Association of mycosis fungoides and Hodgkin's disease

A 69-year-old man developed a Hodgkin's disease 2 years after he started a mycosis fungoides. He presented cutaneous plaques of mycosis fungoides. The first signs of Hodgkin's disease was acquired ichthyosis and loss of weight. Echotomography of the abdomen showed retroperitoneal nodes. A laparotomy was performed and the histopathologic examination of the lymph nodes revealed a Hodgkin's disease type 2 (sclero-nodular). The liver and the bone marrow were involved. A chemotherapy was completed but the patient died 10 months later. The review of the literature showed 24 patients with Hodgkin's disease and mycosis fungoides or Sézary syndrome. Relation between mycosis fungoides, Hodgkin's disease and lymphomatoid papulosis are discussed.     

Mycosis fungoides and cutaneous Hodgkin''s disease in the same patient: a case report

We describe a case of a 34-year-old man who presented with a 10-year history of mycosis fungoides and recent onset of inguinal lymphadenopathy, fever, and cutaneous nodules on the right hip. Biopsy of the inguinal lymph node showed mixed cellularity Hodgkin's lymphoma. Biopsy of the pre-existing skin lesions showed mycosis fungoides, while that of the nodules on the hip revealed cutaneous Hodgkin's lymphoma. Immunohistochemical and molecular analysis demonstrated a null phenotype in the Reed-Sternberg cells of the Hodgkin's disease lesions, and a T-cell phenotype in the lymphoid cells of the mycosis fungoides lesions. He was treated with chemotherapy for Hodgkin's disease, which is in remission 11 years later. Topical nitrogen mustard and maintenance PUVA therapy have been used for the mycosis fungoides, which is also in remission.     

Fine-needle aspiration biopsy of mycosis fungoides presenting as an ulcerating breast mass

A patient with previously diagnosed Hodgkin's disease presented with ulcerating lesions on her scalp and breast. Fine-needle aspiration of the breast lesion revealed convoluted cells consistent with mycosis fungoides. Review of the patient's previously obtained lymph node biopsy specimens revealed a diagnosis of mycosis fungoides rather than Hodgkin's disease. Better communication between hospitals, pathologists, and clinicians may have prevented the erroneous original diagnosis of Hodgkin's disease in this patient.     

Lymphomatoid papulosis and Hodgkin's disease: Are they related?

Two different characteristic types of lymphomatoid papulosis (type A and type B) can be histologically distinguished, that represent the ends of a spectrum. In the present report, two patients are described. One patient with both lymphomatoid papulosis type A and type B lesions for more than 25 years developed Hodgkin's disease (nodular sclerosing type) in the para-aortic and para-iliac lymph nodes. Histologic examination of the skin lesions in the second patient, who had Hodgkin's disease (nodular sclerosing type) in many supradiaphragmatic lymph nodes, showed the characteristic features of lymphomatoid papulosis type A. These findings, together with the results of recent immunohistochemical investigations showing many similarities between the large atypical cells in lymphomatoid papulosis type A lesions and Reed-Sternberg cells in Hodgkin's disease, support the view that lymphomatoid papulosis type A and Hodgkin's disease are closely related conditions. The results of recent studies indicate a close relationship between lymphomatoid papulosis type B and the early stages of mycosis fungoides. Accordingly, the possible relationship between lymphomatoid papulosis types A and B, mycosis fungoides, and Hodgkin's disease is discussed.     

Autoimmune anti-T cell antibodies in the sera of patients with mycosis fungoides

The serum of persons with mycosis fungoides contains IgG antibody believed to be specific for T cells, similar to the auto-antibodies reported in patients with systemic lupus erythematosus, Hodgkin's disease and sarcoidosis. The antibody is present during the active, and disappears during the inactive, phase of the disease.     

