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.     

Annular vasculitis of the head and neck in a patient with sarcoidosis

A patient with bilateral hilar lymphadenopathy due to sarcoidosis presented with erythema nodosum on the shins and upper arms and an unusual annular eruption resembling erythema annulare centrifugum on the head and neck. Biopsy of the latter showed a leucocytoclastic angiitis. The aetiology of sarcoidosis is unknown. There is increasing evidence for the presence of circulating immune complexes in the early stages of acute sarcoidosis which may present as erythema nodosum, iritis, polyarthralgia and bilateral hilar lymphadenopathy (James, 1978). Circulating immune complexes may result in a localized cutaneous vasculitis (Ryan & Wilkinson, 1979).     

Tuberculous lymphadenitis presenting with cutaneous leucocytoclastic vasculitis

Cutaneous leucocytoclastic vasculitis is an inflammatory vascular disorder due to deposition of immune complexes in dermal vessels. A direct or indirect rule of infectious agents in the pathogenesis of such vasculitis has been postulated. We describe a patient with cervical lymphadenitis due to Mycobacterium tuberculosis infection who developed cutaneous vasculitis. The vasculitis resolved with standard antituberculous therapy. The association between leucocytoclastic vasculitis and tuberculous infection has only rarely been reported.     

Congenital syphilis presenting with nephrotic syndrome and leucocytoclastic vasculitis

Nephrotic syndrome is a well-recognized entity in congenital syphilis but leucocytoclastic vasculitis has not yet been described. We report a case of nephrotic syndrome and leucocytoclastic vasculitis associated with congenital syphilis in a 45-day-old male infant who did well after treatment with intravenous penicillin.     

Leucocytoclastic vasculitis induced by prolonged exercise

Summary Many people develop skin symptoms after long-distance walks, but little is known about the aetiology of these. In this study we took 11 biopsies from 10 long-distance walkers who walked 80 km. All biopsies originated from purpuric lesions on the lower legs. which had appeared during walking. In all 11 specimens, signs of a leucocytoclastic vasculitis were present with leucocytoclasis, exocytosis of erythrocytes and a granulocyte/mononuclear perivascular infiltrate. Immunofluorescence investigations showed deposition of C3c in many specimens and immunoglobulin M in some. The occurrence of a leucocytoclastic vasculitis after prolonged exercise may be explained by the existence of an exercise altered cutaneous microcirculation, complement activation and an altered immune function.     

Bowel bypass arthritis dermatitis syndrome: a histological and electron microscopical study

A case of dermal vasculitis with arthralgia after intestinal bypass surgery is reported. A 36-year-old woman developed arthralgia and skin rash, 1 year after an ileo-jejunal bypass operation was performed for overweight (130 kg). Skin biopsy showed leucocytoclastic vasculitis in the dermis. E.M. study showed clumps of platelets around small dermis blood vessels, and polymorphous perivascular infiltration. The symptoms subsided after tetracycline treatment.     

Erythema elevatum diutinum mimicking porphyria cutanea tarda

A case of erythema elevatum diutinum (EED) closely resembling porphyria cutanea tarda (PCT) is reported. The initial skin biopsies were suggestive for PCT but porphyrin levels in the urine, stool and plasma were normal. A further biopsy from an early cutaneous lesion showed a leucocytoclastic vasculitis with fibrinoid necrosis of the vessel walls.     

皮肤白细胞碎裂性与淋巴细胞性血管炎某些粘附分子表达的比较研究

目的：比较细胞粘附分子ＩＣＡＭ－１、ＶＣＡＭ－１和ＥＬＡＭ－１在皮肤白细胞碎裂性和淋巴细胞性血管炎的表达及其意义。方法：采用免疫组化方法研究两型血管料的粘附分子表达情况。结果：角质形成细胞ＩＣＡＭ－１在淋巴细胞性血管炎（８５．７１％）表达明显高于白细胞碎裂性血管炎（１１．１１％（Ｐ〈０．０００１）,ＩＣＡＭ－１、ＶＣＡＭ－１、ＥＬＡＭ－１在两型血管炎血管内皮中表达无区别（Ｐ值分别为１,０．０６８,０．４６）。结论：无论在白细胞碎裂性还是淋巴细胞性血管炎、粘附分子表达均上调。两种血管炎粘附分子表达率的不同反应了局部细胞因子释放的不同,这对揭示血管炎发病机理将具有重要意义。     

Systemic paraneoplastic vasculitis secondary to papillary carcinoma of the thyroid

Systemic vasculitis secondary to thyroid carcinomas is exceptional. We report the case of a 55‐year‐old woman who presented with systemic vasculitis involving leucocytoclastic cutaneous vasculitis and renal disorders secondary to papillary thyroid carcinoma (PTC). Her symptoms resolved completely after total thyroidectomy. Other causes of vasculitis were excluded. To our knowledge, this is only the second case reported of systemic vasculitis associated with PTC in a paraneoplastic manner.     

