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The condition of a patient with advanced mycosis fungoides was treated with cyclic combination chemotherapy. At the sites of regressing tumors, yellow nodules appeared that were clinically and histologically benign xanthomata. The probable multifactorial pathogenesis of dystrophic xanthomata is discussed and previous case reports are reviewed.
We report what we believe to be the first documentation of a patient with both cold urticaria and mycosis fungoides. The patient described a marked worsening of his long-standing lesions of mycosis fungoides at the same time as the onset of cold sensitivity. We believe this suggests a possible association between these 2 rare diseases.
BACKGROUND: Extracorporeal photopheresis (ECP) is an effective treatment for cutaneous T-cell lymphoma. Controversy has arisen regarding its ability to improve survival rates in Sézary syndrome (SS). We describe our experience with ECP in the treatment of SS and erythrodermic mycosis fungoides, with particular emphasis on early predictors of long-term outcome. OBSERVATIONS: We included 17 patients (15 with SS and 2 with erythrodermic mycosis fungoides) who received ECP as initial treatment. Four of these patients were moribund on presentation (Eastern Cooperative Oncology Group Performance Status score, 4) and underwent only 1 to 2 cycles of ECP. The median survival was 56 months for the 11 patients with SS and an Eastern Cooperative Oncology Group Performance Status score of less than 4. If all 15 patients with SS are considered, median survival was 34 months. Response after 5 months of ECP correlated with long-term survival. A low number (<6.0 x10(3)/ micro L) of circulating CD4(+)CD7(-) lymphocytes correlated with response after 5 months of ECP. CONCLUSIONS: Extracorporeal photopheresis is a safe, effective, and well-tolerated treatment for erythrodermic mycosis fungoides and SS. Low numbers of CD4(+)CD7(-) cells in the circulation and a positive response after 5 months of therapy predicted long-term survival. Moribund patients are much less likely to benefit from ECP.
The possibility of the transformation of mycosis fungoides into a lymphoma of another histologic type is problematic. Convincingly illustrated examples are not to be found in the literature. We report on a well-documented case of a man who developed nodular sclerosing Hodgkin's disease 15 years after first presenting with mycosis fungoides.
We describe the case of a 13-year-old boy with stage I hypopigmented mycosis fungoides in whom minimal residual disease was detected with T-cell receptor gamma-polymerase chain reaction after the disease was in complete clinical remission. We further cloned and sequenced the T-cell receptor gamma-polymerase chain reaction product of the lesion in remission and found that the original T-cell clone still existed in decreased amounts. The patient was followed up for 3 1/2 years without any new lesions developing. The clinical significance of this residual malignant T-cell clone in mycosis fungoides remains to be elucidated.
This is a 14-year follow-up of a patient with granulomatous mycosis fungoides. The patient has had nodular lesions of mycosis fungoides for more than 30 years, an unusually long survival for the nodular form of the disease that is often quickly fatal. The role of the granulomatous inflammatory response in his survival is speculated upon.
Ten patients, who had been treated for mycosis fungoides with electron beam radiation ten or more years previously, were examined for signs of radiation dermatitis. Although most patients had had acute radiation dermatitis, only a few manifested signs of mild chronic changes after having received between 1,000 and 2,800 rads.
Three patients with mycosis fungoides, who were long-term employees of a manufacturer of solid fuel propellants, were seen. Two of these patients had tumorous involvement of the central nervous system, which was successfully treated with radiation therapy. The potential relationship of carcinogens is discussed.
A patient with mycosis fungoides (MF) bullosa had a rapidly growing, painful necrotic mass on the left ankle which extended by peripheral bulla formation, clinically resembling pyoderma gangrenosum. Histopathology confirmed MF bullosa with both intraepidermal and subepidermal bulla formation.
