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PubMed1991

A registry-based case-control study of mycosis fungoides.

The etiology of mycosis fungoides is unknown. Two possible causes (an unknown retrovirus with increased prevalence among never-married men, and prior malignancies) were investigated to determine whether they are associated with the incidence of mycosis fungoides. During 1973 to 1986, 953 case patients with mycosis fungoides or Sézary syndrome were registered by the Surveillance, Epidemiology, and End Results program. Each was matched by 5-year age group, sex, ethnicity, and geographic area to four control subjects, one each with cancer of the pancreas, brain, and stomach, and non-Hodgkin's lymphoma. For never-versus ever-married men, none of the relative risks differed significantly from those for women (odd ratios, .8-1.0). For any prior malignancy, the relative risks (and 95% confidence intervals) were 1.3 (.9-2.0), 1.2 (.8-1.8), 1.0 (.7-1.5), and 1.1 (.7-1.6). These data reject the previous relative risk estimate of 3.3 with greater than 99% power, and are consistent with only a small risk, if any, attributable to prior malignancy.

Aged↗
PubMed1994

Emergence of leprosy in a patient with mycosis fungoides.

A patient with mycosis fungoides that had progressed to tumor stage responded to chemotherapy and electron beam treatment, but 6 years later a peripheral neuropathy, extensive plaques, erythroderma, and enlarged pinnae containing acid-fast organisms developed while he was being treated with photopheresis. The skin lesions cleared with administration of rifampin and dapsone, but a reversal reaction biopsy specimen showed features of both mycosis fungoides and leprosy. This case raises the question of whether there may be an association between mycosis fungoides and leprosy.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1975

Bleomycin therapy in mycosis fungoides.

Nine patients with mycosis fungoides in different stages were treated with Bleomycin. Much better results were obtained with this new drug in the six patients with the infiltrative or beginning tumour stage than in the patients in the advanced tumour stage. Complete remission was not seen. In one case the results were objectivized by DNA cytophotometry. The role of Bleomycin in the treatment of mycosis fungoides is discussed. It is concluded that Bleomycin is not the medicament of choice for the treatment of mycosis fungoides.

Aged↗
PubMed1995

Mycosis fungoides palmaris et plantaris.

BACKGROUND: Mycosis fungoides primarily localized to the palms and soles is rare and has been previously reported as cutaneous lymphoma in four patients or as Woringer-Kolopp disease in eight patients. OBSERVATIONS: Four patients were initially diagnosed and treated unsuccessfully for various palmoplantar dermatitides until histopathologic findings revealed mycosis fungoides. Each case exhibited a clonal rearrangement of T-cell receptor gamma genes and immunohistochemical studies consonant with mycosis fungoides. All patients had limited skin involvement without evidence of extracutaneous involvement. CONCLUSIONS: Mycosis fungoides palmaris et plantaris is an uncommon expression of mycosis fungoides that manifests primarily on the palms and soles and clinically may mimic various inflammatory palmoplantar dermatoses. A biopsy is recommended in the evaluation of recalcitrant palmoplantar dermatoses.

Diagnosis, Differential↗
PubMedDate not supplied

Meningeal mycosis fungoides: cytologic and ultrastructural aspects.

Mycosis cells were identified in the pre-morbid cerebrospinal fluid of a patient with neurological symptoms and mycosis fungoides (MF). Light and electron microscopic examination at autopsy confirmed leptomeningeal involvement by mycosis fungoides. The cellular morphology of the non-cutaneous infiltrates supports the concept that mycosis fungoides retains a unique histopathology in its dissemination to the viscera. The importance of cerebrospinal fluid cytology in patients with mycosis fungoides is emphasized.

Brain Neoplasms↗
PubMedDate not supplied

Profile and outcome of childhood mycosis fungoides in Singapore.

Mycosis fungoides (MF) is the most common form of cutaneous T-cell lymphoma. It usually occurs in middle-aged and elderly persons, although several reports have described its occurrence in young children. The aim of this study was to review the profile and outcome of childhood MF in Singapore from 1989 to 1998. A total of nine patients (six males and three females) were diagnosed with MF before the age of 21 years. There were four Chinese, four Malay, and one Indian. The age at the time of histologic diagnosis ranged from 6 to 20 years (mean 14.3 years). Eight of the nine patients presented with hypopigmented patches and plaques. According to TNM staging, three were in stage 1A and six in stage 1B. The treatment modalities included psoralen plus ultraviolet A (PUVA) (n = 5), UVB (n = 2), and potent topical steroids (n = 2). We found that PUVA induced a faster clinical remission, but maintenance PUVA was required to prolong the relapse-free interval. This study also highlighted the need to consider MF in the differential diagnosis of hypopigmented dermatoses in dark-skinned individuals, especially if they occur on the buttocks.

