Cases reported "Disease Progression"

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1/21. Follicular mycosis fungoides.

    We describe a patient with follicular mycosis fungoides (MF), a rare folliculotropic variant of cutaneous T-cell lymphoma (CTCL). Follicular involvement in CTCL usually presents clinically as alopecia mucinosa associated histologically with follicular mucinosis. Follicular MF differs from alopecia mucinosa/follicular mucinosis associated with MF with regard to its clinical presentation, histology and, presumably, prognosis. Our patient presented with the characteristic findings of follicular MF, i.e. infiltrated plaques showing numerous enlarged, comedo-like follicular infundibula; histology was dominated by exclusive folliculotropism of atypical lymphocytes sometimes forming follicular Pautrier's microabscesses, and by lack of epidermotropism and follicular mucinosis. Despite photochemotherapy and treatment with oral retinoids and interferon alpha, the patient's follicular MF rapidly developed into a progressive CTCL with large tumorous lesions, but responded to electron beam therapy. The course of our patient's disease confirms the notion that follicular MF may be associated with a worse prognosis than classical MF. However, electron beam irradiation induced remission of follicular MF that was maintained by a combination therapy consisting of extracorporeal photopheresis and interferon alfa.
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ranking = 1
keywords = mycosis fungoides, fungoides, mycosis
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2/21. Photosensitive mycosis fungoides or actinic reticuloid?

    We report two patients who satisfied the diagnostic criteria for actinic reticuloid (AR) on initial presentation, in whom genotypic analysis of early skin biopsies failed to show T-cell gene receptor rearrangements. Both patients progressed to widespread skin involvement associated with histopathological and genotypic features of mycosis fungoides (MF). Arguably, these patients may have had photosensitive MF from the outset, but their clinical features, phototesting, and subsequent demonstration of a T-cell gene receptor rearrangement in the skin could also suggest progression of AR to MF.
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ranking = 1
keywords = mycosis fungoides, fungoides, mycosis
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3/21. Rapidly progressing mycosis fungoides presenting as follicular mucinosis.

    Follicular mucinosis can occur as a primary idiopathic disorder or can arise in association with benign or malignant disease, most notably mycosis fungoides. We describe a patient with an aggressive folliculotropic variant of mycosis fungoides that initially presented as follicular mucinosis with alopecia. One month after the diagnosis of follicular mucinosis, a diagnosis of mycosis fungoides was made, and 3 months later inguinal lymph node involvement with mycosis fungoides developed. A skin biopsy specimen demonstrated prominent follicular mucinosis with folliculotropism of atypical cells and intrafollicular Pautrier's microabscesses. As demonstrated in this case, follicular mucinosis can be a presenting sign of rapidly progressive mycosis fungoides. In our review of follicular mucinosis and its association with mycosis fungoides, we found that the folliculotropic variant of mycosis fungoides appears more commonly to have an aggressive course than classic mycosis fungoides.
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ranking = 2.4
keywords = mycosis fungoides, fungoides, mycosis
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4/21. mycosis fungoides d'emblee: CD30-negative cutaneous large T-cell lymphoma.

    The d'emblee variant of mycosis fungoides initially described patients with a rapid onset of tumors without progression through patch- and plaque-stage disease. We report a case of a patient with the clinical presentation of mycosis fungoides d'emblee and correlate the histologic and immunophenotypic data with those of a more updated classification scheme.
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ranking = 0.41466191392261
keywords = mycosis fungoides, fungoides, mycosis
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5/21. Localized syringolymphoid hyperplasia with alopecia and anhidrosis.

    We describe a female patient with a localized form of syringolymphoid hyperplasia with alopecia and anhidrosis (SLHA). This woman is the eleventh patient with this disease so far reported in the literature. She suffered from a slowly but continuously progressing single lesion on her right ankle. It took 7 years until the diagnosis of SLHA could be established. Many divergent diagnoses were assumed and different treatments were performed during this time. The final diagnosis was established by histopathology revealing syringotropic T-cell infiltrates. Clinical features were scattered brownish papules, which formed a sharply demarcated erythematous patch lacking hairs and sweat production. The progressive course of the disease and the unresponsiveness to treatments support the current view that SLHA is a syringotropic variant of mucinosis follicularis and therefore should be viewed as a facultative precursor lesion of mycosis fungoides. In our patient, during a 7-year follow-up, no T-cell lymphoma occurred. This case emphasizes the difficulties of establishing the diagnosis of SLHA, which requires cooperation between the dermatologist and dermatopathologist.
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ranking = 0.2
keywords = mycosis fungoides, fungoides, mycosis
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6/21. diagnosis of disseminated zygomycosis using a polymerase chain reaction assay.

