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PubMed2001

Reassessment of lymphocytic atypia in the diagnosis of mycosis fungoides.

The manifestations of mycosis fungoides in its early stage may mimic clinically and histologically those of many benign inflammatory dermatoses. Therefore, the diagnosis of mycosis fungoides remains a major challenge for dermatologists and dermatopathologists. For many years, it has been proposed that atypical lymphocytes within the epidermis constitute one of the diagnostic features in mycosis fungoides. Presence of dermal atypical lymphocytes remains controversial as a diagnostic criterion. We reassessed the feasibility of applying lymphocytic atypia within epidermis and dermis as diagnostic criteria discriminating between mycosis fungoides and spongiotic dermatitis. Thirty cases of mycosis fungoides and 30 cases of spongiotic dermatitis were retrieved from archival hematoxylin and eosin-stained histologic sections. Punch biopsy sections were examined by light microscopy; epidermal and dermal lymphocytes were photographed at 1000x (oil immersion). A total of 92 ektachrome slides (35 mM) were developed, coded, and ordered randomly. For each slide, cells were interpreted as typical or atypical lymphocytes by seven pathologists. Atypical epidermal lymphocytes were judged to be present in 9 +/- 2 out of 16 (56%) cases of mycosis fungoides photographed as compared with 8 +/- 3 out of 16 (50%) in spongiotic dermatitis. Dermal lymphocytic atypia was thought to be present in 14 +/- 6 out of 30 (47%) patients with mycosis fungoides. Thirteen +/- 6 out of 30 (43%) patients with non-mycosis fungoides also displayed dermal lymphocytic atypia. No statistical significance was observed in these comparisons (t test, P >.05). Furthermore, atypia of lymphocytes was deemed to be present in 41, 38, 59, 70, 23, 47, and 40 out of 92 slides examined by the investigators, suggesting that observer variation is a very significant factor in our present study. We conclude that it is not possible to distinguish mycosis fungoides from spongiotic dermatitis merely based on lymphocytic atypia within epidermis or dermis.

Dermatitis↗
PubMed2003

Value of the CD8-CD3 ratio for the diagnosis of mycosis fungoides.

Histopathological diagnosis of mycosis fungoides is difficult, especially in early lesions that may be indistinguishable from inflammatory dermatoses. Mycosis fungoides is a clonal proliferation of mature epidermotropic CD4+ lymphocytes. The aim of this study was to determine the contribution of the CD8-CD3 ratio to the diagnosis of mycosis fungoides. We retrospectively compared the immunophenotypic characteristics of 30 mycosis fungoides with 28 inflammatory dermatoses. The diagnosis of mycosis fungoides was reinforced in all cases by the presence of a cutaneous dominant T-cell clonal population. To analyze exclusively the lymphocytic infiltrates, CD4, which is also expressed by histiocytes, was not considered. The CD8-CD3 ratio was determined separately in the epidermis and the dermis using two methods, one quantitative and the other semiquantitative. Concordance rates between the two methods were higher in epidermal than dermal infiltrates. The mean CD8-CD3 ratio was significantly lower for mycosis fungoides than control cases, with the difference being greater in the epidermal than the dermal component. Although not absolutely specific, a low CD8-CD3 ratio in the epidermal component of a lymphocytic infiltrate supports the diagnosis of mycosis fungoides. It can be evaluated in routine practice using a semiquantitative approach.

Adolescent↗
PubMed2006

Mycosis fungoides with coccidioidomycosis.

Mycosis fungoides is an indolent, epidermotropic, cutaneous T-cell lymphoma of helper/memory T cells that presents as heterogeneous, papulosquamous patches, plaques, and tumors. We present a patient with mycosis fungoides and infection with Coccidioides immitis of the skin, which has not been previously reported.

Adenocarcinoma↗
PubMed1980

A study of the photosensitive factor in relation to skin lesions of mycosis fungoides patients.

