Pulmonary Tuberculosis Presenting with Cutaneous Leukocytoclastic Vasculitis

Summary This case report deals with a rare association: tuberculosis and cutaneous leukocytoclastic vasculitis. The patient was a 36-year-old man with no significant past medical problems. He presented with a palpable purpura on both legs, low-grade fever, cough and expectoration, progressive dyspnea due to a massive left pleural effusion and a symmetric swelling on his ankles and wrists. Skin biopsy yielded a histological diagnosis of leukocytoclastic vasculitis and the primary diagnosis was only achieved after performing a pleural biopsy, which unequivocally showed the presence of Mycobacterium tuberculosis. This case shares many features with the few cases already reported in the medical literature. Possible pathogenic mechanisms are reviewed and discussed in detail. Received: May 4, 1999 · Revision accepted: September 19, 1999     

Bacterial infection presenting as cutaneous vasculitis in adults.

OBJECTIVE: To examine the frequency and clinical features of patients with bacterial infection presenting with biopsy-proven leukocytoclastic cutaneous vasculitis (CV) in a well-defined area of southern Europe (northwestern Spain). METHODS: A retrospective study of an unselected population of adult patients (age > 20 years) with biopsy-proven leukocytoclastic CV diagnosed at the Hospital Xeral-Calde (Lugo, Spain) was carried out from January 1988 through December 1997. Cutaneous vasculitis related to bacterial infection was considered if the vasculitis was confirmed by a skin biopsy showing leukocytoclastic vasculitis, if no drug intake was registered prior to the development of CV, and if bacteriologic evidence of infection was obtained. RESULTS: Four of 138 patients (2.9%) presenting with biopsy-proved CV were diagnosed with leukocytoclastic CV related to bacterial infection. Three patients (2 with bacterial endocarditis and 1 with meningococcemia) met the ACR criteria for the classification of hypersensitivity vasculitis. Another patient with bacterial endocarditis met the criteria for mixed cryoglobulinemia. All of them presented with palpable purpura, high or low grade fever, an elevated erythrocyte sedimentation rate and leukocytosis. CONCLUSION: Cutaneous vasculitis may be the presenting manifestation of bacterial infection. In this respect, rheumatologists should be aware of possible infectious causes of vasculitis, even though they are not common.     

An adult case of Henoch-Sch?nlein purpura complicating common peroneal nerve mononeuropathy

We present an adult patient with Henoch-Schönlein Purpura who developed mononeuropathy in the common peroneal nerve. Upon admission, the patient had palpable purpura in the arms and legs, polyarthralgia, abdominal pain, and leukocytoclastic vasculitis in the skin biopsy. These symptoms disappeared with 30 mg daily of oral prednisolone. One month later, after induction therapy, fever, livedo reticularis and peripheral mononeuropathy developed with hypocomplementemia and the patient was treated successfully with glucocorticoid pulse therapy.     

Leukocytoclastic vasculitis with severe cardiac involvement in an infant: a case report

Severe visceral involvement in leukocytoclastic vasculitis is rare. We report here the case of a 2-month-old male infant with leukocytoclastic vasculitis who initially presented with fever and skin purpura. Endomyocarditis with mitral valve regurgitation, multiple hepatic infarction, and pulmonary hemorrhage developed later. The patient was successfully managed with an aggressive treatment of high frequency oscillation ventilation and extracorporeal membrane oxygenation together with corticosteroid and azathioprine therapy for 3 months. Valvuloplasty was also performed due to irreversible damage of the mitral valve, although long-term oral anticongestive medications were also needed.     

Squamous cell carcinoma of the lung presenting with cutaneous leukocytoclastic vasculitis: a case report

An elderly patient with paraneoplastic cutaneous leukocytoclastic vasculitis (CLV) leading to the discovery of an as-yet asymptomatic, potentially surgically curable squamous cell carcinoma of the lung is presented. This type of vasculitis has not been previously described in association with this type of lung cancer. Since vasculitic paraneoplastic syndromes, including CLV, may develop before the clinical presentation of malignant tumors, the present case and others previously reported strongly suggest that in apparently idiopathic CLV, patients should be evaluated for the presence of occult malignancy that could be curable by early detection.     