Administration, Topical↗
PubMed2004

Therapy for mycosis fungoides.

Treatment of mycosis fungoides (MF) is indicated to reduce symptoms, improve clinical appearance, prevent secondary complications, and prevent progression of disease, all of which may have an impact on survival. Treatment of MF includes topical and systemic therapies, which can be administered alone or in combination. Psoralen and ultraviolet A radiation is effective in early-stage MF, inducing complete remissions in most patients. Psoralen and ultraviolet A radiation may also be combined with low doses of interferon (IFN)-alpha to treat stage I/II disease. However, early aggressive therapy with radiation and chemotherapy does not improve the prognosis. Local radiotherapy or total skin electron beam irradiation has been used with success to control advanced skin disease. Extracorporeal photopheresis may also be used successfully, but it is not generally available. Once the disease becomes refractory to topical therapy, IFN-alpha single-agent or combination chemotherapy may be administered, but the duration of response is often less than 1 year and ultimately all patients will relapse and become refractory. Among chemotherapeutic agents, pentostatin, gemcitabine, and liposomal doxorubicin seem to be particularly effective. Response rates after combined modality therapy with total skin electron beam irradiation and chemotherapy/IFN-alpha appear similar to response rates of chemotherapy alone. Therefore, there is a great need for the further development of novel emerging treatment modalities, such as retinoids (ie, bexarotene) and immunotherapeutic agents (ie, cytokines, tumor vaccines, and monoclonal antibodies), all of which appear to have significant therapeutic potential in patients with MF. Biologically based therapies may reduce the need for genotoxic therapies, such as cytostatics and radiotherapy.

Combined Modality Therapy↗
PubMed1999

Quantitation of intraepidermal T-cell subsets in formalin-fixed, paraffin-embedded tissue helps in the diagnosis of mycosis fungoides.

Differentiation between mycosis fungoides (MF) and cutaneous inflammatory processes can usually be made on clinical and histologic grounds. In difficult cases, immunohistochemical studies can be helpful since MF infiltrates usually contain a predominance of CD4+ lymphocytes, while most inflammatory lesions usually have a mixture of CD4+ and CD8+ lymphocytes. However, this determination has traditionally required the use of frozen tissue, thus severely limiting its usefulness. Recently, antibodies that differentially label CD4+ and CD8+ lymphocytes in formalin-fixed, paraffin-embedded tissue have become available (OPD4 and C8/144B respectively, DAKO (Carpinteria, CA, USA). This study tests the utility of these antibodies in the pathologic diagnosis of MF and inflammatory lesions with significant exocytosis. In 9 cases of MF for which both frozen and fixed tissues were available for comparison, the OPD4+ cell count in fixed tissue was significantly lower than the Leu-3a+ cell count in frozen tissue. Also, the C8/144B+ cell count in fixed tissue was higher than the Leu-2a+ cell count in frozen tissue, although this difference was not significant statistically. In a larger series for which only fixed tissue was available, epidermal CD4:CD8 ratios were significantly greater in 23 MF cases (mean 4.0+/-4.76) than in 35 inflammatory cases (mean 0.6+/-0.42; p = 0.001). Thus, although the studied antibodies appear to detect different epitopes in frozen versus paraffin-embedded tissue, demonstration of an elevated CD4:CD8 ratio in fixed tissue supports the diagnosis of MF, and is a helpful adjunct to routine histopathology.

Aged↗
PubMed1986

Epidermal mucinosis in mycosis fungoides.

In many cases of mycosis fungoides there is widening of the epidermal intercellular spaces (i.e., spongiosis) and papillary dermal fibrosis with minimal papillary dermal edema. Twenty biopsies of mycosis fungoides, stained with a modified colloidal iron procedure, were analyzed to substantiate the notion that the spongiosis resulted from the formation of an osmotic gradient because of intercellular acidic mucopolysaccharide deposition. In nineteen of twenty cases of patch-plaque mycosis fungoides, there was positive intercellular deposition of acidic mucopolysaccharides. In addition to the contribution of acidic mucopolysaccharides to the process of spongiosis, the biologic significance of epidermal mucin deposition is discussed. Mycosis fungoides should be added to the growing list of diseases in which there is epidermal mucinosis.

Adult↗
PubMed2005

[Mycosis fungoides presenting as annular erythema].

