallmark of cutaneous vasculitis. Small-vessel vasculitis is a common vasculitis manifestation associated with acute or chronic infection. It is also characteristic of a systemic disease whether infectious or not. The pathogenic mechanisms appear to be complex: immune complex formation, vessel damage or altered vessel function mediated directly by infectious agents, humoral or cellular immunologic response. It is also a reaction to mixed cryoglobulinemia. Diagnosis of cutaneous vasculitis is simple (palpable purpuric eruption, nodules, vesiculobullous lesions, ulcerations), but etiological investigation is often difficult because the infectious origin is only rarely demonstrated. This type of purpura occurs in bacterial endocarditis and therefore blood cultures must be performed in any febrile patient particularly in the presence of a cardiac murmur. In fact the viral, parasitic or bacterial infectious origin is demonstrated in less than 30% of the cases of leucocytoclastic vasculitis. While focal sepsis is often found and its eradication should be followed-up, its role has not been proven particularly as antibiotics alone themselves can cause hypersensitivity vasculitis. Finally, mention must be made of virus induced vasculitis (B and C hepatitis, cytomegalovirus, parvovirus), antiviral treatment which permits better control of vasculitis.

[Polyarteritis]. / Poliarteriti.

Polyarteritis is an uncommon childhood disease. The different forms of polyarteritis are described. Polyarteritis nodosa is a rare multisystemic disease characterized by high fever, calf pain and subcutaneous nodules. Infantile polyarteritis nodosa shows a peculiar predilection for the coronary arteries and has the worst prognosis. Polyarteritis cutaneous is the localized and mild form of polyarteritis. Hypersensitivity angiitis is described as a leukocytoclastic vasculitis identified by palpable purpura.

[Cutaneous leukocytoclastic vasculitis as a marker of systemic diseases]. / Vasculitis leucocitoclástica cutánea como marcador de enfermedades sistémicas.

Fifty-eight patients with cutaneous leukocytoclastic vasculitis were evaluated. The most frequent cutaneous manifestation was purpura, followed by palpable purpura. Vascular inflammation was confirmed by skin biopsy: two distinct patterns of cellular infiltrate were found, a neutrophilic-predominant and a mononuclear-predominant type, as a manifestation of the dynamic evolution of the process. On the basis of the associated disease we grouped our cases as follows: autoimmune disease, reaction, malignancy, infection and idiopathic.

[Hypersensitivity angiitis, Henoch-Schönlein purpura].

Pathogenesis and treatment of hypersensitivity angiitis and Henoch-Schönlein purpura were summarized. Both diseases were included in the category of "leukocytoclastic vasculitis". Hypersensitivity angiitis was frequently associated with drug or infectious exposure and the involvement of venules and capillaries. Many patients with this disease have detectable autoantibodies to neutrophil cytoplasmic antigens (ANCA) typically reactive with myeloperoxidase. Henoch-Schönlein purpura was characterized by vasculitis in multiple organs such as the skin, joints, gastrointestinal tract and kidneys. Henoch-Schönlein purpura nephritis revealed marked deposition of IgA (mainly IgA1) and C3 in the glomerular mesangial areas and capillary walls by immunofluorescence. Combinations of intensive plasma exchange, steroids and cyclophosphamide were effective for patients with both diseases.

Shape and configuration of skin lesions: targetoid lesions.

What is probably the first description of targetoid or iris lesions, as they appear in erythema multiforme (EM), can be found in Thomas Bateman's 1836 textbook "Practical Synopsis of Cutaneous Diseases According to the Arrangement of Dr. Willan." EM was initially described by Bateman and later by von Hebra as an acute self-limiting skin disease, symmetrically distributed on the extremities with typical concentric "targetoid" or "iris" lesions, and often recurrent. Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) were added to this syndrome later. A newer classification has created two disease spectra: EM consisting of EM minor and EM major (or bullous EM), and SJS and TEN. EM minor and EM major are often recurrent, postinfectious (especially after herpes and mycoplasma) disorders with low morbidity and almost no mortality. SJS and TEN are usually severe drug-induced reactions with high morbidity and poor prognosis. The target lesions found in each form of the disease are described and defined. Although the term "target lesion" originated from the description of EM and despite its being the dominant lesion in this disease, it is not pathognomonic for EM, and these lesions can sometimes appear in other diseases. Short descriptions of these other diseases are presented.

Retrospective analysis of adult patients with cutaneous leukocytoclastic vasculitis.

A retrospective analysis was conducted on 93 adult patients with cutaneous leukocytoclastic vasculitis from St. Vincent's Hospital Melbourne to determine the classification, aetiology, severity and prognosis of this population of patients. We developed a new classification system for the purposes of our study based on modifications to the Chapel Hill Consensus Conference definitions for vasculitic syndromes. The results of our study indicate that an obvious cause was not found in 44.1% of patients. Of the patients with secondary vasculitis, the commonest causes were drugs and infections, accounting for a total of 40.9% of patients. Extracutaneous involvement was found in 39.8% of patients. Patients with symptoms resolving in less than 3 months accounted for 59.1% of the population, whereas 24.8% of patients had either symptoms lasting three or more months or evidence of recurrent symptomatology. There were 6 deaths (6.91%) and the rest were lost to follow up. The majority of patients in this retrospective series were classified as having hypersensitivity vasculitis, which is a relatively benign disorder limited mostly to skin with a low incidence of extracutaneous involvement (15.8%). Nevertheless, evidence of systemic involvement or sepsis need to be excluded as this may have important implications for patient treatment and outcome.

Cutaneous vasculitis: a review.

As the skin is commonly involved in systemic vasculitic disorders as well as those hypersensitivity states whose expression is largely skin-confined, cutaneous vasculitic lesions offer a window to diagnosis and a ready source of accessible tissue for biopsy. In this review, we discuss the pathologic manifestations of chronic vasculitic syndromes such as granuloma faciale and erythema elevatum diutinum; IgA-associated vasculitis including Henoch-Schonlein purpura; vasculitis seen in the setting of cryoglobulinemia and hypergammaglobulinemia of Waldenstrom, hereditary deficiencies of complement, and IgA deficiency; those leukocytoclastic vasculitides resulting from hypersensitivity reactions to drug, chemical and foodstuff ingestion; and those vasculitides seen in patients with systemic diseases such as polyarteritis nodosa, rheumatoid arthritis, mixed connective tissue disease, systemic lupus erythematosus, Sjogren's syndrome, relapsing polychondritis, Behcet's disease, Wegener's granulomatosis, and allergic granulomatosis of Churg and Strauss.

[Necessity to include allergic vasculitis caused by antibiotics in the list of occupational diseases]. / Obosnovanie neobkhodimosti vneseniia v spisok professional'nykh zabolevanii allergicheskikh vaskulitov, obuslovlennykh vozdeistviem antibiotikov.

Basing on the results of original research and foreign literature data, the author proved the necessity of including antibiotic induced allergic vasculitis in the occupational diseases list. These forms of vasculitis are characterized by specific clinical features, including nervous system, internal organs and skin disorders, and can be referred to as the secondary allergic vasculitis. Their including in the OD list in important for proper diagnosing occupational pathology and subsequent labour ability expertise, since resulting occupational disability is in most cases caused by NS disorders.