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PubMedDate not supplied

Mycosis fungoides presenting as pigmented purpuric dermatitis.

Mycosis fungoides, a cutaneous T-cell lymphoma, typically presents as indolent, progressive, and persistent erythematous patches or plaques with mild scaling and over time can evolve into tumor stage with tumor nodules. Other presentations include eczematous, psoriasiform, poikilodermatous, and hypopigmented patches. We report Mycosis fungoides in a 14-year-old boy presenting as pigmented purpuric dermatitis and review the relevant literature. This is a rare presentation of a condition that is uncommon in the pediatric population. In our patient, histologic features were typical of Mycosis fungoides presenting as pigmented purpuric dermatitis. The clinical features, pathology, molecular biology, and the relationship between these two entities are discussed.

Adolescent↗
PubMed2002

Mycosis fungoides involving the oral cavity.

Mycosis fungoides is a malignant T-cell lymphoproliferative disease with a predilection for cutaneous involvement. Extracutaneous disease is uncommon and oral mucosal involvement is rare. We describe a case of mycosis fungoides involving the hard palate treated with radiotherapy. The relevant literature on this topic is reviewed.

Aged↗
PubMed1988

Gallium-67 uptake in cutaneous lesions of mycosis fungoides.

The literature on gallium imaging in mycosis fungoides is limited and conflicting. A case of mycosis fungoides with increased uptake of Ga-67 in clinically noninfected skin lesions is reported. The literature regarding mycosis fungoides and gallium imaging is reviewed.

Aged↗
PubMedDate not supplied

Mycosis fungoides and the Sézary syndrome: pathology, staging, and treatment.

Mycosis fungoides and the Sézary syndrome are forms of cutaneous T-cell lymphoma. Mycosis fungoides is an uncommon disease: only about 500 new cases are diagnosed in the United States annually. The median age of onset is 55 years and there is a 2:1 male predominance. The etiology of mycosis fungoides is unknown. Although occupational exposures have been implicated, case control studies fail to support this hypothesis. Mycosis fungoides is typified by cutaneous plaques which may evolve into tumors over the course of time. It is often preceded by a lengthy pre-mycotic phase prior to the time of definitive diagnosis. In its earliest diagnostic phase, there may only be slightly scaling patches with a limited distribution. Indurated lesions evolve into plaques, which may become more generalized in their distribution. As the severity of skin involvement increases, there is an increasing likelihood of spread to extracutaneous sites. The pathology of this disease is marked by involvement of the epidermis (Pautrier microabscesses). Immunologic studies characterize these cells as belonging to the helper T-cell subset. Genotypic analysis demonstrates monoclonal rearrangements of the T-cell receptors of the infiltrating cells. The staging system for mycosis fungoides considers the extent of skin involvement, presence of lymph node or visceral disease, and detection of abnormal cells in the peripheral blood. Patients with disease limited to the skin (90% of newly diagnosed cases) are treated best with topical or cutaneous therapies. Common modalities include psoralen photochemotherapy (PUVA), topical chemotherapy (nitrogen mustard) and total skin electron beam therapy. Both topical nitrogen mustard and electron beam therapy have good initial response rates (73% and 100%) and may achieve long-term disease-free survival, especially in patients with initially limited disease. Even if the response is incomplete or relapse occurs, substantial and very important palliation is generally achieved with topical therapy. Recurrent or resistant cutaneous disease will require the use of sequential topical treatment. The median survival time of patients who present with disease limited to the skin is greater than 10 years, and many deaths in this group are from intercurrent causes, especially in patients with limited or generalized plaque disease. If cutaneous tumors are present, the majority of these patients will eventually die from disease-related causes. The prognosis of patients who develop extracutaneous disease is exceedingly poor (median survival time, approximately 1 year).(ABSTRACT TRUNCATED AT 400 WORDS)

Humans↗
PubMed1990

Mycosis fungoides bullosa.

A case of mycosis fungoides bullosa is presented. The results in our study confirmed that the predominant atypical lymphoid cells in the bullae, peripheral blood, and involved lymph nodes expressed the T-cell helper phenotype using immunophenotyping techniques. The literature is reviewed, confirming that our case demonstrated cells of the T-helper phenotype, not only in the skin but also in the blood and lymph node tissue. Bullous lesions in mycosis fungoides are rare.

Aged↗
PubMed1981

Mycosis fungoides associated with Mediterranean lymphoma.