INTRODUCTION: Mycosis fungoides is a lymphoma, the classical clinical form of which involves erythematosquamous lesions. However, it can present various atypical aspects: hyper pigmentation or hypo pigmentation, suggestive of pyoderma gangrenosum or ichtyosis. We report a case of mycosis fungoides, unusual in its presentation in the form of centrifugal annular erythema. OBSERVATION: A 78 year-old man had developed a parapsoriasis in plaques for more than 20 years. In May 2002 he consulted because of the recent infiltration of one of the plaques, without concomitant pruritus. The clinical examination revealed 3 lesions of the popliteal groove of the right groin and the left cheek suggestive of centrifugal annular erythema. Histology, revealing Pautrier microabscesses, was compatible with the diagnosis of mycosis fungoides. Evolution was marked by the spontaneous regression of the plaque on the face and remission of the other two plaques after local treatment with chloromethin and topical corticosteroids. Nevertheless, new plaques appeared despite continued treatment, combined with PUVA therapy sessions. DISCUSSION: When searching the literature, we only found one other case of mycosis fungoides, the clinical aspect of which was a centrifugal annular erythema, but in which the histological examination confirmed the diagnosis of mycosis fungoides. Our case report is also unusual in the clinical regression of the lesion on the face, without treatment; this has only been reported in two cases. Mycosis fungoides can appear in various clinical forms. The centrifugal annular erythema form is rare, but this diagnosis should be evoked.

Adrenal Cortex Hormones↗
PubMed1986

Leu-8 and Leu-9 antigen phenotypes: immunologic criteria for the distinction of mycosis fungoides from cutaneous inflammation.

The distinction of mycosis fungoides from reactive cutaneous inflammation can be difficult. Unfortunately, since many reactive processes exhibit predominantly a mature helper T cell phenotype similar to that expressed by most cases of mycosis fungoides, standard immunologic marker studies have not been very helpful in differential diagnosis. To determine whether novel immunophenotypic criteria could be developed that correlate with the diagnosis of cutaneous involvement by mycosis fungoides, we studied the expression of Leu-8 and Leu-9 antigens by T cells in forty-one skin biopsy specimens from twenty-seven patients with mycosis fungoides and thirty-four skin biopsy specimens from thirty-three controls with a variety of benign cutaneous diseases. These antigens are expressed by the majority of normal T cells in the blood and lymphoid tissues but are often absent in T cell lymphomas or expressed by only a minority of tumor cells. Semiquantitative grading of the percentage of Leu-8+ and Leu-9+ T cells in our patients revealed that deficiency of these antigens (i.e., expression by less than or equal to 33% of T cells) was more prevalent among mycosis fungoides patients than among controls and became more specific for mycosis fungoides as the percentage of Leu-8+ and Leu-9+ T cells decreased. In initial biopsies, less than or equal to 33% of T cells were Leu-8+ in 82% of mycosis fungoides patients versus 15% of controls, while less than or equal to 10% of T cells were Leu-8+ in 52% of mycosis fungoides patients versus only 3% of controls.(ABSTRACT TRUNCATED AT 250 WORDS)

Antigens, Differentiation, T-Lymphocyte↗
PubMed1977

Cerebriform (Sézary like) mononuclear cells in healthy individuals: a morphologically distinct population of T cells. Relationship with mycosis fungoides and Sézary's syndrome.

The ultrastructural and surface marker characteristics of lymphocytes in human cord blood and peripheral blood of healthy donors were studied with respect to the presence of cerebriform mononuclear cells similar to those occurring in the dermal infiltrate of patients with mycosis fungoides (mycosis cells), and the skin infiltrate and peripheral blood of patients with Sézary's syndrome (Sézary cells). Cerebriform monuclear (Sézary-like) cells are characterized by a high nucleus-cytoplasm ratio, deep and narrow nuclear identations, condensed chromatin at the nuclear membrane and cytoplasm poor in organelles. Of the lymphoid cells in human cord blood and peripheral blood of healthy donors 6.7 and 8.7% respectively proved to be cerebriform mononuclear cells. Since these cells invariably form E-rosettes they are part of the T-cell population in healthy individuals. The finding of similar cells in the skin infiltrate of patch test areas of patients allergic to rubber, formalin and peruvian balsam--an expression of cellular immunity mediated by T-cells--suggests that these cells are reactive T cells. Not all (up to 85%) of the cerebriform mononuclear cells in patients with mycosis fungoides and Sézary's syndrome have T-cell membrane characteristics as shown by E-rosette formation. This suggests the presence of two populations of cerebriform mononuclear cells in mycosis fungoides and Sézary's syndrome. The relationship of cerebriform T cells as seen in healthy individuals with cerebriform or atypical mononuclear cells occurring in the Sézary syndrome and mycosis fungoides is discussed.