Hodgkin Lymphoma in a patient with mycosis fungoides: molecular evidence for separate cellular origins

We report the case of a man with mycosis fungoides (MF), who, 11 years after diagnosis, developed Hodgkin's disease. Although MF is associated with a higher than expected prevalence of other malignancies, including Hodgkin lymphoma, analysis of cells from the skin and lymph nodes showed findings that suggest a separate cellular origin for the two diseases.     

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.     

Lymphomas.

The diagnosis of lymphoma currently relies on a combination of clinical, routine histopathologic, and immunohistochemical studies, sometimes supplemented by other special techniques such as flow cytometry, DNA analysis, and gene rearrangement study. This review considers the practical diagnosis of cutaneous lymphoma, with particular emphasis on three areas: Hodgkin's disease, non-Hodgkin's lymphomas and their differentiation from lymphocytoma cutis, and mycosis fungoides and other T-cell lymphomas.     

Concurrent eosinophilic fasciitis and cutaneous T-cell lymphoma. Eosinophilic fasciitis as a paraneoplastic syndrome of T-cell malignant neoplasms?

Eosinophilic fasciitis has been reported to precede hematologic malignant neoplasms such as myelomonocytic leukemia, lymphocytic leukemia, and Hodgkin's lymphoma. In this case study, eosinophilic fasciitis occurred concurrently with cutaneous T-cell lymphoma (mycosis fungoides). The clinical diagnosis of eosinophilic fasciitis was based on painful sclerodermatous lesions on the extremities and trunk without acrosclerosis. There was histologic confirmation with edema and lymphocytic inflammation in the superficial muscular fascia and dermis. Deposition of immune reactants was found in the fascia and dermis. In addition, peripheral eosinophilia and circulating immune complexes were detected. The diagnosis of cutaneous T-cell lymphoma (mycosis fungoides) was based on extensive erythematous cutaneous plaques, dermal and epidermal lymphocytic atypia, loss of pan-T-cell immunologic markers, and a cutaneous lesional T-cell receptor beta-chain rearrangement by Southern blot analysis. Eosinophilic fasciitis may occur as a paraneoplastic syndrome associated with hematologic malignant neoplasms, including mycosis fungoides. Cytokines or lymphokines released by activated immunocytes, either malignant leukocytes or normal leukocytes reacting to malignant cells, may be responsible for the eosinophilia and sclerosis seen in these associated hematologic malignant neoplasms.     

Chemokine receptor expression on neoplastic and reactive T cells in the skin at different stages of mycosis fungoides

We analyzed the expression of 13 chemokine receptors in mycosis fungoides, in order to assess the contribution of chemotaxis to the pathogenesis of the disease. Material from skin biopsies of six patients with early disease and six patients at the tumor stage of mycosis fungoides was analyzed by immunohistochemistry and partly also by flow cytometry. The receptors CCR1, CCR2, CCR3, CCR5, CCR6, CXCR1, CXCR2, CXCR5, and CX3CR1 were rarely and inconsistently detected in lesional skin and thus their participation in mycosis fungoides could largely be ruled out. In contrast, CCR4, CXCR3, and CXCR4 were substantially expressed on both mycosis fungoides cells and the surrounding reactive T cells in the early patch and plaque stages of the disease, indicating an involvement of these chemokine receptors in the disease process. In the tumor stage of mycosis fungoides, we interestingly observed a loss of a relevant chemokine receptor in four out of six patients. In three patients CXCR3 and in one patient CCR4 was absent on tumor mycosis fungoides cells, whereas the reactive T cells showed normal levels of expression. Within these samples, tumor mycosis fungoides cells exhibited high levels of CCR7, a chemokine receptor central for the entry of T cells to lymphatic tissue. Taken together, our data suggest that the loss of one or more of the chemokine receptors involved in the homing of the mycosis fungoides cells to the skin may trigger the latent potential of these cells to metastasize into regional lymphatic tissue.     

