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Marian T McEvoy

Publications and source records attributed to Marian T McEvoy.

11 recordsLinked to original sources

Early cutaneous gene transcription changes in adult atopic dermatitis and potential clinical implications.

Atopic dermatitis (AD) is a common pruritic dermatitis with macroscopically non-lesional skin that is often abnormal. Therefore, we used high-density oligonucleotide arrays to identify cutaneous gene transcription changes associated with early AD inflammation as potential disease control targets. Skin biopsy specimens analysed included normal skin from five healthy non-atopic adults and both minimally lesional skin and nearby or contralateral non-lesional skin from six adult AD patients. Data were analysed on an individual gene basis and to identify biologically relevant gene networks. Transcription levels of selected genes were also analysed by quantitative PCR. Differential transcription occurring early in AD skin was indicated for (i) individual genes such as C-C chemokine ligand (CCL)18, CCL13, and interferon-alpha2 (IFNalpha2), (ii) genes associated with peroxisome proliferator-activated receptor (PPAR)alpha- and PPARgamma-regulated transcription, and possibly for (iii) immunoglobulin J-chain and heavy chain isotype transcripts. These data suggest that local changes in immunoglobulin-associated transcription may favour IgE over secretory immunoglobulin (multimeric IgM and IgA) expression in AD skin. Decreased PPAR activity appears common to both AD and psoriasis, and reduced cutaneous IFNalpha2 transcription also appears characteristic of AD. Identification of these genes and pathways will direct future research towards controlling AD.

Adult↗

Bexarotene treatment of late-stage mycosis fungoides and Sézary syndrome: development of extracutaneous lymphoma in 6 patients.

Bexarotene is a retinoid drug that is approved for the treatment of cutaneous T-cell lymphoma. We report 6 cases in which the initiation of bexarotene therapy for cutaneous T-cell lymphoma was temporally associated with the progression of internal disease despite improvement in cutaneous signs and symptoms. It is possible that bexarotene contributed to this progression. Although bexarotene therapy may alleviate symptoms and signs of cutaneous T-cell lymphoma, careful surveillance of lymph nodes and solid organs during treatment is advised.

Adult↗

Neutrophilic dermatosis of the hands: four new cases and review of the literature.

BACKGROUND: Isolated or predominantly hand involvement in Sweet's syndrome, pyoderma gangrenosum, or pustular vasculitis is a rare presentation in the spectrum of neutrophilic dermatoses and is often associated with an occult malignancy or other systemic inflammatory disorder. When these disorders occur on the hands, they are often clinically indistinguishable, but they can sometimes be separated histologically by the presence of papillary dermal edema (Sweet's syndrome), ulceration and necrosis (pyoderma gangrenosum), or vasculitis (pustular vasculitis). These distinctions may be arbitrary, however, and reflect differences in the temporal course of the disease and in the degree of inflammation at the time of biopsy. METHODS: We report four cases of neutrophilic dermatosis affecting the hands and a review of the literature for similar cases. RESULTS: Of the four patients presented, two had associated carcinomas and one had myelodysplasia in transition to leukemia. The cutaneous symptoms preceded the finding of an occult malignancy. Thirty-two reported cases of neutrophilic dermatoses presenting on the hands showed clinicopathologic features similar to those in our series. Taken together, the mean age at diagnosis was 60.5 years, and 58% of the patients were female. Twenty-five per cent (nine patients) also had myelodysplasia or leukemia, 14% (five patients) ulcerative colitis, 6% (two patients) carcinoma, 6% (two patients) Crohn's disease, and 6% (two patients) seropositive arthritis. CONCLUSIONS: These cases illustrate the importance of recognizing that neutrophilic dermatoses may present uniquely or predominantly on the hands. This presentation is distinctive, and prompt diagnosis may prevent unnecessary medical or surgical therapy and may lead to the earlier diagnosis and treatment of an associated malignancy or other systemic disorder.

Aged↗

Topical tacrolimus in the treatment of symptomatic oral lichen planus: a series of 13 patients.

BACKGROUND: Oral lichen planus (OLP) is a relatively common, chronic inflammatory condition, which frequently presents with symptoms of pain and irritation. OLP is often difficult to manage. Therefore there is a need for more effective and safer therapies for symptomatic OLP. OBJECTIVE: Our purpose was to determine the effectiveness of topical tacrolimus as therapy for symptomatic OLP. METHODS: A retrospective review was performed of 13 patients with symptomatic OLP treated with topical tacrolimus between September 1999 and September 2000. RESULTS: Eleven of the 13 patients reported definite symptomatic response to treatment and 2 had no response. Eight patients had a partial response, whereas 3 patients had a complete response with respect to lesion clearance. Seven of the responding patients had no flares with continued treatment. The other 4 patients noted flares soon after stopping the treatment. Side effects were rare and minor. CONCLUSIONS: In this retrospective case series of 13 patients, topical tacrolimus was well tolerated and appeared to be an effective therapy to control symptoms and clear lesions of OLP.

Administration, Cutaneous↗

Effect of insulin-dependent diabetes mellitus on response to extracorporeal photopheresis in patients with Sézary syndrome.