Mycosis fungoides was observed in a 71-year-old male with Mediterranean lymphoma, a B-cell malignancy. It is proposed that this association is not incidental since hypergammaglobulinaemia and even monoclonal gammopathies have repeatedly been described in cutaneous T-cell lymphomas. Mediterranean lymphoma might have resulted from (a) helper cell activities of tumor T-lymphocytes, (b) common antigenic stimuli, or (c) deranged T-B cooperation due to concomitant mycosis fungoides.

Aged↗
PubMed1977

Photochemotherapy for mycosis fungoides.

Twelve cases of cutaneous mycosis fungoides (MF) with erythroderma, plaques, and tumors were treated with photochemotherapy. Methoxsalen followed two hours later by ultraviolet light (UVA) resulted in clinical clearing of skin lesions in seven patients with plaque stage MF and one patient with erythrodermic MF. Four patients with both plaque and tumor stage MF showed clearing of plaques but not the tumors. Phototherapy units consisted of boxes with 16 to 64 UVA lights. The mean numbers of treatments to clear was 17.4. The mean total dose to clear was 134.8 joules. The mean dose of maintenance UVA was 5.6 joules/sq cm. Histopathologic clearing of mycosis fungoides was demonstrated with the clinical clearing.

Adult↗
PubMed1976

Oral methoxsalen photochemotherapy of mycosis fungoides.

The cutaneous manifestations of mycosis fungoides have been successfully treated in nine patients for 16 to 28 months with oral methoxsalen and subsequent irradiation with longwave ultraviolet light. The efficacy of this therapy was confirmed in one patient, who showed complete clearing of generalized plaques after 1 month (12 treatments) except for a shielded control area which worsened during this period. Methoxsalen photochemotherapy may prove a valuable addition to therapies currently available for mycosis fungoides and may obviate some of the problems associated with conventional management of this disorder.

Administration, Oral↗
PubMed1995

Reassessment of histologic parameters in the diagnosis of mycosis fungoides.

The histologic diagnosis of mycosis fungoides (MF) can be difficult to establish and is based on interpretation of numerous subtle changes, most of which may be present to some degree in many inflammatory and neoplastic cutaneous conditions. To reassess the diagnostic criteria for making a histologic diagnosis of MF, we retrospectively reviewed histologic sections from 64 patients with mycosis fungoides (MF+) and compared the findings with sections from 47 patients who were biopsied to exclude MF and were shown not to have the disease (MF-). Patients were selected as MF+ or MF- independent of histologic findings based on the clinical course with at least 3 years of follow-up and immunophenotyping results. Following patient selection, at least two observers reviewed each slide without knowledge of final diagnosis and graded the intensity of approximately 25 histologic parameters. On univariate analysis, the following parameters were significant at beyond the p = 0.01 level: Pautrier's abscesses, haloed lymphocytes, exocytosis, disproportionate epidermotropism, epidermal lymphocytes larger than dermal lymphocytes, hyperconvoluted intraepidermal lymphocytes, and lymphocytes aligned within the basal layer. Haloed lymphocytes proved to be the most robust discriminator of MF from non-MF on multivariate analysis. These findings show that whereas many previously described features do discriminate between MF and inflammatory mimics, others are much less specific. Furthermore, few cases demonstrate all histologic features; for example, Pautrier's microabscesses were seen in only 37.5% of our cases. We conclude that a combination of specific histologic parameters can be used to establish a microscopic diagnosis of MF without the necessity of confirmatory immunophenotyping in the vast majority of cases.

Abscess↗
PubMed1980

Subpopulations of T lymphocytes in a patient with fulminant mycosis fungoides.

A patient with fulminant Mycosis fungoides was found to have a very high percentage of T lymphocytes with Fc receptors for IgG and a low percentage of T lymphocytes with Fc receptors for IgM. The total number of T lymphocytes was normal, but the in vitro function of the lymphocytes was depressed in short-term cultures, though not in cultures of 5 days' duration. T lymphocytes with Fc receptors for IgG are considered to have suppressor functions, and the immunological changes in this patient may be explained by an increased suppressor cell activity. Other investigators have proposed that the abnormal lymphocytes in Mycosis fungoides are T helper cells. After treatment with electron beam and transfer factor, our patient developed uraemia due to uric acid crystallization in the kidneys. This complication to the treatment given seems not to have been reported before.

Aged↗
PubMed1986

The nature of mycosis fungoides.