Follicular mucinosis: clinical and histopathologic study

Fifty-nine patients with the histologic diagnosis of follicular mucinosis (alopecia mucinosa) were evaluated retrospectively. Thirty-seven were male and 22 were female; ages ranged from 10 to 76 years. Of 19 patients with mycosis fungoides, 16 had initial lesions of follicular mucinosis on the trunk or extremities. Two patients had Hodgkin's disease and follicular mucinosis; both were younger than 20 years of age. Seven other patients were 20 years of age or younger. Two of the nine adolescent patients had persistent plaques of follicular mucinosis up to 18 years after diagnosis. Evaluation of biopsy specimens for lymphocytes, eosinophils, nonlymphoid cell infiltration, epidermal lymphocytic exocytosis, mucin deposition, and epidermal hyperplasia revealed no predominant feature that differentiated the group with benign disease from the group with mycosis fungoides. We conclude that no single clinical or histopathologic observation predicts which patients with follicular mucinosis will have a benign course and that evaluation of multiple clinical and histologic variables is necessary.     

Mycosis fungoides: classic disease and variant presentations

Mycosis fungoides is a peripheral non-Hodgkin's T-cell neoplastic process, representing the most common type of primary cutaneous malignant lymphoma. Neoplastic lesions classically show skin predilection and characteristic clinical and histologic features in patch, plaque, and tumor stages. In addition, several clinicopathologic variants of mycosis fungoides have been delineated, including poikiloderma atrophicans vasculare (parapsoriasis variegata), Sézary syndrome, granulomatous mycosis fungoides, hypopigmented mycosis fungoides, folliculocentric mycosis fungoides, syringotropic mycosis fungoides, and Woringer Kolopp disease. We will review the salient features of patch, plaque, and tumor stage mycosis fungoides in this article and follow with a discussion of these variant clinicopathologic presentations and of therapeutic modalities.     

Lymphomatoid papulosis update. A historical perspective

Soon after lymphomatoid papulosis was identified and named, it became apparent that a whole constellation of clinically benign, histologically malignant, continuing, self-healing eruptions existed. To this group of assumed entities without names, the inclusive term rhythmic paradoxical eruption spectrum was proposed. Some bands of this spectrum have since been delineated, and their surmised overt malignant potential realized. Recent immunologic studies have identified a number of shared immunologic markers among the various bands of this spectrum, which helps to explain the interrelationships and occasional coexistences among lymphomatoid papulosis, mycosis fungoides, Hodgkin's disease, and other lymphoproliferative disease states.     

The prognosis of patients with lymphomatoid papulosis associated with malignant lymphomas

Lymphomatoid papulosis (LyP) is a disorder which generally runs a benign course, but can sometimes be associated with a malignant lymphoma. Information about the prognosis of these LyP-associated lymphomas is, however, fragmentary. In this study, the clinical data of 50 LyP-associated malignant lymphomas, including 11 patients of our own group and 39 reported in the literature, are evaluated. Three main groups of LyP-associated malignant lymphomas could be distinguished: cases associated with mycosis fungoides (19/50 cases). Hodgkin's disease (12/50 cases) and (CD30+) large-cell lymphomas (16/50). The results of this study demonstrate that patients with mycosis fungoides. Hodgkin's disease, and (CD30+) large-cell lymphomas limited to the skin have a favourable prognosis. However, the prognosis of patients developing a systemic (CD30+) large-cell lymphoma proved generally poor. The results of this study also indicate that the risk of an individual LyP patient developing systemic lymphoma is less than 5%.     

THE TREATMENT OF MYCOSIS FUNGOIDES WITH PUVA*

Twenty patients with mycosis fungoides were treated with photochemotherapy using oral psoralens and long wave ultraviolet light (PUVA) over a two-year period. PUVA was effective in producing a diminution of cutaneous deposits of mycosis fungoides with each clinical pattern of presentation. In most patients complete clearing could not be achieved, and in those considered free of disease, sustained total clearing off PUVA could not be maintained. Lack of response to the effect of PUVA if reinstituted for recurrence of disease did not occur. The palliative use of PUVA for the treatment of mycosis fungoides is recommended.     