Immunohistochemistry of nodular vasculitis. A possible role of delayed cellular hypersensitivity]

The physiopathogenesis of nodular vasculitis is still unknown: there is probably an initial Arthus' phenomenon responsible for the lesions of the deep vessels, but the frequent recurrences and the long time duration of the hypodermic nodules are still not understood. Dendritic protein S100 positive cells had previously been observed within hypodermal granulomas. The purpose of this study was to confirm these observations in a large group of nodular vasculitis, and to compare the expression of S100 protein in other vascular diseases and in pure panniculitis. Immunohistochemical staining for S100 protein was performed on paraffine-embedded samples: 45 cases of nodular vasculitis, 21 of panniculitis, 10 of superficial leucocytoclastic vasculitis, 10 of periarteritis nodosa, and 10 of erythema nodosum were analyzed by means of optic microscopy. Numerous dendritic S100 protein positive cells were found within hypodermal granulomas in most of the cases of nodular vasculitis, but these cells were absent in the 3 cases of initial deep leucocytoclastic vasculitis lesions of nodular vasculitis. The mean percentage of these cells was 4.5 p. 100 in the hypodermal inflammatory areas; they were observed mainly around vascular lesions. Hypodermal dendritic cells were absent or less numerous in all other cases, and a high number of such cells seems to be observed only in nodular vasculitis. Because of their dendritic morphology, and the high expression of S100 protein, these cells could be interdigitated cells, which are known to present antigens to T-lymphocytes. They could play an important role in generating the chronicity of nodular vasculitis, by a mechanism of delayed cell-mediated hypersensitivity, after the initial lesions caused by immune complexes.     

Annular lesions of cutaneous sarcoidosis with granulomatous vasculitis

Sarcoidosis is known to be involved in diseases with vasculitis as sarcoid vasculitis. However, vasculitis in cutaneous sarcoidal lesions is extremely rare. Here we describe a case of sarcoidosis with multiple annular skin lesions with granulomatous vasculitis. A 62‐year‐old female was diagnosed with sarcoidosis by chest‐abdominal computed tomographic examination and laboratory tests. The skin lesions had appeared on her lower limbs 2 years before. Physical examination showed multiple infiltrated annular eruptions on the lower extremities. A skin biopsy of an area of erythema showed multiple non‐caseating epithelioid cell granulomas in the dermis and subcutaneous fat and granulomatous vasculitis with fibrinoid degeneration in the subcutaneous fat. There are two types of vasculitis in sarcoidosis: leukocytoclastic and granulomatous vasculitis. Ulcers and livedo were more common in granulomatous vasculitis than in leukocytoclastic vasculitis. The present case had unique annular skin lesions of sarcoidosis with granulomatous vasculitis.     

Studies on a patient with leucocytoclastic vasculitis ''pyoderma gangrenosum'' and paraproteinaemia

A patient with leucocytoclastic vasculitis, pyoderma gangrenosum, chronic pyelonephritis and a paraprotein of IgA type is described. The vasculitis responded favourably to dapsone therapy, while the pyoderma gangrenosum improved only when steroids were also given. During an exacerbation following withdrawal of dapsone, the serum IgG level fell early, but the β_1c globulin level remained normal. Immunofluorescence studies on a vasculitic lesion showed granular deposits of IgG and β_1c the walls of the blood vessels and a more extensive exudate of fibrin. Skin testing with bacterial antigens and patch testing to potassium iodide produced lesions which clinically, histologically and immunologically resembled the spontaneous vasculitic lesion.     