BACKGROUND: Extracorporeal photopheresis (ECP) has become a primary therapy for selected forms of cutaneous T-cell lymphoma, especially Sézary syndrome. Variability in response of patients with Sézary syndrome to ECP has been reported. OBJECTIVE: Our purpose was to determine whether underlying medical conditions influence the efficacy of ECP in patients with Sézary syndrome. METHODS: We retrospectively reviewed the medical records of 55 patients with Sézary syndrome who received ECP between 1987 and 2000. Efficacy criteria included decrease in Sézary cell count, erythroderma, lymphadenopathy, organomegaly, and pruritus. RESULTS: Thirty-four patients responded well and 10 patients responded partially to ECP; 11 patients had no response. Nine patients with no response to ECP had insulin-dependent diabetes mellitus (IDDM). IDDM was documented in only 2 patients with a good response and in no patients with a partial response to ECP. CONCLUSION: Patients with Sézary syndrome and IDDM typically respond poorly to the standard ECP treatment regimen.

Adult↗

Extracorporeal photopheresis and adjuvant aerosolized granulocyte-macrophage colony-stimulating factor for Sézary syndrome.

Encouraged by preliminary phase 1 studies of aerosolized granulocyte-macrophage colony-stimulating factor (GM-CSF, sargramostim) in the treatment of patients with melanoma and other malignancies, we treated a 72-year-old patient with Sézary syndrome, using alternate-week cycles of aerosolized GM-CSF in combination with monthly cycles of extracorporeal photopheresis (ECP). Sézary syndrome, one of the more aggressive forms of cutaneous T-cell lymphoma, is a devastating and highly symptomatic form of non-Hodgkin lymphoma in which malignant clones of mature helper CD4 T cells, containing large, convoluted nuclei known as Sézary cells, circulate in the blood and infiltrate skin. Extracorporeal photopheresis, an immunomodulatory therapy, has become a primary treatment for patients with Sézary syndrome. This pheresis-based therapy combines psoralen and ultraviolet A radiation as systemic photochemotherapy to induce immune responses. However, the activity and efficacy of ECP vary considerably. To our knowledge this is the first patient with Sézary syndrome treated with adjuvant aerosolized GM-CSF combined with ECP. It produced clinical improvement and decreased the number of circulating Sézary cells in a previously ECP-refractory patient.

Administration, Inhalation↗

Treatment of late-stage Sézary syndrome with 2-Chlorodeoxyadenosine.

BACKGROUND: 2-Chlorodeoxyadenosine (2-CdA), a purine adenosine analog, is safe and effective chemotherapy for patients with hairy cell leukemia and low-grade lymphomas. Adverse effects include neutropenia, lymphocytopenia, and infectious complications. Our objective was to evaluate the efficacy of 2-CdA (2-6 seven-day cycles) in the treatment of late-stage, recalcitrant Sézary syndrome. METHODS: Retrospective review of medical records of six patients with Sézary syndrome who had received 2-CdA cycles at Mayo Clinic, Rochester between March 1995 and March 2000. Variables assessed from the records included improvement in global appearance, extent of erythroderma, size of lymph nodes, pruritus, and leukocyte, lymphocyte, and absolute Sézary cell counts. RESULTS: Two patients, both with stage III Sézary syndrome, whose previous treatment consisted of only two modalities, responded well to the treatment, with moderate to total clearing of erythroderma and pruritus associated with a significant decrease in Sézary cell counts. The other four patients had only a partial response (one patient) or no response (three patients) to 2-CdA. The mortality rate was 50%. All three patients died of Staphylococcus aureus sepsis. However, only one patient was receiving 2-CdA treatment when he died. The other two patients died 8 and 9 weeks after the last 2-CdA cycle. This high mortality rate is attributed to infectious complications after 2-CdA treatment in patients with recalcitrant disease. CONCLUSION: 2-Chlorodeoxyadenosine shows efficacy in stage III Sézary syndrome, but it also carries a substantial risk of septic complications and mortality. It can be used if no other suitable alternatives are available. Caution should be exercised in all these patients regarding skin care and avoidance of infections or sepsis.

Aged↗

Sustained remission of Sézary syndrome.

Sézary syndrome, an aggressive form of cutaneous T-cell lymphoma, is a devastating, highly symptomatic form of non-Hodgkin lymphoma. Malignant clones of mature helper CD4 T cells containing large, convoluted nuclei known as Sézary cells circulate in the blood and infiltrate the skin. Clinical features include exfoliative erythroderma, generalized lymphadenopathy, alopecia, onychodystrophy, palmoplantar hyperkeratosis, and ectropion. Patients often have severe pruritus, burning sensations, pain, bleeding from excoriations, and disfigurement. Extracorporeal photopheresis, an immunomodulatory therapy, has become a primary therapy for these patients. This pheresis-based therapy uses psoralen and ultraviolet A radiation-mediated photochemotherapy to induce immune responses. The effects of extracorporeal photopheresis vary considerably. We report sustained remission (2 years) in a patient with Sézary syndrome. Previously he had received extracorporeal photopheresis and interferon alfa-2b injections. He is the only one of 55 patients with Sézary syndrome treated at Mayo Clinic (Rochester, Minnesota, USA) to achieve sustained remission on extracorporeal photopheresis alone.

Aged↗