Cutaneous leukocytoclastic vasculitis and myelodysplastic syndrome with little or no evidence of associated autoimmune disorders-a case report and a brief review of the literature

Cutaneous leukocytoclastic vasculitis (CLV) is a necrotizing inflammatory disease of the small vessels in the dermis. Approximately 50% of cases with CLV are primary or idiopathic and the remainder may be associated with various diseases. Less than 1% CLV occurs in association with malignancies including leukemia, lymphoma, and myelodysplastic syndrome (MDS). The pathogenetic mechanism of CLV remains speculative and is generally believed to be related to autoimmune processes. We report here a 77-year-old white woman who presented with contemporaneous occurrence of CLV and MDS (WHO subtype RAEB-2). Autoantibodies and immune-complexes were not detected either in the serum or by direct immunofluorescense in the skin biopsy. The clinical course of MDS remained steady, but the cutaneous lesion resolved within 2 weeks with minimal or no specific therapy except corticosteroids. We suggest that absence of autoimmune phenomena and immune-complex in the skin lesions of CLV may be a hallmark of good prognosis.     

Systemic cholesterol embolization syndrome associated with myeloperoxidase-anti-neutrophil cytoplasmic antibody

A 75-year-old man was transferred to our department because of development of severe renal impairment after coronary artery bypass grafting. Hemodialysis was initiated for postsurgical oliguria and lung congestion. On transfer, he showed systemic purpura rashes and diffuse blue mottlings on his toes with marked eosinophilia and an elevated level of C-reactive protein. Cutaneous biopsy revealed cholesterol crystal embolism and leukocytoclastic vasculitis in dermal arterioles. Myeloperoxidase-anti-neutrophil cytoplasmic antibody titer was increased. Upon oral corticosteroid therapy following intravenous pulse steroid therapy, the purpura dramatically diminished, renal function improved, and hemodialysis was discontinued. Active treatment with corticosteroids may be effective for cholesterol embolization syndrome, particularly when clinical and laboratory manifestations mimic systemic vasculitis.     

Circulating IgA producing cells in the differential diagnosis of Henoch-Sch?nlein purpura

The number of immunoglobulin producing cells was determined by a plaque forming cell assay in 23 patients with definite Henoch-Sch?nlein purpura, in 5 children with probable Henoch-Sch?nlein purpura, in 11 patients with leukocytoclastic vasculitis not associated with a collagen vascular disease, and in 2 age matched control groups of healthy individuals. In the group with leukocytoclastic vasculitis, the numbers of circulating Ig producing cells were within normal limits, and lower than those found in definite Henoch-Sch?nlein purpura for IgA secreting cells (t = 8.26, p less than 0.001) and IgM secreting cells (t = 2.78, p less than 0.01). The number of circulating IgA secreting cells discriminated between definite Henoch-Sch?nlein purpura and leukocytoclastic vasculitis with a diagnostic sensitivity of 95.7% and specificity of 90.9%. Four of the 5 patients with probable Henoch-Sch?nlein purpura also had an increased number of IgA secreting cells. Therefore, an increased number of circulating IgA secreting cells in a patient with hypersensitivity vasculitis without a collagen vascular disease supports the diagnosis of Henoch-Sch?nlein purpura.     

Isolated Colonic Leukocytoclastic Vasculitis Causing Segmental Megacolon: Report of a Rare Case

Abstract We report the case of a 44-year-old white man who presented with progressively worsening crampy abdominal pain and distention. Deterioration of his clinical picture along with leukocytosis and radiographic evidence of severe colonic dilation rendered exploratory laparotomy necessary. Greatly distended and inflamed transverse and descending colon were evident and an extended left colectomy was performed. Characteristic changes of leukocytoclastic vasculitis in the serosal and muscular layers of the resected colon were demonstrated at histopathologic examination. Systemic leukocytoclastic vasculitis, usually coexisting with Henoch-Schönlein purpura, commonly affects the small bowel with clinical evidence of ischemia or bleeding. Colon involvement is infrequently reported in the context of systemic disease. Isolated colonic leukocytoclastic vasculitis without extraintestinal manifestations is rare. A previously unreported case of localized leukocytoclastic vasculitis of the left colon resulting in the impressive presentation of megacolon, without the presence of any precipitating factor or associated systemic disease is presented here, with an overview of the related literature.     