Some mycosis fungoides patients show abnormal light reactions in apparently normal skin. Clinically it is observed that lesional skin most easily burned during photochemotherapy. For this reason phototests to broad wavelength regions were performed in 9 mycosis fungoides patients, close to skin lesions and at 3 and 10 cm from cutaneous lesions. The light doses giving the minimal erythemal dose, were of about the same magnitude at a distance of 3 and 10 cm from the lesions. One third of the patients showed abnormal reactions following UVA and UVB. More abnormal reactions were seen close to lesional skin, and a displacement towards increased light sensitivity was demonstrated.

Aged↗
PubMed2003

Microsatellite instability is associated with hypermethylation of the hMLH1 gene and reduced gene expression in mycosis fungoides.

Fifty-one mycosis fungoides samples were analyzed for microsatellite instability (MSI) using the panel of markers recommended for hereditary nonpolyposis colorectal cancer kindred and a panel we designed for cutaneous T cell lymphoma in order to compare detection rates and determine if MSI is a genome-wide phenomenon. Samples demonstrating MSI were analyzed for abnormalities of the hMLH1 gene including loss of heterozygosity, mutations, and promoter hypermethylation. MSI was detected in 16% using the hereditary nonpolyposis colorectal cancer panel and 22% with the cutaneous T cell lymphoma panel. Overall, 27% demonstrated MSI and 73% had a stable phenotype. hMLH1 gene studies did not detect loss of heterozygosity or reveal any mutations. Promoter hypermethylation was detected in nine of 14 patients with MSI, however (64%). In addition hMLH1 and hMSH2 protein expression was studied using immunohistochemical techniques. Five of nine patients with MSI and hMLH1 promoter methylation showed abnormal hMLH1 protein expression with normal hMSH2 gene expression. All other patients tested demonstrated normal hMLH1 and hMSH2 protein expression. MSI was found to be more prevalent in tumor stage mycosis fungoides (47%) than early stage disease (20%) and was associated with an older age of onset of mycosis fungoides. MSI may be a consequence of hMLH1 promoter hypermethylation in mycosis fungoides patients and may prevent transcription in a subset of patients. This suggests that the development of a mutator phenotype may contribute to disease progression in mycosis fungoides.

Adaptor Proteins, Signal Transducing↗
PubMed1983

Mycosis fungoides: an overview.

Mycosis fungoides (MF) is a lymphoma that appears to have a unique relationship with the skin. In recent years our knowledge about this disease has increased considerably, in particular regarding the nature of the malignant cells and their functional characteristics. Furthermore, there is mounting evidence suggesting a viral etiology. Since its first description by Alibert in the early part of the past century, MF has been the subject of much controversy in regard to fundamental issues such as clinical and histological criteria for the establishment of the diagnosis [1] as well as its relationship to other lymphomas and skin diseases. Some of this controversy remains today. Mycosis fungoides is a relatively uncommon disease, the incidence being in the order of 1-2 million per year [1,2]. As is the case with most neoplastic diseases, the incidence increases with age; however, relatively speaking MF is not exceptional among individuals in their twenties or thirties. As for sex distribution there is a slight preponderance of men [3-5].

Adult↗
PubMed2000

Failure of detection of mucin in the clear halos around the epidermotropic lymphocytes in mycosis fungoides.

Epidermotropic lymphocytes in mycosis fungoides typically reside in clear lacunae. The material forming this space is unknown. Thirty specimens from 30 patients with mycosis fungoides were stained with alcian blue, modified Mowry's colloidal iron and mucicarmine to determine if some form of mucin could be identified. Using these stains, no form of mucin was noted in the lacunae surrounding the epidermotropic lymphocytes of mycosis fungoides. The cause of the clear spaces around epidermotropic lymphocytes in mycosis fungoides remains unexplained, but is unlikely to represent mucin deposition.

Alcian Blue↗
PubMed1999

Biliary tract obstruction secondary to mycosis fungoides: a case report.

Mycosis fungoides is a cutaneous T-cell lymphoma that can disseminate to multiple organs. We report a patient who presented with obstructive jaundice caused by isolated involvement of the extrahepatic biliary tree by mycosis fungoides. Initially, endoscopic examinations and biopsies of the biliary tree and liver failed to reveal a cause for the obstructive symptoms. Finally, surgical resection of the gallbladder and extrahepatic ducts was performed. Examination revealed a dense, mixed lymphocytic infiltrate with atypical cells within the mucosa. Gene rearrangement studies confirmed the presence of a monoclonal T-cell population. The pattern of the gene rearrangement in the biliary tree was identical to that found in a previous skin biopsy that showed mycosis fungoides. Although liver involvement by mycosis fungoides is not uncommon, disease isolated to the extrahepatic biliary tree has not previously been reported. This case should alert clinicians and pathologists to yet another cause of obstructive jaundice.

