Cases reported "Purpura"

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1/33. Intravascular and diffuse dermal reactive angioendotheliomatosis secondary to iatrogenic arteriovenous fistulas.

    Reactive angioendotheliomatosis is a rare benign process that has been mainly described in patients with systemic infections, such as subacute bacterial endocarditis or tuberculosis, and in association with intravascular deposition of cryoproteins. Histopathologically, it is characterized by a proliferation of endothelial cells within vascular lumina resulting in the obliteration of the involved vessels. Another rare variant of reactive angioendotheliomatosis has been described in the lower extremities of patients with severe peripheral vascular atherosclerotic disease. It consists of violaceous and purpuric plaques histopathologically characterized by diffuse proliferation of endothelial cells interstitially arranged between collagen bundles of the reticular dermis. This second variant has been named diffuse dermal reactive angioendotheliomatosis. We report two patients with reactive cutaneous angioendotheliomatosis appearing distally to arteriovenous fistulas used for hemodialysis because of chronic renal failure. The first patient showed intravascular reactive angioendotheliomatosis, while the second one had purpuric plaques that were characterized histopathologically by diffuse dermal angioendotheliomatosis. Both patients showed an arteriovenous "steal" syndrome with distal ischemia, and it is possible that a local increase of vascular endothelial growth factor, as is the case in hypoxia situations, induces the endothelial proliferation. To the best of our knowledge, cutaneous reactive angioendotheliomatosis has not been previously described in association with arteriovenous shunts.
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2/33. Acute infantile hemorrhagic oedema.

    Acute infantile hemorrhagic oedema (AIHO) was first described in 1913 but, despite frequent reports in the European literature, it is not well recognized in the English language literature. It is considered by many to be a variant of Henoch Schonlein purpura (HSP) because of similarities in cause and histopathology. However, because of the benign nature of this condition and frequent absence of IgA associated with HSP, it may be sensible to consider this as a distinct variety of cutaneous small vessel vasculitis (CSVV). We report this case to highlight the condition and emphasize its benign nature.
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3/33. Duodenojejunitis: is it idiopathic or is it Henoch-Schonlein purpura without the purpura?

    BACKGROUND: Henoch-Schonlein purpura is a small-vessel vasculitic disease that most often affects the skin. Gastrointestinal manifestations have been well described, including duodenojejunal inflammation (DJI). methods: Four children with DJI and clinical features of HSP are described, in whom the rash was either not present or appeared atypically late in the illness. RESULTS: The characteristic rash did not develop in three children, and it developed much later in one. The patients (three boys and one girl) were aged between 7 and 9 years (mean, 7.5 years). growth characteristics were normal. In all patients, pain occurred acutely with colicky abdominal pain in the spring or fall of the year, and all stools were positive for occult blood. No infectious cause was identified. Upper gastrointestinal endoscopic examinations demonstrated significant visual and histologic duodenitis in a pattern consistent with previous reports in children with known HSP. factor xiii activity was absent. immunoglobulin a levels were increased in three of four children. All children made a prompt recovery with the administration of intravenous glucocorticoids. In one child, the characteristic rash of HSP developed 18 weeks after the initial examination. CONCLUSION: Duodenojejunal inflammation may be the primary manifestation of HSP, even in the absence of the characteristic rash.
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4/33. Palpable purpura at the site of previous herpes zoster in association with mixed cryoglobulinemia and hepatitis c virus infection.

    A 70-year-old woman affected with chronic active hepatitis c and mixed cryoglobulinemia presented a palpable purpura on her abdominal skin in a metameric configuration, fourteen months after a herpes zoster in the same site. Histopathology showed a small vessel leukocytoclastic vasculitis in the superficial dermis. Post-zoster eruptions are variable, and their spectrum is still expanding, although the pathogenesis remains to be elucidated. Perhaps our case represents an isomorphic reaction, because this palpable purpura, probably related to HCV infection, occurred several months after herpes zoster.
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5/33. Chronic pigmented purpura associated with odontogenic infection.

    Five patients with chronic pigmented purpura associated with odontogenic infection are described. Four patients had Schamberg's disease, and one had itching purpura. These patients were resistant to topical corticosteroid treatment, but appearance of purpuric spots ceased after treatment for periodontitis, pulpitis, or both. No circulating immune complexes were detected, and neither immunoglobulin nor complement was deposited in the papillary vessels of the skin. Odontogenic infection might be one of the precipitating factors for chronic pigmented purpura.
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6/33. Segmental pigmented purpura.

