Mycosis fungoides—response to therapy and survival patterns in 85 cases

The clinical course and management of 85 cases of mycosis fungoides presenting over a 20-year period are described. The extent of skin involvement and presence of lymphadenopathy were seen to be important prognostic factors. The following survival patterns were observed. In eight cases with limited plaque disease there was prolonged disease-free survival after treatment with kilovoltage X-rays; after a median follow-up of 64 months there was only one case of progression to generalized disease. In 49 cases of generalized skin disease (plaque disease +/- tumours; erythroderma) treated with total skin electron beam therapy there was a 24% 5-year disease-free survival; this potential for long-term survival was seen in cases with generalized plaque disease without lymphadenopathy. The remainder of the cases showed a continuously relapsing course. The extent of skin involvement and presence of lymphadenopathy can both be used as known prognostic factors to adopt a logical plan of treatment.     

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.     

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.     

Mycosis fungoides: a retrospective study

The clinical course of ninety patients with the histopathologic diagnosis of mycosis fungoides is reviewed. An attempt is made to correlate sex, age, of onset, lesion type, and form of therapy with outcome of the disease. The data indicate that mycosis fungoides affects predominantly middle-aged males. Patients developing tumors or erythroderma in middle age or later tend to have a shorter survival. More aggressive therapy is associated with shortened survival. Most deaths were due to unknown or unrelated processes and therapeutic complications. The data support the theory that mycosis fungoides can be a relatively nonaggressive cutaneous lymphoma. In an attempt to treat aggressively, we may be exposing patients to increased mortality.     

Mycosis fungoides: a dermatological masquerader

Mycosis fungoides (MF), a low-grade lymphoproliferative disorder, is the most common type of cutaneous T-cell lymphoma. Typically, neoplastic T cells localize to the skin and produce patches, plaques, tumours or erythroderma. Diagnosis of MF can be difficult due to highly variable presentations and the sometimes nonspecific nature of histological findings. Molecular biology has improved the diagnostic accuracy. Nevertheless, clinical experience is of substantial importance as MF can resemble a wide variety of skin diseases. We performed a literature review and found that MF can mimic >50 different clinical entities. We present a structured framework of clinical variations of classical, unusual and distinct forms of MF. Distinct subforms such as ichthyotic MF, adnexotropic (including syringotropic and folliculotropic) MF, MF with follicular mucinosis, granulomatous MF with granulomatous slack skin and papuloerythroderma of Ofuji are delineated in more detail.     

Mycosis fungoides: a morphological study

Skin biopsies from nineteen biopsy proven patients with mycosis fungoides were studied by using a contrasting dichrome stain in 0.5 μm thin sections, and comparing the results to those of normal skin and non-lymphomatous dermatoses. An abnormal cellular component was observed in mycosis fungoides, consisting of cells that exhibited a great diversity in cellular morphology. In addition to large and small types of ‘mycosis cells’, variants with less indented nuclei and three types of blast cells were identified. Intermediate forms suggested a process of differentiation. All types showed a basic similarity in form to a normal lymphoid cell of human skin, but they differed by their larger size, more pronounced nuclear grooving and nuclear hyperchromasia.     

Mycosis fungoides: disease evolution and prognosis of 309 Dutch patients

OBJECTIVES: To determine the disease course of Dutch patients with mycosis fungoides and to define factors related to disease progression and survival. DESIGN: A multicenter, 13-year, retrospective cohort analysis. SETTING: Eight dermatology departments collaborating in the Dutch Cutaneous Lymphoma Group. PATIENTS: Three hundred nine patients with mycosis fungoides registered between October 1985 and May 1997, including 89 patients with limited patches or plaques (stage Ia), 135 with generalized patches or plaques (stage Ib), 46 with skin tumors (stage Ic), 18 with enlarged but uninvolved lymph nodes (stage II), 18 with lymph node involvement (stage III), and 3 with visceral involvement (stage IV). MAIN OUTCOME MEASURES: Response to initial treatment, sustained complete remission, actuarial disease progression, and overall and disease-specific survival per clinical stage. RESULTS: The median follow-up was 62 months (range, 1-113 months). For the entire group, the actuarial overall and disease-specific survival was 80% and 89% at 5 years, and 57% and 75% at 10 years, respectively. The actuarial 5-year disease-specific survival of patients with stage Ia, Ib, and Ic disease was 100%, 96%, and 80%, respectively, and only 40% for patients with stage III disease. Using multivariate analysis, the presence of extracutaneous disease, the type and extent of skin involvement, the response to initial treatment, and the presence of follicular mucinosis were independently associated with higher disease progression and mortality rates. The calculated risks of disease progression at 5 and 10 years gradually increased from 4% to 10% for those with stage Ia disease, from 21% to 39% for those with stage Ib disease, and from 32% to 60% for those with stage Ic disease; for those with stage III disease, the risk remained at 70% at 5 and 10 years. The overall risk of disease progression at 5 and 10 years was 24% and 38%, respectively, for the total study group. CONCLUSION: At least within the first 10 years after diagnosis, disease progression and mycosis fungoides-related mortality occur in only a subset of patients generally presenting with advanced disease.     