Thirty-four patients with a clinical diagnosis of plaque stage mycosis fungoides, poikiloderma atrophicans vasculare (poikiloderma) and parapsoriasis en plaques (parapsoriasis) were investigated for evidence of extracutaneous disease using a biochemical screen, blood films, bone marrow examination, chest radiograph, abdominal ultrasound, isotope liver scan, liver biopsy and lymphangiography. Skin biopsies were also taken from these patients and, in order to rule out non-specific histological change, from 29 controls with inflammatory skin disease; the slides were examined blind by two independent observers for evidence of mycosis fungoides or poikiloderma and graded accordingly. Both observers were able to differentiate significantly histological grades of mycosis fungoides corresponding to the clinical diagnosis although exact histological grades only corresponded on 35 per cent of slides. Features graded as classical or probable mycosis fungoides were found in between 22 and 67 per cent of patients with parapsoriasis and between 67 and 100 per cent of those with poikiloderma. We found no evidence of extracutaneous disease in any of our patients. Lymphangiograms were abnormal in 30 of 31 examinations but the changes were non-specific and did not correspond to disease type, duration or extent. Four patients had apparently unrelated co-existing disease including chronic lymphatic leukaemia in two, a monoclonal gammopathy and autoimmune haemolytic anaemia. This study shows that in its early stages mycosis fungoides is predominantly if not primarily a cutaneous disease. The findings also suggest that parapsoriasis and poikiloderma are part of the same disorder as mycosis fungoides or evolve into it. Aggressive treatment to prevent progression to mycosis fungoides plaque and tumour stage should therefore be tried. The findings support the idea that mycosis fungoides is a reactive rather than a neoplastic disorder.

Adolescent↗
PubMed1981

The prognostic value of membrane markers and morphometric characteristics of lymphoid cells in blood and lymph nodes from patients with mycosis fungoides.

For 26 patients with mycosis fungoides (MF), the type and extent of the skin lesions, the percentage of cerebriform mononuclear cells (CMC), and T and B lymphocytes in the peripheral blood and lymph nodes were correlated with MF involvement of regional lymph nodes, the clinical course, and response to therapy. Skin tumors and an involvement of more than 25% of the skin correlated well with lymph node involvement. Normal percentages (2--18%) of CMC in the peripheral blood were found for MF patients without lymph node involvement when compared with those found for patients with benign erythroderma and healthy donors. Elevated circulating CMC percentages (greater than 20%) were observed in 9 of 11 MF patients with lymph node involvement. In the lymph node cell suspensions from nine of ten MF patients with lymph node involvement, increased CMC values were found (greater than 15%), whereas two of three MF patients without lymph node involvement showed percentages comparable (4% and 7%, respectively) with those of the control lymph nodes. In the peripheral blood of patients with MF, decreased percentages of T cells (less than or equal to 55%) were found predominantly for patients with lymph node involvement, whereas normal percentages were noted for most of the patients without lymph node involvement. No consistent differences in the percentage of T and B cells in the lymph node cell suspensions were found between MF patients with and without lymph node involvement. Patients with lymph node involvement showed in general a partial response to therapy with an unfavorable clinical course in contrast to patients without lymph node involvement. Increased percentages of CMC (greater than 20%) and decreased percentages of T cells (less than or equal to 55%) in the peripheral blood, the presence of skin tumors, and involvement of more than 25% of the skin are prognostically unfavorable signs for patients with mycosis fungoides.

Aged↗
PubMed2006

Clinicopathologic features and T-cell receptor gene rearrangement findings of mycosis fungoides palmaris et plantaris.

BACKGROUND: Mycosis fungoides palmaris et plantaris (MFPP), characterized by hyperkeratotic patches or plaques confined to the palms and soles, is rare and easy to misdiagnose because of the clinical similarity to psoriasis, cutaneous inflammatory dermatoses, and dermatophytic infections. The literature about MFPP mostly consists of case reports with short-term follow-up. OBJECTIVE: Our purpose was to evaluate the clinicopathologic features, T-cell receptor (TCR) gene rearrangement findings, and prognosis of MFPP. PATIENTS AND METHODS: This retrospective study has been reviewed in the clinicopathologic, TCR gamma gene rearrangement findings and follow-up study of 12 patients with MFPP. RESULTS: The duration of diseases ranged from 9 months to 25 years with a mean duration of 5.3 years. Clinically, hyperkeratotic patches and plaques were observed in all cases, with 6 cases having developed on the palms and soles and 6 cases on the palms only. In TNM classifications, all cases were confined to T1N0M0 (stage IA) showing an early stage of mycosis fungoides (MF). Histopathologic findings revealed marked hyperkeratosis, parakeratosis with plasma, epidermotropism, convoluted lymphocytes, haloed lymphocytes, dense infiltrate of lymphocytes in all 12 cases (100%), Pautrier's microabscess in 9 cases (75%), a wiry bundle of collagen in 11 cases (91.7%) and basilar epidermotropism in 3 cases (25%). TCR gamma gene rearrangement was performed except for one case and monoclonality was detected in 10 of 11 cases. In the comparison group with cutaneous inflammatory dermatoses, all cases showed polyclonality. Treatment was done with Re-PUVA (acitretin and PUVA), ultraviolet A1, as well as systemic acitretin and methotrexate. Most patients showed a good response. In the follow-up study of 9 cases for a mean period of 47.6 months, only one patient's skin lesions were extended to the trunk and face, but the other patients had no sign of extracutaneous involvement. LIMITATIONS: These results were obtained from patients with MFPP in Korea. A cooperative study with other ethnic groups will be helpful. CONCLUSIONS: If a patient has recalcitrant palmoplantar dermatosis, MFPP should be suspected and histopathologic studies with TCR gene rearrangement should be done for early diagnosis of MFPP.