Localized vasculitis of the gastrointestinal tract

BACKGROUND AND OBJECTIVES: Isolated vasculitis of the gastrointestinal (GI) tract is a rare entity. Endoscopic biopsies have low sensitivity to diagnose intestinal vasculitis, even though the endoscopic findings may be suggestive of this condition. Our aims were to describe a case of biopsy-proven colonic leukocytoclastic vasculitis and review the literature. METHODS: A patient with biopsy-proven colonic leukocytoclastic vasculitis is described. A Medline database search of cases with localized GI vasculitis between January 1985 and September 2005 was conducted. RESULTS: A 32-year-old man was admitted to the hospital because of abdominal pain and diarrhea. A colonic biopsy showed leukocytoclastic vasculitis. There are very few articles on leukocytoclastic GI vasculitis as a separate disease, and most of them emphasize the difficulty in classification. Unlike our case, in former cases of localized vasculitis a diagnosis was made after surgery. Although our patient had steroid-refractory biopsy-proven isolated intestinal vasculitis, treatment with intravenous cyclophosphamide resulted in rapid resolution of symptoms and surgery was not required. CONCLUSIONS: In patients with abdominal pain a diagnosis of intestinal vasculitis should be considered. Immunosuppressive therapy allowed our patient to avoid surgery and may be similarly beneficial in other similar cases.     

Pfeifer-Weber-Christian's panniculitis in an obese patient with antinuclear antibody-positive leukocytoclastic vasculitis

Pfeifer-Weber-Christian's panniculitis is a rare syndrome characterized by fever, arthralgias, fatigue and recurrent nodular panniculitis. It has been associated with pancreatic diseases, trauma, connective tissue diseases, alpha-1-antitrypsin deficiency, systemic lupus erythematosus, infections, lymphoproliferative diseases and neoplasias. We report the case of a 43-year-old obese male patient who presented with asthenia, arthralgias, intermittent fever, skin erythema and a large hard-elastic tumor of the right calf. Laboratory analysis revealed increased values of the immunophlogosis parameters and positivity for serum antinuclear antibodies. Surgical drainage of the abscess-like tumor mass, revealed leakage of a sterile, subflavious, oily and thick liquid; a skin biopsy showed intra and perivascular infiltration by neutrophils, diagnostic for leukocytoclastic vasculitis. Treatment with prednisone induced clinical improvement and normalization of the laboratory data. The clinical picture, laboratory data and efficacy of prednisone therapy confirmed that the patient developed Pfeifer-Weber-Christian's panniculitis in the clinical setting of an antinuclear antibody-positive leukocytoclastic vasculitis.     

Acute brucellosis presenting as leukocytoclastic vasculitis.

We present the case of a 64 year-old-male fur trader from Lerida (Spain), who consulted us because of a 2-week history of night fever and the presence of non-pruritic purpuric lesions on the distal third of the lower limbs. Laboratory findings demonstrated positive serology for brucellosis and cutaneous biopsy showed leukocytoclastic vasculitis. The pathogenesis of the cutaneous process is discussed. We also suggest that brucellosis should be included among the infections taken into account in patients suffering from leukocytoclastic vasculitis coming from brucella-endemic areas.     

Quinidine-induced vasculitis

Four patients developed nonthrombocytopenic purpura two to three weeks after initiation of quinidine therapy. The skin lesions disappeared and did not recur after cessation of quinidine therapy. Histologic examination revealed leukocytoclastic vasculitis with deposition of C3, IgA, and/or IgM in the small dermal vessels. Since quinidine purpura is usually associated with thrombocytopenia, the possibility of leukocytoclastic vasculitis as an additional cause of purpura is stressed.     

Henoch-Schonlein purpura and prostate cancer

INTRODUCTION: The association between adult Henoch-Schonlein purpura and prostatic carcinoma is exceptional. We report a new case. EXEGESIS: The diagnosis of prostatic adenocarcinoma, suspected because of prostatic induration is made after anatomopathological study. The patient has all the clinical (purpura, polyarthralgia), biological (circulating monocmonal IgA), and histological (leukocytoclastic vasculitis, with IgA and C3 deposits) criteria of Henoch-Schonlein purpura too. The simultaneous appearance of vasculitis and neoplasia is known. The association between adult Henoch-Schonlein purpura and malignant neoplasm like lung carcinoma or lymphoma exists too. The initial events leading to the development of these vasculitis could be tumor antigens or abnormal IgA production. CONCLUSION: The simultaneous appearance of these two disease asks here the question of mechanisms implicated in these association.     

