Microscopic polyangiitis. A systemic vasculitis with a positive P-ANCA

A young girl presented with a purpuric rash on lower limbs, fever, eosinophilia, peripheral neuropathy and progressive renal insufficiency. She developed vesicles, purpuric macules and papules on the head, several nodules on the palmar sides of hands and lingers, splinter haemorrhages, and a disfiguring, facial oedema. A renal biopsy specimen disclosed a focal and segmental necrotizing glomerulonephritis with crescents. Peripheral ANCA with antimyeloperoxidase specificity [P-ANCA (MPO)] was positive and cytoplasmic ANCA with PR 3 specificity was negative. Treatment with prednisone and cyclophosphamide was started with a good clinical response, stabilization of renal insufficiency and disappearance of P-ANCA (MPO). Our case fulfils the diagnostic criteria for microscopic polyangiitis (microscopic polyarteritis, MPA). namely a segmental necrotizing and crescentic glomerulonephritis associated with extrarenal vasculitis involving small-sized vessels, without granulomas or asthma. This is a rare disease, which has a poor prognosis in the absence of aggressive therapy, and is infrequently reported in dermatological journals.     

Comparison of cutaneous manifestations in systemic polyarteritis nodosa and microscopic polyangiitis

Background  The cutaneous manifestations of microscopic polyangiitis (MPA) and polyarteritis nodosa (PAN) have not been compared since their distinction.
Objectives  To compare the clinical and pathological cutaneous manifestations in a series of patients with systemic MPA and PAN.
Methods  Patients with MPA ( n  =   162) and PAN ( n  =   248) from the database of the French Vasculitis Study Group were diagnosed according to the American College of Rheumatology and/or the Chapel Hill Consensus criteria. Purpura, livedo, nodules, urticaria, skin necrosis, oral and genital ulcers were recorded when present. Fifty-five skin biopsies were analysed. Clinical and histological skin data were compared in the following groups: MPA, PAN and two PAN subsets: PAN with and PAN without hepatitis B infection. The prevalence of systemic and biological manifestations were analysed in relation to the presence or absence of skin lesions. The χ² test was used for statistical studies.
Results  Cutaneous manifestations were present in 44% of MPA and PAN. Purpura was the most frequent manifestation (26% cases of MPA vs. 19% cases of PAN, P  =   0·026). Urticaria was more frequent during PAN (6% vs. 1·2%, P  =   0·015). Skin lesions were more frequent during PAN in the absence of HBV infection (54% vs. 30%, P  <   0·05). No significant difference was detected from the histological data. Patients with skin lesions (either MPA or PAN) presented arthralgias and ocular manifestations more frequently. Mononeuritis multiplex was associated with skin lesions in the MPA group ( P  <   0·05).
Conclusions  The clinical or histological analysis of cutaneous lesions is not helpful for distinguishing PAN from MPA.     

Leukocytoclastic vasculitis: a window to systemic churg strauss syndrome

A twenty year old male presented with purpuric lesions with chronic painful ulcers over the lower extremities and a recurrent pruritic rash on the trunk for 10 years. He was diagnosed as idiopathic leukocytoclastic vasculitis (LCV) after investigations failed to reveal a systemic association. He was treated with immunosuppressants at each visit with partial remission. In 2004, he was diagnosed with bronchial asthma and allergic rhinitis. In his recent admission, he showed necrotic ulcers on legs and extensive shiny, truncal micropapules. Examination revealed maxillary sinus tenderness and loss of sensation on the medial aspect of the left lower limb. Biopsy of ulcer and the micropapules showed the presence of extravascular eosinophils, while hematological investigations showed peripheral eosinophilia of 18%, raised serum Immunoglobulin E (IgE), Anti nuclear antibody (ANA) positivity and negative antineutrophil cytoplasmic antibody (ANCA). Radiography confirmed maxillary sinusitis, nerve conduction studies revealed mononeuritis of the anterior tibial nerve and pulmonary function tests (PFT) were normal. Clinical examination and investigations pointed towards the diagnosis of Churg-Strauss syndrome (CSS). This report highlights the development of full-blown CSS over a period of 12 years in a patient initially diagnosed as idiopathic LCV, emphasizing the need for regular follow-up of resistant and recurrent cases of LCV.     

Urticarial vasculitis and recurrent pleural effusion: a systemic manifestation of urticarial vasculitis

A 62-year-old woman with an 8-year history of urticaria, arthralgia and arthritis developed recurrent pleural effusion. A thorough laboratory workup during repeated attacks of concomitant urticaria and pleural effusion disclosed only hypocomplementemia and the histopathological finding of the skin biopsy revealed leukoclastic vasculitis. These findings are compatible with the entity of urticarial vasculitis. It is thus suggested that pleural effusion can form part of the systemic manifestations of urticarial vasculitis.     