Adolescent↗
PubMed1994

Hypopigmented mycosis fungoides.

BACKGROUND: Only five cases of purely macular hypopigmented mycosis fungoides have been published in the literature. These patients all had clinical evidence of disease 7 months to 10 years prior to histologic diagnosis, suggesting that this clinical presentation of mycosis fungoides is easily misdiagnosed. OBSERVATIONS: Three African-American males, ages 9, 15, and 22 years, were found to have mycosis fungoides on evaluation of skin biopsy specimens after initially being clinically diagnosed with, and treated for, pityriasis alba. All three had typical histopathologic changes and cell marker studies showing a relative loss of the CD7 antigen, which is normally present on the majority of T cells. One patient who was treated with methoxsalen plus UV light (PUVA) therapy had clearance of his disease clinically and histologically. CONCLUSION: From the literature, it would appear that macular hypopigmented mycosis fungoides is exceptionally rare. This perceived rarity may be inaccurate due to either relative underreporting, incorrect diagnoses, or both. From the eight patients now reported to date, it may be said that hypopigmented mycosis fungoides may be seen in nonwhite individuals and more often than not, has onset before age 20 years.

Adolescent↗
PubMed2006

Mycosis fungoides presenting as reticular erythematous mucinosis.

BACKGROUND: Mycosis fungoides has numerous clinical presentations. METHODS: We present two cases in which mycosis fungoides clinically mimicked reticular erythematous mucinosis. RESULTS: Both patients had extracutaneous involvement. One had a fatal outcome. CONCLUSIONS: We suggest that full systemic evaluation be performed at the time of presentation of patients with this variant of mycosis fungoides.

Aged↗
PubMedDate not supplied

Follicular mycosis fungoides: variability of a rare entity.

OBJECTIVE: Three patients with follicular mycosis fungoides (FMF), a rare variant of folliculotropic cutaneous T-cell lymphoma, are discussed. Follicular involvement in cutaneous T-cell lymphoma commonly presents clinically with alopecia, follicular cysts, or comedo-like lesions usually associated with follicular mucinosis and strong epidermotropism. In contrast, FMF, in a strict sense, has been defined as a lymphocytic infiltration of hair follicles in the absence of both epidermal invasion and follicular mucin. DISCUSSION: Case 1 shows a clinically stable form of FMF characterized by circumscribed alopecia and its persistent resistance to topical therapeutic approaches. In contrast, Cases 2 and 3 demonstrate that classic mycosis fungoides lesions and FMF can occur simultaneously and that transitions occur from mycosis fungoides to FMF and vice versa. These observations strengthen the concept that FMF and mycosis fungoides represent just two variants of one entity of cutaneous T-cell lymphoma. In Case 3, rapid progression into tumor-stage mycosis fungoides and the follicular infiltration was controlled with bexarotene. CONCLUSIONS: Because it was previously suggested that FMF may have a worse prognosis compared with classic mycosis fungoides, this impressive response to bexarotene is of great clinical interest and may spark new studies employing this novel retinoid for FMF treatment.

Adult↗
PubMed1994

Epidemiology of mycosis fungoides.

Study of the epidemiology of mycosis fungoides has been hampered by methodologic issues. Mycosis fungoides does appear to be increasing in frequency more rapidly than lymphoma in general. There is substantial geographic variation, as well as variation by race and gender. Analytic studies have not demonstrated additional risk factors.

Adult↗
PubMed1993

Ocular involvement in mycosis fungoides.

A case of mycosis fungoides with bilateral intraocular involvement is reported. The diagnosis was established with the aid of a vitreous biopsy. Intraocular involvement, though rare, may be an early sign of extracutaneous disease.

Eye Neoplasms↗