17 research outputs found

    widespread livedoid vasculopathy

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    A 37-year-old woman with a 13-year history of widespread livedo reticularis and recurrent, painful ulcerative skin lesions was referred to our department because of a relapse of cutaneous manifestations of the skin lesions involving almost the whole body surface; malar erythema and oedema, non-scarring alopecia and fever were also associated. Routine laboratory data, immunological investigations and coagulation parameters were normal or negative. Histology was consistent with livedoid vasculopathy. A good clinical response was obtained using intravenous methylprednisolone combined with pentoxifylline. Livedoid vasculopathy is a rare, distinctive dermatosis that can be associated with systemic autoimmune disorders or present in an ''idiopathic'' form. The latter is at present regarded as a non-inflammatory thrombotic disease that may occur in patients with coagulation abnormalities. It is noteworthy that, in the present case, despite long-standing and dramatic cutaneous features, serious systemic complications have not developed and the patient's seroimmunologic and coagulative profile has remained normal. Key words: autoimmune diseases; coagulation abnormalities; livedoid vasculopathy

    Anetodermic lupus panniculitis and antiphospholipid antibodies: Report of three cases

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    Anetoderma is a rare cutaneous disease characterized by a loss of normal elastic tissue that is presented clinically as localized areas of wrinkled or flaccid skin. This form may be associated with several immunological abnormalities, most notably lupus erythematosus and antiphospholipid antibodies with or without clinical manifestations of the antiphospholipid syndrome. A retrospective study was conducted with the aim of summarizing the clinical characteristics, course and laboratory findings in three women with anetodermaassociated lupus erythematosus panniculitis, an unusual variant of cutaneous lupus erythematosus. The 3 patients (of the 12 patients with lupus erythematosus panniculitis seen by us since 1990) were all at a young age at onset of panniculitis (median, 22 years). None of the patients developed severe systemic involvement up to 9 years (median, 5 years) from onset of the disease. The most noteworthy laboratory finding was the presence of antiphospholipid antibodies. Anetodermic lupus erythematosus panniculitis may be regarded as an uncommon variant of cutaneous lupus erythematosus mainly affecting young females and showing a favourable clinical course, although the patients should be followed and screened for the emergence of antiphospholipid syndrome. Antiphospholipid antibodies could play a role in the elastolytic process, leading to anetoderma. Key words: anetoderma; antiphospholipid antibodies; lupus erythematosus; panniculitis

    nodal peripheral t cell lymphoma with secondary granulomatous cutaneous involvement

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    Sir, The presence of a granulomatous pattern has rarely been described in cutaneous lymphomas, mycosis fungoides (MF) being the variant more frequently reported (1 ā€“ 4). The aetiology of granulomatous reactions remains unclear and its prognostic value is still disputed, although a favourable influence on outcome has been hypothesized (5). We report here the case of an 87-year-old man with nodal peripheral T-cell lymphoma (PTL) with secondary cutaneous involvement, who clinically presented with multiple eruptive papular and nodular skin lesions on the upper part of the body and histologically showed a strong granulomatous reaction in the skin

    Ichthyosiform Mycosis fungoides

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    Background: Mycosis fungoides (MF) is a skin malignancy of T helper lymphocytes with a wide clinical spectrum. Among the atypical variants of MF, there is an ichthyosis-like presentation. However, to date, only 1 case of ichthyosiform MF has been reported. Objective: Our goal was to summarize the clinical characteristics and course, and the pathological, immunohistochemical and molecular genetic findings on 4 patients with ichthyosiform MF. Methods: A retrospective study was conducted. Results: The 4 patients represented 1.8% of the 221 patients with MF seen by us since 1975. None progressed to systemic disease in up to 12 years (median, 10 years) after the onset of the cutaneous manifestations. Interestingly, skin lesions typical of so-called follicular MF (FMF) were associated in 3 of 4 cases, whereas cutaneous manifestations of classic MF were absent in all 4 patients. Conclusion: Ichthyosiform MF represents a rare variant within the clinicopathologic spectrum of MF usually featuring a benign course and a tendency to be associated with lesions of FMF but not with lesions of classic MF. Copyright Ā© 2002 S. Karger AG, Basel
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