Cutaneous manifestations in patients with microscopic polyangiitis: two case reports and a minireview

Microscopic polyangiitis is a systemic small vessel vasculitis, which often has cutaneous and musculoskeletal features. Microscopic polyangiitis is a member of the family of anti-neutrophil cytoplasmic auto-antibody (ANCA)-associated vasculitides and is strongly associated with anti-myeloperoxidase (MPO)-ANCA. Titres of MPO-ANCA may reflect disease activity and play a pathogenic role. Patients with microscopic polyangiitis usually present with erythematous macules on the extremities as the first cutaneous manifestation. Skin biopsy specimens from the erythema reveal small-sized vessels that are infiltrated with neutrophils, consistent with leukocytoclastic vasculitis, in the deep dermis to the subcutaneous fat tissue. The cutaneous involvement is present at an early stage of microscopic polyangiitis with other non-specific symptoms, such as arthralgias and myalgias. The initial cutaneous manifestations are important in early diagnosis of possible ANCA-associated vasculitides with elevated ANCA titres.     

Microscopic polyangiitis presenting urticarial erythema and Henoch-Schonlein purpura: two case reports

Microscopic polyangiitis (MPA) is well known as a life-threatening member of a group of systemic vasculitis diseases. We report two cases of MPA. Case 1 was a 79-year-old-man who had been diagnosed with anti-neutrophil-cytoplasmic-antibody associated vasculitis (ANCA associated vasculitis) with alveolar hemorrhage and crescentric glomerulonephritis (CrGN). He presented with urticarial erythema in the abdomen, legs and back. The skin biopsy specimens showed leukocytoclastic vasculitis on the upper dermis. Case 2 was a 74-year-old-man, who presented with purpura on the abdomen, buttocks and legs that were similar to Henoch-Schonlein purpura (HSP). He also suffered from interstinal pneumonia. His renal biopsy specimens showed glomerulosclerosis and the peripheral pattern anti-neutrophil cytoplasmic antibody (P-ANCA) was positive. We reviewed the skin eruptions that had been reported with MPA, including our cases.     

Hypocomplementemic urticarial vasculitis: a rare presentation of systemic lupus erythematosus

BACKGROUND: Urticarial vasculitis is a small-vessel vasculitis, presenting clinically as persistent urticarial skin lesions and microscopically as leucocytoclastic vasculitis. Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a distinct type of urticarial vasculitis with multiorgan involvement, whose etiology and link with other diseases are still unknown. Some authors have suggested that HUVS can be accompanied by systemic lupus erythematosus (SLE), and others believe that it is a rare subtype of SLE. Urticarial vasculitis is seen in 7-8% of SLE, while 50% of HUVS patients are diagnosed with SLE. OBSERVATIONS AND RESULTS: We report a case of HUVS associated with SLE with fatal outcome unresponsive to the combination of systemic corticosteroids and azathioprine. CONCLUSIONS: SLE and HUVS share both clinical and laboratory features and are probably not separate entities. It is mostly likely that HUVS and SLE fall into the same spectrum of autoimmune diseases. HUVS is probably a subset of SLE. As both diseases can fatally, it should be kept in mind that the overlap of SLE and HUVS may exhibit a relatively rapid progression and poor prognosis.     

Saturday night purpura: An uncommon presentation of exercise-induced vasculitis

Exercise-induced vasculitis (EIV) is a benign cutaneous vasculitis that affects healthy individuals. We report a case of EIV in a 15-year-old male patient with a striking clinical appearance triggered by a combination of prolonged standing and disco dancing in a warm environment. Pediatric dermatologists should be aware of this activity as a possible trigger of EIV, especially in teenagers.     

Endothelial cell pathology as a marker for urticarial vasculitis: a light microscopic study

Various criteria have been used for the diagnosis of urticarial vasculitis, and for the classification of patients with chronic urticaria. In the present study, semithin Epon-embedded sections were obtained from fifteen patients using a method of fixation optimal for the preservation and examination of the dermal microvasculature. This allowed patients to be classified according to the presence or absence of significant endothelial cell pathology. This feature proved a more reliable indicator of associated humoral abnormalities than the nature of the perivascular infiltrate as seen on paraffin-embedded sections. Endothelial cell necrosis per se was a rare event and could not be used as a reliable criterion for the diagnosis of urticarial vasculitis. Most cases showed either endothelial hypertrophy or shrinkage.     