Adult↗
PubMed1987

Diffuse, progressive hyperpigmentation: an unusual skin manifestation of mycosis fungoides.

Pigmentary changes in mycosis fungoides usually occur in association with poikiloderma atrophicans vasculare or following therapy and regression of lesions. Several cases of hypopigmented mycosis fungoides have also been reported. We present the case report of a patient who developed pruritic, diffuse macular hyperpigmentation of the skin. Biopsy specimens from hyperpigmented skin revealed histologic and ultrastructural features typical of mycosis fungoides. Giant melanin granules were found in the tumor cells, as well as in keratinocytes and Langerhans cells. As far as we know, this is the first report of cutaneous hyperpigmentation as a single presenting sign of mycosis fungoides.

Aged↗
PubMed2000

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.

Diagnosis, Differential↗
PubMed2002

Treatment of childhood mycosis fungoides with topical PUVA.

Mycosis fungoides is the most common type of cutaneous T-cell lymphoma, which is usually observed in mid to late adulthood. We report 5 cases of mycosis fungoides in children, all presenting as patch- and plaque-stage disease most commonly involving the buttocks. Histologic examination showed in every case the typical features of mycosis fungoides. In 4 of the 5 cases, the infiltrating lymphocytes were characterized by the T-cell phenotype CD3(+), CD4(+), CD8(+); and in 3 cases, a monoclonal rearrangement of the T-cell receptor gamma (TCR-gamma) gene was found. Three children received topical PUVA treatment, and the other two were treated with mid-potency topical corticosteroids, resulting in complete clinical remission. A management approach to mycosis fungoides with topical PUVA may be appropriate for children.

Buttocks↗
PubMed1977

[Morphology and monocytopoesis of mycosis fungoides].

Nature and nosology of mycosis fungoides have to be reconsidered because there is now evidence of the t-cell nature of the atypical lymphoid cells within the mycoside infiltrate. Therefore the concept of the reticulum cells in the pathogenesis of mycosis fungoides must be corrected. On the ground of morphological and immunological reasons these cells do not exist in the dermis at all. These new findings are the basis of our morphological study in patients with mycosis fungoides. Concentrating on the polymorphous lymphohistiocytic cells, which cannot be classified further by the method of paraffin-thin sections, light microscopically, the exact differentiation is only possible with semi-thin-sections. With this technique there can be made visible the typical features of nuclei of mycosis fungoides cells. The mycosis fungoides cells are characterized by a large nucleus with hyperconvoluted nuclear membranes, prominent nucleoli, irregular distribution of heterochromatin and sparse cytoplasma. The monocytopoiesis, scheduled by the relative number and the 3H-thymidine labeling indices of promonocytes and the activity of naphthol-AS-D-chloroacetate esterase in blood monocytes was markedly increased. These findings are of special interest, because macrophages are not only able to phagocyte but also play a crucial role in immunology. The elevated monocytopoiesis in all stages of mycosis fungoides points to a stimulation of the immunologic system. This supports the hypothesis of a persistent antigen, which stimulates by means of the functional intact monocyte-macrophage system the lymphocyte system leading to a permanent transformation with augmentation of these immunoblasts (mycosis fungoides cells).

Humans↗
PubMed1995

[Conjugal mycosis fungoides].