A question of persistence: Langerhans cells and graft‐versus‐host disease

Langerhans cells (LCs) have been scrutinized many times in studies of the pathogenesis of graft‐versus‐host disease (GVHD). As migratory dendritic cells, LCs are capable of direct antigen presentation to cytotoxic T cells. Their self‐renewal capacity has led to speculation that persistent recipient LCs could provide a continuous source of host antigen to donor T cells infused during hematopoietic stem cell transplantation (HSCT). In this issue of Experimental Dermatology, a new study examines at the relationship between recipient LCs and chronic GVHD.     

Tumor Stage Mycosis Fungoides in a Child

Mycosis fungoides is uncommon in children and most often presents as stage IA/IB. We present a case of stage IIB mycosis fungoides in a 13‐year‐old boy and discuss diagnostic examination and treatment considerations.     

大疱性蕈样肉芽肿

报告1例大疱性蕈样肉芽肿.患者男,40岁.因躯干、四肢红斑伴瘙痒6年,出现斑块半年、水疱1个月就诊.皮肤科检查:面部、躯干及四肢泛发红斑和斑块,部分斑块浸润明显.右下腹斑块上可见0.5 cm×2 ca的水疱.皮损组织病理检查:真皮浅层明显水肿,部分区域可见表皮下疱形成,真皮浅层可见异形明显的单一核细胞呈带状浸润.部分单一核细胞侵入表皮,形成Pautrier微脓肿.免疫组化染色结果示LCA( )、CD4( )、CD45RO( ).结合临床、免疫组化和组织病理检查结果,诊断为大疱性蕈样肉芽肿.     

Hypopigmented Mycosis Fungoides in a White Female

A rare case of a young Caucasian female with hypopigmented mycosis fungoides is described. We reviewed and discussed the literature.     

Mycosis fungoides and Sézary syndrome: diagnostic and prognostic relevance of cellular antigen expression

Mycosis fungoides and Sézary syndrome (cutaneous T-cell lymphomas [CTCL]) are recognized, especially in the early stages, as being a potentially difficult diagnostic problem when they have to be histologically differentiated from a series of inflammatory disorders (ID). The diagnostic value of immunophenotyping is controversial; almost all studies have been semiquantitative. The prognostic implications of the antigen expression in CTCL are not known. To acquire more insight into these problems, the expression of differentiation-associated and activation/proliferation-associated antigens was examined in cryostat sections from 35 patients with conclusively diagnosed CTCL and 19 patients with ID. Positive cells were calculated as numbers/mm2 tissue section. The results were analysed statistically. Single antigen expressions were not helpful for differentiation between CTCL and ID. High percentages (89%-96%) of patients with CTCL stages I, II, and III and patients with ID could be classified correctly (discriminant analysis) by combined quantitative assessment of the expression of antigens Ki67 and Ki1/CD30 in the dermis and CD25 in the epidermis. In later stages (IVa, IVb) the note of correct classifications was worse because of the decrease in discriminating properties of the respective antigens. The biological role of antigen expressions was analyzed together with the clinical parameters (age, stage of disease). Stage was the parameter with the most significant influence on patient survival. CD1a-positive epidermal cells (Langerhans cells) were the only cell population that was significantly (P = 0.011) associated with survival. Death resulting from CTCL was significantly (P = 0.003) less frequent in patients with epidermal CD1a-positive cell densities higher than 90 cells/mm2 in comparison with patients with lower numbers.(ABSTRACT TRUNCATED AT 250 WORDS)     

Pathogenesis of Mycosis fungoides

Mycosis fungoides is the most common type of primary cutaneous lymphomas. The phenotype of the tumor cell corresponds to an effector/memory‐type of helper T cell which, given its repertoire of homing receptors, is specialized for recirculation through the skin. In recent years genetic analyses have uncovered various chromosomal aberrations in the tumour cells of mycosis fungoides. Their relevance to the pathogenesis and clinical appearance are discussed in the following.