BACKGROUND: The lichenoid tissue reaction (LTR) is present in a variety of dermatoses. Numerous histologic variants of mycosis fungoides (MF) have also been described. OBJECTIVE: Our purpose was to analyze a subset of patients with mycosis fungoides (MF) who had a prominent LTR at the time of presentation. METHODS: Clinical and pathologic features were evaluated in 12 patients with MF and an LTR. RESULTS: The primary complaint was intense pruritus (in 10 of 12 patients). An accelerated course was noted in one half of the patients including four with Sézary syndrome. Four patients died: two with Sézary syndrome and two with extensive patch/plaque MF. Lymphocyte atypia, prominent basal cell layer epidermotropism, and the presence of plasma cells and eosinophils may help to distinguish lichenoid MF from lichen planus. CONCLUSION: A subset of patients with MF present with lichenoid changes. The pathologic features may have a striking resemblance to lichen planus. Our findings suggest that lichenoid changes in MF tend to be associated with intense pruritus and may connote a poor prognosis.
A specific molecular probe for the malignant lymphoid clone of lesions of a patient with mycosis fungoides was developed. As a clone-specific marker the junctional region of rearranged T-cell-receptor-gamma (TCR-gamma) genes was used. An oligonucleotide primer complementary to these sequences was designed. Using this primer and polymerase chain-reaction technology, early hypopigmented lesions of the patient, which were previously unclassifiable by conventional microscopy, were analysed. The study demonstrates, on the molecular level, that these hypopigmented lesions contain tumour clone-specific DNA and may represent early manifestations of mycosis fungoides.
There are many types of skin disease that fit into the classification of cutaneous lymphoma, but mycosis fungoides is by far the most common of this group. It is a non-Hodgkin's lymphoma of T-cell origin that presents in the skin. Mycosis fungoides often evolves for years without a specific diagnosis because it can present as an eczematous or psoriasiform eruption. Patients identified in the early stages and treated appropriately have a normal life expectancy.
A 32-year-old white male was diagnosed as having mycosis fungoides in 1976; bone marrow biopsy and aspiration in August 1984 revealed infiltration with neoplastic cells. Cytogenetic analysis of the cells from the bone marrow specimen showed that 48 of 50 metaphases contained an extra Y chromosome (i.e., 47,XYY). The remaining two cells were hypotetraploid and hyperpentaploid, respectively, with a common marker derived from chromosome #2. The metaphases obtained by PHA stimulation of peripheral blood cells showed a 47,XYY pattern. An interleukin 2 (IL-2) dependent T-cell line was established from the patient's blood mononuclear cells; all metaphases of this line had an extra Y chromosome. Thus, this case is one of a mycosis fungoides developing in an XYY male.
Mycosis fungoides (MF) is a cutaneous T-cell lymphoma that may mimic, clinically and histologically, other more common entities before definitive diagnosis. We report a case of plaque-stage MF in a young woman with hepatic failure and present an overview of some of the diagnostic features and therapeutic options available for this disease.
The d'embleé 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'embleé and correlate the histologic and immunophenotypic data with those of a more updated classification scheme.
We describe an unusual case of pulmonary involvement by mycosis fungoides in a 31-year-old woman. Disseminated disease had developed despite cutaneous irradiation and systemic combination chemotherapy. Microscopy of biopsied tumour deposits in the lung showed a polymorphous, angiocentric lymphoid infiltrate, including atypical cells with convoluted nuclei which displayed a T-cell phenotype. Numerous epithelioid granulomas were also present. These histological appearances represent an unusual pattern of pulmonary involvement by disseminated mycosis fungoides.
We describe a patient who developed mycosis fungoides, a T-cell neoplasm, two years following the chemotherapy and radiotherapy of a diffuse, large-cell ("histiocytic") lymphoma. Immunologic studies documented a "null-cell" phenotype for the large-cell lymphoma. Therefore, the proliferation of two distinct malignant lymphoid clones is believed to have developed in the patient. The value of immunologic marker studies in this case was to aid in the diagnosis of the two separate neoplasms, as well as to aid in the selection of appropriate specific therapy directed against each neoplasm. As the association of large-cell lymphoma and mycosis fungoides in one patient represents a very rare event, we discuss several hypotheses about the possible interrelation of the two neoplasms.
Follicular mycosis fungoides (FMF) is a rare cutaneous T cell lymphoma characterized by an atypical lymphoid infiltrate spreading within and around hair follicles without epidermotropism or follicular mucin deposits. Its occasional presentation with minimal epidermal involvement and/or follicular mucinosis suggests the need for uniform histologic criteria. We describe a new case of FMF associated with follicular mucinosis and discuss its morphologic spectrum of presentation.