Erythema elevatum diutinum--evidence for disease-dependent leucocyte alterations and response to dapsone

Erythema elevatum diutinum (EED) is a type of leucocytoclastic vasculitis of unknown aetiology. We report a patient with unusually widespread and disabling EED that had been unresponsive to corticosteroids and antibiotics, but resolved on dapsone. Biopsies of fresh lesions showed typical features of leucocytoclastic vasculitis, with prominent neutrophil infiltration, marked expression of the beta(2)-integrins CR3 and LFA-1, and increased mast cell numbers. Older lesions exhibited granulation tissue and fibrosis, macrophages were more dominant, beta(2)-integrins were expressed less markedly, and mast cell numbers were lower. In vitro chemotaxis of the patient's peripheral blood neutrophils prior to treatment showed increased random migration and directed migration towards interleukin-8 (by 424%), but a profoundly decreased responsiveness towards the bacterial peptide analogue N-formyl-methionyl-leucyl-phenylalanine (fMLP) (by 98%). These values returned to normal after dapsone treatment and clinical improvement 5 months later. These findings support the concept that in EED, activation via cytokines such as interleukin-8 allows a selective recruitment of leucocytes to tissue sites, while immune complexes and bacterial peptides sustain the persistent local inflammatory infiltrate and the leucocytoclastic vasculitis.     

Allergic vasculitis following ingestion of celecoxib?

A 76-year-old patient developed cutaneous vasculitis on the lower legs on the 8th day of treatment with the selective cox-2-inhibitor celecoxib (Celebrex((R))) and the proton-pump inhibitor omeprazole. The patient had no history of allergic reactions. The patient had already been treated previously with omeprazole without any side effects. A cutaneous biopsy confirmed the diagnosis of leucocytoclastic vasculitis. The purpuric skin lesions regressed within 3 weeks after withdrawing the two newly introduced drugs. We discuss the potential role of Celecoxib in triggering this vasculitis.     

Acute leucocytoclastic vasculitis and aquagenic pruritus long preceding polycythemia rubra vera

We describe the unusual case of a 67-year-old woman with aquagenic pruritus, immediately following acute cutaneous leucocytoclastic vasculitis and preceding the development of polycythemia rubra vera by 3 years.     

Leucocytoclastic vasculitis and angio-oedema associated with inflammatory bowel disease

Two cases are reported in which purpuric skin plaques due to leucocytoclastic vasculitis and angio-oedema were the presenting signs of inflammatory bowel disease. The aetiological implications are discussed. One case was controlled with oral colchicine, the other with oral Salazopyrine.     

Leucocytoclastic vasculitis and influenza vaccination

Influenza vaccination is recommended for all people over 75 years of age and for an expanding range of other indications. Side-effects of influenza vaccination are usually mild but we describe four cases of leucocytoclastic vasculitis following influenza vaccination. The four patients, who were all elderly, presented with cutaneous vasculitis but all had abnormal urinalysis suggestive of associated renal involvement. Since 1974 only 10 cases of vasculitis following influenza vaccination have been reported in the literature. The clinical features of these cases are reviewed. As the use of influenza vaccination is likely to increase, dermatologists should be aware of the possible association with vasculitis.     

Iododerma and acute respiratory distress with leucocytoclastic vasculitis following the intravenous injection of contrast medium

A 72-year-old woman with chronic renal failure requiring haemodialysis developed acute iododerma twice, after receiving iodide contrast dye for radiological procedures. Iododerma was localized to the face, scalp and elbows and was associated with papular purpura of the legs. Histopathology of the skin lesions showed acute necrotizing vasculitis. During the second skin eruption the patient developed acute respiratory distress, which was treated with corticosteroids. During the first eruption asymptomatic infiltrates were present on chest X-ray which disappeared 2 months later. At the present time iododerma seems more frequent in patients with renal failure. Iodides may also be responsible for pulmonary abnormalities, which are sometimes asymptomatic. All these features may be due to leucocytoclastic vasculitis following iodide ingestion.     

Hypergammaglobulinaemic purpura associated with alcoholic liver cirrhosis

Hypergammaglobulinaemic purpura (HP) is commonly found in Sjögren’s syndrome. We report a rare association of HP arising in a patient with alcoholic liver cirrhosis. A 65‐year‐old man presented with palpable purpura on the legs. Histological examination of a biopsy taken from the purpura found leucocytoclastic vasculitis in the superficial and mid‐dermis. The activity of the vasculitic skin lesions correlated with liver dysfunction. Increased IgA and IgG levels, and hypocomplementaemia, may account for the pathogenesis of the hypergammaglobulinaemic immune complex‐mediated vasculitis in this case.     

表现为血疱的持久性隆起性红斑1例

患者男,70岁。双手、足、肘、膝出现红斑、血疱6个月。皮损组织病理示:表皮下水疱,疱内可见大量嗜中性粒细胞,真皮内可见弥漫的嗜中性粒细胞浸润,血管管壁及管周可见纤维素样渗出及核碎裂。诊断:持久性隆起性红斑。临床医师应警惕以血疱为表现的持久性隆起性红斑。