Adolescent↗
PubMed1982

Hyperkeratosis in mycosis fungoides.

A case of mycosis fungoides with hyperkeratotic changes of the nails, palms, and soles is presented. Although these features have been reported in the older literature, most contemporary authors neglect to mention this interesting cutaneous change in mycosis fungoides.

Antineoplastic Agents↗
PubMedDate not supplied

Unilesional (segmental) mycosis fungoides presenting in childhood.

Mycosis fungoides is rare in children, and a unilesional presentation is also rare. A 13-year-old Kuwaiti boy with unilesional mycosis fungoides is described. Clinically he had a single indurated large plaque on the left shoulder with histopathologic features typical of cutaneous T-cell lymphoma. The diagnosis was further supported by the presence of a T-cell clone discovered through molecular biology studies of paraffin-embedded material. No other lesions were detected. The lesion showed a favorable response to local radiotherapy.

Adolescent↗
PubMed2006

Decreased CD117 expression in hypopigmented mycosis fungoides correlates with hypomelanosis: lessons learned from vitiligo.

Hypopigmented mycosis fungoides is an uncommon clinical variant of cutaneous T-cell lymphoma. We hypothesized that hypomelanosis in hypopigmented mycosis fungoides may have a similar mechanism as in vitiligo, a condition in which it is believed that alterations in expression of CD117 (stem cell factor receptor/KIT protein) on epidermal melanocytes and abnormal interactions between melanocytes and surrounding keratinocytes may play a pathogenic role. To test the hypothesis that similar mechanisms might also explain hypopigmentation in hypopigmented mycosis fungoides, skin specimens from five cases each of hypopigmented mycosis fungoides and vitiligo were studied immunohistochemically for immunophenotype of the infiltrating cells, CD117 (expressed by epidermal melanocytes), and pan melanoma cocktail of antigens (gp100, tyrosinase, and MART-1) expression; cases of conventional mycosis fungoides and normal skin were studied in parallel as controls. Our findings confirm a predominance of CD8+ neoplastic T cells in hypopigmented mycosis fungoides. Similarly, the epidermal lymphocytic infiltrate in vitiligo was also composed of CD8+ cytotoxic T cells, in contrast to an epidermal infiltrate composed of CD4+ T cells in conventional mycosis fungoides. The average number of epidermal CD117 expressing cells followed the same pattern of decreased expression in hypopigmented mycosis fungoides as in vitiligo, whereas the levels in conventional mycosis fungoides were higher, and similar to that observed in normal skin. Furthermore, a decreased number of melanocytes per high-power field of the length of the biopsy was present in hypopigmented mycosis fungoides and vitiligo, as compared with either conventional mycosis fungoides or normal skin, suggesting a correlation between decreased expression of CD117 and decreased number of melanocytes. We propose that decreased expression of CD117 and its downstream events in melanocytes may be initiated by cytotoxic effects of melanosomal-antigen-specific CD8+ neoplastic T lymphocytes, resulting in destabilization of CD117 and leading to dysfunction and/or loss of melanocytes in the epidermis of hypopigmented mycosis fungoides.

Antigens, Neoplasm↗
PubMed1990

Immunotactoid glomerulopathy associated with mycosis fungoides.

A patient with mycosis fungoides developed a nephrotic syndrome. Renal biopsy revealed deposits of a highly organized fibrillar material which did not stain with the typical amyloid stains; this picture was consistent with the diagnosis of non-amyloidotic fibrillary glomerulopathy or immunotactoid glomerulopathy. We believe this is the first case reported of immunotactoid glomerulopathy associated with mycosis fungoides. Possible pathogenetic implications are discussed with reference to previous publications.

Female↗
PubMed1992

State of the art therapy of mycosis fungoides and Sézary syndrome.

Mycosis fungoides and Sézary syndrome are low-grade, non-Hodgkin's lymphomas with initial cutaneous involvement. Early stage patients have a good prognosis and are commonly treated with topical agents. However, most patients eventually develop resistant skin disease or visceral disease, necessitating systemic treatment. Single agent and combination chemotherapy have resulted in high response rates, although these are short lived and not without treatment toxicities. Other modalities that have been tried with promising early results are: interferon, fludarabine, retinoids, cyclosporin, 2'-deoxycoformycin, leukapheresis, photopheresis and monoclonal antibodies (unlabeled, radiolabeled, or toxin labeled). We will discuss the available treatment options, as well as our approach to the patient with mycosis fungoides or the Sézary syndrome.

Antineoplastic Agents↗