Systemic hypersensitivity vasculitis associated with bronchiectasis.

Systemic hypersensitivity vasculitis developed in a 53-year-old man during acute exacerbation of bronchiectasis infected with Pseudomonas aeruginosa. High grade fever, mononeuropathy multiplex, cutaneous vasculitis, and biopsy specimen-proved mesangioproliferative glomerulonephritis with crescent formation and leukocytoclastic vasculitis associated with circulating immune complex occurred. Corticosteroid and cyclophosphamide therapy was effective for vasculitis and bronchiectasis.     

A case of henoch-schönlein purpura nephritis in pulmonary tuberculosis

A 38-year-old man with pulmonary tuberculosis developed purpura over both lower extremities and renal disturbance after starting antituberculosis treatment. A renal biopsy and skin biopsy were performed to diagnose the new clinical manifestations, and leukocytoclastic vasculitis with IgA and C3 deposition were detected. Henoch-Schönlein purpura nephritis (HSPN) was diagnosed on the basis of the clinical and pathologic findings, and prednisolone therapy was added. The skin lesions disappeared in 7 days after starting steroid therapy, and renal function gradually improved. These results suggested that the pathogenesis of HSPN might be the consequence of the deposition of the circulating immune complexes. The treatment of HSPN has been not established yet. We should consider how to use steroid therapy for HSPN and call attention to the recurrences of renal disturbance and pulmonary tuberculosis. It is thus recommended to follow patients with HSPN in tuberculosis for long periods.     

Gastrointestinal involvement in Henoch-Schönlein purpura

We report a 20-year-old young man with Henoch-Sch?nlein purpura (HSP) who presented with abdominal pain, arthralgia, and skin rash. The gastrointestinal tract was viewed by upper endoscopy and colonoscopy. We found characteristic endoscopic findings in the stomach, cecum and sigmoid colon, the combination of which has rarely been demonstrated in one patient. Histologic examination of skin biopsy specimens and gastrointestinal tract revealed leukocytoclastic vasculitis with positive staining for IgA in the capillaries. The clinical course of this patient was further improved after the combined therapy with steroids and immunosuppressive drug.     

Hexamethylene bisacetamide-induced cutaneous vasculitis

Nonthrombocytopenic purpura developed in a patient receiving hexamethylene bisacetamide, a tumor-differentiating agent in vitro that is in phase I clinical trials. Histopathological examination revealed features of leukocytoclastic vasculitis with deposition of IgA and complement in the dermal vasculature. Leukocytoclastic vasculitis, an extremely rare complication of therapy with antineoplastic agents, is reported to alert others using this compound of this association.     

Hautmanifestationen der verschiedenen Vaskulitiden

The skin not only represents the organ which often reveals the first signs of systemic vasculitis, but also the organ which is most frequently involved in vasculitis. These diseases encompass systemic vasculitides and those which appear to involve the skin only. Among those vasculitides restricted to the skin, some are yet typically associated with other systemic diseases, such as nodular vasculitis, which often occurs during infections by M. tuberculosis, or erythema elevatum diutinum in patients with gammopathy. The type and localization of skin lesions give valuable indications as to the type of vasculitis. Subcutaneous nodules which ulcerate and are surrounded by livedo racemosa are suggestive of polyarteritis nodosa, a palpable purpura with predilection for the lower legs is almost pathognomonic for immune complex vasculitis (e.g. IgA vasculitis or cutaneous leukocytoclastic vasculitis), hemorrhagic papules and necrotic plaques which occur in acral areas after cooling indicate cryoglobulinemic vasculitis, hemorrhagic papules and macules which develop in patients who start to feel worse and develop fever should arouse suspicion of septic vasulitis, while the simultaneous presence of ulcerating nodules and hemorrhagic papules without predilection for the lower legs will suggest ANCA-associated vasculitis. The different morphology of the cutaneous signs of the various vasculitides depends to a large extent on the size of the vessels primarily involved. In this review the cutaneous signs of vasculitides will be presented with reference to the revised nomenclature of the Chapel Hill Consensus Conference from 2012.