1/11. A case of intravascular large B-cell lymphoma mimicking erythema nodosum: the importance of multiple skin biopsies.BACKGROUND: Intravascular lymphoma is a rare disease characterized by the proliferation of neoplastic monuclear cells within the lumens of small blood vessels. The neoplastic cells are usually of B-cell origin, and rarely of T-cell or histiocytic origin. Although this clinicopathological entity of lymphoma has not been listed in general pathological classifications such as REAL classification or the Working Formulation, it is recently in the WHO classification scheme, which is essentially an updated REAL scheme, and the EORTC classification scheme. methods: In this report, a 62-year-old woman with intravascular large B-cell lymphoma was observed by clinical, histopathological, immunohistochemical and molecular methods. RESULTS: A 62-year-old woman presented with large erythematous macules on the bilateral thighs and lower legs. The lesions were accompanied with hard, tender, intradermal or subcutaneous nodules mimicking erythema nodosum. Histopathological examination in the first biopsy revealed non-specific panniculitis compatible with erythema nodosum. The second biopsy revealed emboli of atypical lymphocytes within many of the dilated and proliferated vessels in the deep dermis and subcutaneous tissue. These cells were positive for L-26 and kappa light chain, and negative for lambda light chain, factor viii-related antigen, CD30, CD34, CD68 and UCHL-1. These findings confirmed the diagnosis of intravascular large B-cell lymphoma. A laboratory examination showed a high level of LDH and abnormal cells in the bone marrow. An MRI of the brain and computed tomographic (CT) scans of the chest and abdomen revealed no evidence of malignancy. Before the treatment, the size of the nodules decreased spontaneously by about 50% in one month and significantly in two months. Although combination chemotherapy, which consisted of CHOP, brought her partial remission, she experienced neurological symptoms 6 months after the initial treatment and died of brain metastasis 9 months after the treatment. CONCLUSIONS: This is a unique case for two following reasons: 1) the first biopsy revealed non-specific findings compatible with erythema nodosum; and 2) before the treatment, the nodules regressed spontaneously. Dermatologists should take multiple skin biopsies for EN lesions with the non-specific histopathological findings not to refute the existence of this disease.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/11. Concurrence of sarcoidosis and aortitis: case report and review of the literature.takayasu arteritis (TA) is a rare manifestation of systemic large vessel vasculitis which affects predominantly the aorta and its main branches, but often remains unrecognised owing to delayed diagnosis and non-characteristic clinical features. sarcoidosis, too, is a systemic inflammatory disease which can affect virtually any organ system. Reports about the coincidence of both diseases have appeared. The case presented here is characterised by a significant time lag between detection of TA and appearance of clinical signs of sarcoidosis. The woman, now 39 years old, had erythema nodosum, circumscript alopecia, and recurrent uveitis, which dated back to 1980 and was attributed to sarcoidosis. At least 12 years later aortic valve insufficiency with progressive cardiac failure developed. histology performed at the time of aortic valve prosthesis in 1997 disclosed a diagnosis of TA, which was confined to the aortic root. Incidentally, sarcoidosis was diagnosed in adjacent lymph nodes. A thorough check up failed to detect further manifestations of TA; thus, possibly, the patients had aortitis similar to, but not identical with, TA.Several related cases previously reported are discussed, suggesting that both diseases may be inherently related as they are characterised by certain non-specific, immunoinflammatory abnormalities. This case report suggests that the prevalence of TA, or related forms of arteritis, may be higher than expected and should be considered, especially in younger patients with non-characteristic cardiovascular symptoms and suspected systemic inflammatory disease. Moreover, the association with sarcoidosis in this and other previously described cases suggests that the two diseases may be related and that TA or TA-like vasculitis may even be a complication of sarcoidosis.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