INTRODUCTION: The pathophysiology of mycosis fungoides remains uncertain but HTLV I or a similar virus could be involved. We observed a couple who developed mycosis fungoides suggesting the infectious hypothesis might indeed be valid. CASE REPORT: A 70-year-old man who had often travelled in foreign countries developed parapsoriasis en plaques, lymphomatoid papulosis and mycosis fungoides successively over a thirty year period. Several years after the first manifestation of mycosis fungoides, his wife also developed a single plaque of mycosis fungoides. The diagnosis was confirmed on pathology slides and immunohistochemistry tests as well as on the basis of T-receptor gene rearrangement in both patients. Search for HTLV I was negative using serology tests and PCR on circulating lymphocytes. COMMENTS: The epidemiological situation in our observation (several trips in foreign countries and the delayed development of mycosis fungoides in the wife) favours the hypothesis of an infectious mechanism. Search for HTLV I was unsuccessful with classical virology methods. Certain recent work suggests a virus similar but different from the HTLV I virus could be involved in the pathogenesis of mycosis fungoides.

Aged↗
PubMed1988

Granulomatous variants of cutaneous T-cell lymphoma. The histopathology of granulomatous mycosis fungoides and granulomatous slack skin.

Granulomatous mycosis fungoides is an unusual histologic variant of mycosis fungoides, a condition that is ordinarily indolent. Granulomatous slack skin, like granulomatous mycosis fungoides, shows epidermotropism, granulomatous inflammation, a clonal T-helper cell population, and progression to systemic lymphoma in some cases. Unlike granulomatous mycosis fungoides, it is characterized clinically by bulky, pendulous skin folds. The similarities between the two conditions prompted us to compare the histologic features. We reviewed 24 biopsies from 10 patients with granulomatous mycosis fungoides. These showed several distinct histologic patterns, including three cases that mimicked granuloma annulare. We also reviewed biopsy specimens from four patients with granulomatous slack skin. These specimens had a more stereotypic appearance, with permeation of the entire dermis and subcutis by lymphocytes, marked epidermotropism, and a more even distribution of granulomas and giant cells within the infiltrate. Biopsies of fully developed lesions of granulomatous slack skin showed elastolysis involving the full thickness of the dermis--a feature not seen in any of our granulomatous mycosis fungoides cases. Biopsy specimens from granulomatous mycosis fungoides and granulomatous slack skin may be mistaken for nonneoplastic granulomatous dermatitides, but they can usually be distinguished from these by the presence of epidermotropism or atypical lymphocytes. Because several of our patients with granulomatous mycosis fungoides died after courses of unremarkable length, it seems unlikely that the presence of granulomas is invariably correlated with a more benign course than nongranulomatous mycosis fungoides.

Adolescent↗
PubMed1986

Cutaneous malignancies and metastatic squamous cell carcinoma following topical therapies for mycosis fungoides.

Specific treatments for mycosis fungoides, including electron beam irradiation, topical mechlorethamine, and psoralen plus ultraviolet A (PUVA) may be associated with the development of skin cancers after a variable latency period. Because these treatments are often not curative, topical therapies for mycosis fungoides, administered sequentially or concomitantly, are being used increasingly in order to control recurrent disease. This report documents the development of multiple cutaneous tumors, including squamous cell carcinoma, basal cell carcinoma, actinic keratoses, keratoacanthomas, and one case of lentigo maligna, in seven patients who received topical therapies for mycosis fungoides. In contrast to the usual latency period between ionizing radiation therapy and the development of skin cancer, two of our patients who had received prior PUVA therapy developed multiple skin tumors upon completion of electron beam irradiation. The development of metastatic squamous cell carcinoma in two of the other seven patients with multiple cutaneous neoplasms suggests that this potential hazard must be considered in the evaluation and treatment of patients with mycosis fungoides.

Administration, Topical↗
PubMed1977

Lymphography in the assessment of mycosis fungoides.

Extracutaneous manifestations of mycosis fungoides imply a bad prognosis and are a major cause of death. Benign dermatopathic lymphadenopathy is associated with mycosis fungoides and often precedes lymphomatous infiltration. In this study, 10 patients in the early stages of mycosis fungoides underwent clinical and lymphographical examinations. In one the lymphoma was already present in lymph nodes. Six had signs of dermatopathic adenopathy which was verified by lymph node biopsy in 5. In one of these the disease later progressed to a malignant lymphoma. The frequent occurrence of lymph node involvement justifies the use of lymphography collaterally with staging laparotomy to determine the presence of pathologic retroperitoneal lymph nodes.

Adult↗