Urticarial vasculitis in a patient with systemic lupus erythematosus: a case report

A patient with systemic lupus erythematosus is discussed, who presented with clinical and histological features of urticarial vasculitis. It is concluded that these two syndromes can co-exist and that they should be considered as variants of the same immune-complex syndrome.     

Recurrent annular erythema with purpura: a new variant of leucocytoclastic vasculitis responsive to dapsone

Annular lesions are rarely reported in the clinical spectrum of leucocytoclastic vasculitis, except in the acute baemorrbagic oedema of the skin. We report tbree patients who suffered from an extraordinary recurrent annular dermatitis, for 4 years in one case and for decades in tbe otber two. The eruption was cbaracterized by purpuric lesions that bad a centrifugal evolution, creating target0-or polycyclic patches disseminated on the limbs and trunk. Tbe palienls' general condition remained excellent during tbe attacks. All lesions spontaneously disappeared within 2 weeks, but recurred monthly. In all three cases, the histological changes were consisteni with leucocytoclastic vasculitis. One patient had ulcerative colitis and another had a benign immunoglobulin A (IgA) monoclonal gammopalhy. These two patients showed a good response to dapsone tberapy. This dermatosis probably represents a new and rare variant of leucocytoclastic vasculitis.     

Purpura simplex (inflammatory purpura without vasculitis): a clinicopathologic study of 174 cases.

Purpura simplex manifests clinically as macular purpuric and petechial pigmented, golden, annular, or lichenoid lesions. These subtypes have been termed Schamberg's purpura, lichen aureus, Majocchi's purpura, and Gougerot-Blum purpura. Histologically, there is inflammation and hemorrhage without fibrinoid necrosis of vessels. One hundred seventy-four cases were retrospectively reviewed. In some patients, the eruption was related to medications. Treatment was usually of limited benefit. Fifty-eight of 87 patients (67%) who had follow-up data did eventually have clearing of lesions. Because of the clinical and histologic similarities among the subtypes, the inclusive term purpura simplex is favored.     

Adamantiades-Beh?et disease: necrotizing systemic vasculitis with a fatal outcome]

Vascular involvement in Beh?et's disease was first mentioned by Adamantiades and occurs in addition to the classic symptom triad. In the present work an unusually fulminant course of Adamantiades-Beh?et disease with a lethal outcome is reported in a 43-year-old male patient of German origin. The first symptoms were painful oedemas of the joints, followed 1 year later by bipolar aphthous lesions and by multiple progressive ulcers of the skin and the oral mucosa. Histologically, pustule formation with underlying vasculitis was diagnosed. In spite of systemic treatment with azathioprine and high-dosed prednisolone, the course was progressive with multiple necrotizing ulcers, and the patient died 18 months after the onset of the disease with signs of heart insufficiency and cachexia. The autopsy findings revealed multiple lesions of ulcerating systemic vasculitis of the skin and other visceral organs, including the lungs and the central nervous system, and also endocarditis verrucosa ulcerosa of the mitral valve.     

Urticarial vasculitis associated with a monoclonal IgM gammopathy: Schnitzler''s syndrome

We describe two cases of Schnitzler's syndrome presenting with urticarial vasculitis, in which there was a monoclonal IgM of kappa light-chain isotype. Skin histology showed a perivascular leucocytic infiltrate and leucocytoclasis. One patient had an abnormal complement profile and positive rheumatoid factor while the other had antibodies to unidentified soluble nuclear antigens, anti-cytoplasmic antibodies and circulating immune complexes. In addition, in the first case deposits of IgM, C3 and, to a lesser degree, of IgG were observed in the walls of small blood vessels.     

Clinical and immunological characterization of patients with systemic sclerosis overlapping primary biliary cirrhosis: a comparison with patients with systemic sclerosis alone

Limited cutaneous systemic sclerosis (ISSc) or CREST syndrome is sometimes complicated by primary biliary cirrhosis (PBC). To characterize the clinical and immunological features of patients with SSc overlapping PBC (SSc-PBC), the clinical and laboratory data of 11 SSc-PBC were studied. Since all of the SSc-PBC were ISSc, the features of SScP-BC were compared with those of 266 patients with ISSc alone. The sera from SSc-PBC showed a significantly higher positivity for anti-centromere antibody (ACA) (p<0.0005) and higher reactivity to centromere protein-C. Although SSc-PBC more frequently exhibited calcinosis and telangiectasia (p<0.05), there was no statistical difference in other features of CREST syndrome. There was no statistical difference in other scleroderma-related features either. SSc-PBC were more frequently complicated with Sjögren syndrome (p<0.05). The most prominent feature of SSc-PBC was a higher incidence of ACA than in ISSc alone.