3/11. Cardiac and great vessel thrombosis in Behcet's disease.Behcet's disease (BD) is a chronic relapsing systemic vasculitis in which orogenital ulceration is a prominent feature. The disease affects many systems and causes hypercoagulability. We present a 27-year-old male patient who exhibited widespread great vessel thrombosis including right atrial and ventricular thrombi in the setting of right-sided infectious endocarditis and orogenital aphthous ulcerations and erythema nodosum due to BD. We reviewed the enigmatic prothrombotic state of BD, and discuss our prior experiences in this field.- - - - - - - - - - ranking = 2.5keywords = vessel (Clic here for more details about this article) |
4/11. Cutaneous polyarteritis nodosa after streptococcal necrotizing fasciitis.polyarteritis nodosa (PAN) is a necrotizing arteritis of small and medium-sized vessels. It may present with hypertension and/or renal insufficiency. Peripheral neuropathy, myopathy, joint pains, testicular pain, and ischemic myalgias may also be seen. Gastrointestinal involvement may lead to gangrene of the bowel, peritonitis, perforation, intra-abdominal hemorrhage, and pancreatitis. The cutaneous manifestations include tender subcutaneous nodules grouped along the course of superficial arteries of the lower extremities, with or without an overlying livedo reticularis. Although multisystem involvement is characteristic, sometimes only one organ or system may be involved. Associations with viral hepatitis (both B and C) and streptococcal infection have been established for PAN. Recurrent strep infections of the upper respiratory tract, streptococcal glomerulonephritis and rheumatic fever have previously been linked to PAN. This report extends the spectrum of associated streptococcal infections to include necrotizing fasciitis.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
5/11. Ultrastructure of keloid: an unusual incident involving lepromatous leprosy.A patient with lepromatous leprosy developed keloids on the dorsum of both arms in response to ulcerations due to acute erythema nodosum leprosum reactions. Electron microscopic examination of the keloidal dermis showed a morphology indicative of increased production of normal collagen fibrils. The greatest cellular changes from normal were in fibroblasts which were enlarged due to increased amounts of rough endoplasmic reticulum and extensive Golgi complexes. Nuclear folds were also evident in these fibroblasts. Some cells, considered to be fibroblasts, were filled with cytoplasmic filaments and contained bizarre shaped nuclei. mast cells, blood vessels and nerve processes were also present.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
6/11. Takayasu's disease with cutaneous involvement.Takayasu's arteritis (TA) is a chronic inflammatory and fibrosing arteriopathy that can also involve cutaneous vessels. The disease typically presents with a prepulseless phase that overlaps or is followed by the characteristic pulseless stage. In both phases of TA, cutaneous manifestations may be present. Lesions considered to be 'specifically' associated with TA have been described most frequently simulating erythema nodosum, erythema induratum and pyoderma gangrenosum. We report 2 Caucasian patients with TA and nodular cutaneous lesions. Nine skin biopsies from these patients were studied. A necrotizing vasculitis was present in 5 biopsies. We review those patients with TA and well-documented cutaneous manifestations in the English literature, with special interest in nodular lesions, the most frequent cutaneous manifestation of TA in Caucasian patients. Biopsies from lesions with similar morphology frequently show different histological findings.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
7/11. Histopathologic spectrum of erythema nodosum.erythema nodosum (EN) is the most common panniculitis and histologically represents the prototype of a septal panniculitis. However, the histologic findings can be quite variable. We describe four patients with EN who each underwent two consecutive biopsies. In each case, the first biopsy showed histopathologic features that fall outside the usual spectrum of disease. Two cases showed predominantly neutrophilic infiltrates with focal suppuration as well as vasculitis of medium-sized arteries. The areas of suppuration were more extensive in the first case prompting special stains for microorganisms that were all negative. The third case demonstrated a lobular panniculitis with a predominantly lymphohistiocytic infiltrate. Special stains were negative in this case as well. The fourth case revealed vasculitis of a medium sized artery, small vessel vasculitis, and a mixed septal and lobular panniculitis with a polyclonal population of atypical lymphocytes. In all patients, the clinical course and the subsequent biopsy were classic for EN. We conclude that lobular neutrophilic panniculitis with suppuration, small vessel vasculitis, and even medium vessel arteritis may rarely occur in EN. There are few clues in these unusual cases that allow for a specific diagnosis from the start, and often, a second biopsy is required.- - - - - - - - - - ranking = 1.5keywords = vessel (Clic here for more details about this article) |