    Invasive pulmonary zygomycosis is an uncommon opportunistic infection in patients with haematological malignancies. Clinical manifestations are in distinguishable from the more frequent invasive aspergillosis. Standard diagnostic methods like culture and microscopy from respiratory secretions have a low diagnostic sensitivity. A case in which proven invasive pulmonary zygomycosis was confirmed using a panfungal polymerase chain reaction assay in blood is presented. Since zygomycosis requires more aggressive treatment than aspergillosis (high-dose amphotericin b and surgical intervention), the polymerase chain reaction assay may improve the outcome of these often fatal infections by guiding the therapeutic approach through an early, non-invasive diagnosis.
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ranking = 0.099686423768852
keywords = mycosis
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7/21. Common clonal T-cell origin in a patient with T-prolymphocytic leukaemia and associated cutaneous T-cell lymphomas.

    An unusual course was observed in a patient with indolent T-prolymphocytic leukaemia (T-PLL) who subsequently developed mycosis fungoides (Mf), lymphomatoid papulosis (LyP) and cutaneous CD30 anaplastic large cell lymphoma (ALCL). polymerase chain reaction analysis demonstrated identical monoclonal T-cell receptor-beta and -gamma gene rearrangements in all the different clinical entities. Furthermore, cytogenetic studies revealed the same aberrant clone with trisomy of chromosome 8 in T-PLL and ALCL cells. This unique observation suggests that in T-PLL, the leukaemic cells might undergo secondary transformation, subsequently resulting in different phenotypes of cutaneous T-cell lymphoma.
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ranking = 0.2
keywords = mycosis fungoides, fungoides, mycosis
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8/21. mycosis fungoides progression and chronic solvent exposure.

    The effect of repeated exposure to specific chemicals on the initiation or progression of mycosis fungoides (MF) remains unsettled. A patient with low-grade patch stage MF progressively developed MF plaques restricted to his arms, and a tumour on his right thigh. These areas were subject to repeated exposure to solvents. His thigh was indeed in close contact with his trousers pocket where he used to store a wiping rag drenched into white spirit and cellulosic thinner. immunophenotyping these lesions revealed a dense LCA , CD2 , CD3 , CD4 , CD5 , CD7 , CD45 , CD45RO T-cell infiltrate admixed with many factor xiiia dendrocytes. T-cell receptor rearrangement analysis identified a monoclonal T-cell infiltrate. An internal work-up remained negative. Stopping further solvent exposure failed to improve his condition. Oral corticotherapy combined with low-dose interferon-alpha2a halted disease progression. This observation suggests that long-term solvent exposure may trigger MF and hasten its progression from the patch stage to the plaque and tumour stages.
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ranking = 0.36033679699308
keywords = mycosis fungoides, fungoides, mycosis
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9/21. Follicular mycosis fungoides mimicking a cutaneous B-cell lymphoproliferative disorder.

    Follicular mycosis fungoides (MF) is an uncommon histological variant of MF characterized by infiltrates of atypical lymphocytes around and within the epithelium of the hair follicles (folliculotropism). Here we report a patient with rapidly progressive follicular MF on the face, associated with concurrent typical MF lesions elsewhere. The histology was unusual, as apart from dense lymphoid infiltrates showing folliculotropism and epidermotropism, there was a prominent B-cell component with germinal centres, leading to an initial diagnosis of cutaneous B-cell lymphoma. The final diagnosis of follicular MF was established on demonstration of clonal T-cell receptor gene arrangements and lack of clonality for heavy chain gene rearrangements. This case illustrates a variant of MF that has a more rapid progression than the otherwise indolent course of classical MF over many years, and the diagnostic pitfalls, whereby the histology can mimic a B-cell proliferative disorder.
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ranking = 1
keywords = mycosis fungoides, fungoides, mycosis
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10/21. Thoracic actinomycosis presented with tracheoesophageal fistula and fatal pulmonary infection.

    We report a case of a 60-year-old Saudi woman who developed tracheoesophageal fistula and fatal pulmonary infection secondary to thoracic actinomycosis. The cause, clinical presentation, radiological features and treatment of thoracic actinomycosis are discussed.
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ranking = 0.085445506087587
keywords = mycosis
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