    A segmental distribution of pigmented purpura (PP) is rare. Our patient presented at 5 years of age with red-brown petechial macules and telangiectases on her chest, the inner and dorsal aspects of the right upper extremity, and the dorsal aspect of the hand, which had developed slowly over the previous 7 months. The lesions were intermittently pruritic. A biopsy from the right arm revealed discrete hydropic degeneration of the basal layer of the epidermis, dilation of the blood vessels, numerous extravasated erythrocytes and lymphocytes, and lymphocytes in the lower part of the epidermis. The skin disease resolved almost completely within 18 months. The general physical examination revealed an open foramen ovale, slight hypogammaglobulinemia (G and A), and enhanced fragility of the blood vessels.
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keywords = blood vessel, vessel
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7/33. purpura with cold urticaria in a patient with hepatitis c virus infection-associated mixed cryoglobulinemia type III: successful treatment with interferon-beta.

    We describe a 54-year-old man with hepatitis c virus (HCV) infection-associated cryoglobulinemia type III. The patient had suffered from cold-induced urticaria that left purpuric eruptions up to 1 cm in diameter, intermittent migratory joint pain for seven years and mild liver dysfunction for nine years. hemophilia a was diagnosed when the patient was 26 years old, and he was then given infusions of factor viii for a short time. In both skin biopsy samples from urticarial and purpuric eruptions, mild inflammatory infiltration by polymorphonuclear leukocytes with nuclear dust, extravasation of erythrocytes and deposition of IgM and C3 in the superficial blood vessels were observed. After antiviral treatment with interferon-beta, the clinical symptoms and the cryoglobulin and HCV-rna in the serum disappeared. There has been no recurrence in the subsequent nine years.
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keywords = blood vessel, vessel
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8/33. Chronic vulvar purpura: persistent pigmented purpuric dermatitis (lichen aureus) of the vulva or plasma cell (Zoon's) vulvitis?

    BACKGROUND: Lichen aureus is localized variant of persistent pigmented purpuric dermatitis that typically affects the legs and can be associated with delayed hypersensitivity reactions or vascular abnormalities. plasma cell vulvitis (Zoon's vulvitis) is a rare condition that frequently contains hemosiderin deposits and is suspected to be a mucosal reaction pattern due to variety of insults, most often local irritation or trauma. CASE REPORT: A 50-year-old female with longstanding complaints of spotting, vulvar dryness, irritation, and dyspareunia presented with circumscribed, purpuric, erythematous vulvar patches. Past estrogen cream treatment evoked symptoms of discomfort. On biopsy, siderophages and extravasated red blood cells were found in conjunction with a lichenoid, lymphocyte and plasma cell infiltrate, and dilated dermal and intraepithelial vessels. CONCLUSIONS: Reported herein is an unusual vulvar dermatosis that is best classified as a localized variant of persistent pigmented dermatosis (lichen aureus) but overlaps clinically and histologically with Zoon's vulvitis. This constellation of findings may represent a site-specific mucosal reaction to an erosive process that could either be inflammatory (hypersensitivity reaction) and/or traumatic in nature.
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9/33. Intractable livedoid vasculopathy successfully treated with hyperbaric oxygen.

    We describe a new method for treating livedoid vasculopathy. The typical presentation of livedoid vasculopathy includes chronic, recurrent painful ulcers, satellite scar-like atrophy and telangiectasia involving the lower extremities. Histologically, these lesions show areas of ulceration and dermal vessel occlusion without frank inflammatory cell infiltration. There is currently no satisfactory therapy available for this disease. Hyperbaric oxygen (HBO) has recently established itself as one of the most effective methods of treating ischaemic wounds, including diabetic ulcers. We used this therapy in two patients whose lesions were resistant to multiple therapeutic modalities. Not only did their ulcers respond rapidly to the HBO therapy, but the disturbing wound pain also resolved at the same time. To our knowledge, this is the first successful trial of HBO therapy in livedoid vasculopathy. We believe this to be a very promising new therapy for livedoid vasculopathy and to be worth further investigation.
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10/33. Fatal fulminant hypereosinophilic syndrome with a petechial rash in a 16-year-old girl.

    Peripheral blood eosinophilia and eosinophilic tissue infiltration characterize the hypereosinophilic syndrome, which if untreated can be fatal. Its manifestations are protean. Often a diagnosis of exclusion, it presents diagnostic challenges. A fatal case of hypereosinophilic syndrome in a 16-year-old girl with petechiae, edema, urticaria, and diffuse erythema unfolded over 2 weeks. The histopathology of the petechiae demonstrated an eosinophilic and mixed cell interstitial and perivascular infiltrate and microthrombi in vessels. There was no evidence of T-cell clonality. Despite treatment with cyclosporine and methylprednisolone, she died. The age group; fulminant, fatal course without neoplasia; striking eosinophilia; and petechial rash are notable.
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