8/11. Clinical and histologic variations among thirty patients with Lucio's phenomenon and pure and primitive diffuse lepromatosis (Latapi's lepromatosis).The clinical and histologic experience with 30 patients who had Lucio's phenomenon, and pure and primitive diffuse lepromatosis (Latapi's lepromatosis) has been reviewed. The unanticipated clinical findings were a male to female ratio of nearly 1:1, a 21 month median time of onset of erythema nodosum leprosum (Type 2 reaction) after starting antibacterial treatment, and an absence of a stocking-glove pattern of anesthesia in 7 patients. The only unanticipated histologic finding was a lepromatous-granulomatous vasculitis, occurring in comparatively large vessels, or in vessels made large by pathologic changes, located near the dermal-subcutaneous interface. This finding was present in 6 of the 22 patients with histologic material available for review. In 2 of these 6 this vasculitis was identified before the onset of Lucio's phenomenon. With one conspicuous exception, the onset of treatment with a microbicidal agent was associated with a cessation of new lesions of Lucio's phenomenon within one week. Long-term morbidity, other than Type 2 reaction, was found in 22 of the 25 patients followed for more than 1.3 years. Usually this was the consequence of Latapi's lepromatosis, specifically venous insufficiency and/or loss of protective sensation, and only rarely from Lucio's phenomenon, specifically scar formation. Briefly summarized are the seven patients who had had a skin biopsy before the onset of Lucio's phenomenon, as well as the two patients who were considered to be atypical. Criteria for the diagnosis of Latapi's lepromatosis, in the absence of Lucio's phenomenon, are also considered.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/11. Cutaneous manifestations of takayasu arteritis. A retrospective study of 80 cases.The aim of this retrospective study is to delineate in europe the frequency and type of cutaneous manifestations associated with takayasu arteritis (TA). Eighty patients with TA were analyzed. Symptoms suggestive of Raynaud's syndrome were noted in 11 patients (14%) and could be directly related to large vessel involvement. Other skin lesions were observed in 10 patients (12.5%). Five had acute tender erythematous nodules on the legs with a clinical diagnosis of erythema nodosum; 2 had subacute ulcerated nodules of the legs; 1 had pyoderma-gangrenosum-like ulcerations of the four limbs which resulted from the breakdown of subcutaneous nodules; 1 had lupus-like malar flush, and the last one had urticarial lesions with livedo reticularis. skin samples were obtained from 4 patients. Three of them agreed that reiterated biopsies be done on recurrent lesions. A granulomatous vasculitis was observed in 2 cases involving hypodermal arterioles in one case and veins in the other. The other pathological findings were septal and lobular panniculitis which can be associated with granulomatous vasculitis. Different histological findings on reiterated biopsies were frequently found. The absence of any other etiology and chronological arguments suggested a relationship between these skin lesions and TA. tuberculosis was probable in 1 case but apparently was not related to the skin lesions.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
10/11. Mycobacterial subcutaneous arteritis.The authors report three patients with subcutaneous erythematous nodules in different phases of development, unspecific systemic symptoms, positive PPD test, and normal chest x-rays. The histopathological study of the older nodules showed a granulomatous arteritis with a few acid-fast bacilli in the vascular wall. The nodules at an early phase showed an unspecific panniculitis with some acid-fast bacilli in apparently normal cutaneous vessels. These findings suggest that the mycobacterium has a vascular tropism and may cause a primary granulomatous arteritis.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
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