Anetodermic mycosis fungoides: a new clinicopathological variant of mycosis fungoides

Mycosis fungoides is the most common type of primary cutaneous T-cell lymphoma. Several rare clinicopathological variants of mycosis fungoides have been described. Patients with these variants often also have classic mycosis fungoides at other sites of the body. Anetoderma is a cutaneous disorder in which multiple, oval lesions or atrophic plaques with wrinkled surface develop progressively due to loss of the dermal elastic tissue. Primary anetoderma occurs when there is no underlying associated disease and it arises on clinically normal skin, whereas secondary anetoderma appears in the same site as a previous specific skin lesion. There is a large list of heterogeneous dermatoses associated with secondary anetoderma. Two patients developed areas of secondary anetoderma on plaque stage lesions of mycosis fungoides. The lesions consisted of exophytic nodular lesions, with very soft consistency on palpation, scattered over the hyperpigmented plaques in one patient and violaceous indurated plaques with overlying epidermal atrophy and mild scale in the other. Histopathological study demonstrated that the cells involving the dermis were mainly T-helper lymphocytes, with few histiocytes and some multinucleate giant cells engulfing distorted elastic fibres. Elastic tissue stain demonstrated that elastic fibres were almost completely absent in the dermis of the anetodermic lesions. Anetodermic mycosis fungoides should be added to the list of clinicopathological variants of mycosis fungoides and mycosis fungoides should also be considered as a possible disease causing secondary anetoderma. Anetodermic mycosis fungoides shows clinical and histopathological features different from those of granulomatous slack skin.     

Immunohistochemical expression of IL-10 in mycosis fungoides

Abstract Immunoreactivity of the cytokine IL-10 has been investigated in situ in mycosis fungoides (MF). Expression of IL-10 was detected using immunohistochemistry in skin biopsies (n=8) and T-cell lines (n= 2) from mycosis fungoides patients. IL-10 positivily was seen in the dermal cell infiltrates and in T-cell lines in mycosis fungoides. The dermal IL-10 reaction in a skin biopsy from an active lesion in MF indicates the possibility for disease progression.     

Pseudotumorous folliculotropic mycosis fungoides

Fungating nodules and infiltrated plaques are usually equated with advanced tumor stage mycosis fungoides. We report an 85-year-old man who presented in this way but multiple skin biopsies revealed that the bulk of his nodules were due to marked follicular hyperplasia as a result of folliculotropic mycosis fungoides. This clinical presentation may be best described as a pseudotumorous form of mycosis fungoides dominated by follicular epithelial hyperplasia rather than lymphocytic proliferation characteristic of true tumor stage disease. Similar presentations have been described as a verrucous and hyperplastic variant of mycosis fungoides due to the presence of prominent epidermal hyperplasia.     

Adenosine deaminase activity in sera of patients with psoriasis, mycosis fungoides and adult T cell leukemia.

Adenosine deaminase activities in sera were measured in 18 psoriatic patients, 8 mycosis fungoides patients, and 9 patients with adult T cell leukemia. Adenosine deaminase activity in the sera of the psoriatic patients showed no significant increase. An elevated adenosine deaminase activity was observed in 7 of the 8 patients with mycosis fungoides and 8 of the 9 patients with adult T cell leukemia. After chemotherapy, adenosine deaminase activity in serum of acute adult T cell leukemia was reduced. Adenosine deaminase activity in the sera of 2 patients with smoldering adult T cell leukemia was more elevated, with exacerbation of the disease. It is difficult to grade the extension of the tumors in plaque stage mycosis fungoides and smoldering adult T cell leukemia. To know the progression of the disease is critical in determining its management. These results indicate that adenosine deaminase activity in serum is one of the reliable indicators for the grading of mycosis fungoides and adult T cell leukemia.