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Background: In the course of mycosis fungoides, pilofollicular manifestations without mucinosis (papules, keratoses, comedones, or epidermal cysts) are rare (15 cases reported). Therefore, histological and clinical diagnoses may be difficult. The clinical course and histopathological and immunohistochemical findings in 9 patients are described., Observations: Pilofollicular lesions were present at the onset (n = 3), before (n = 3), or during a relapse of mycosis fungoides (n = 3). Comedones and epidermal cysts were most frequent (n = 5). They disappeared with lymphoma therapy (n = 4), therapy with isotretinoin (n = 3), or spontaneously (n = 1), or they persisted (n = 1). Clues to the histopathological diagnosis consisted of pilotropism of the infiltrate with minor alteration of the hair follicle walls. The infiltrate was monomorphous and composed of sezariform CD4+ lymphocytes. Pilotropic or peripilofollicular infiltrates, or the absence of infiltrate, were detected in consecutive biopsy specimens obtained from the same patient. The keratinocyte expression of intercellular adhesion molecule type 1 was observed in the hair follicle bulb in front of the pilotropic infiltrate but not in the epidermis. No staining was observed in biopsy specimens of 6 of 7 patients with follicular mucinosis, of folliculitis lesions, or of normal hair follicles., Conclusions: Our findings indicate the role of adhesion molecules in pilotropism leading to mechanical obstruction of the follicle by tumoral cells followed by hyperkeratosis and cyst formation. It remains to be determined if the expression of intercellular adhesion molecule type 1 is the cause or the consequence of pilotropism. By becoming more aware of it, this variant of mycosis fungoides is probably not so rare.

Besides the classical forms of cutaneous T-cell lymphoma (C-CTCL), such as mycosis fungoides and Sézary's syndrome, unique variants may be encountered. The classification of these rare cutaneous T cell lymphomas is problematic and controversial. Newer classifications of lymphoma in general, such as the Revised European-American Lymphoma (REAL) Classification, emphasize well-established clinico-pathological entities. It seems appropriate to attempt to bring greater clarity to the classification of cutaneous T-cell lymphomas using the same principles. In this review, we list and characterize the rare variants of cutaneous T-cell lymphoma, such as (1) clinical, histological and immunological variants of mycosis fungoides; (2) progressive cutaneous T-cell lymphoma (P-CTCL) including transformed classical cutaneous T-cell lymphoma (TC-CTCL) and primary progressive cutaneous T-cell lymphoma (PP-CTCL); (3) angiocentric and angioimmunoblastic cutaneous T-cell lymphomas; (4) large cell anaplastic, CD30+ cutaneous T-cell lymphoma; (5) HTLV-I-associated adult T-cell leukemia/lymphoma (ATLL); (6) cutaneous manifestations of primary extracutaneous T-cell neoplasias; (7) unclassifiable cutaneous T-cell lymphoma.

It is well documented that mycosis fungoides (MF), a cutaneous T-cell lymphoma, has a variable clinical course. Unbiased stereological estimates of three-dimensional volume-weighted mean nuclear size (nucl vV) of mycosis cells were obtained in a retrospective study of 18 patients with a total of 34 biopsies of cutaneous plaque and tumor-stage MF. The value of nucl vV in the first sampled biopsy, as well as the average and highest values, were determined in biopsies from each patient. The patients were divided into two groups, either above or below the group median. There was a strong positive correlation between shorter survival and an average value of nucl vV > 104 microns 3 or a highest value of nucl vV > 126 microns 3 (2p = 0.002 and 0.003, respectively). A nucl vV > 91.6 microns 3 in the first biopsy was also suggestive of a shorter survival (2p = 0.07). There was no clear pattern of nucl vV evolution in the patients with multiple biopsies, but the impact of various therapeutic regimens cannot be assessed. Certain estimates of nucl vV appear to be good prognostic indicators in plaque and tumor-stage MF, but further study of a larger series of patients is needed to corroborate these results and assess the impact of differing therapeutic regimes.

The term cutaneous T-cell lymphoma designates a group of neoplasms of skin homing T-cells that show considerable variation in clinical presentation, histological appearances, immunophenotype and prognosis. The disadvantages of currently available histological classification schemes are discussed and a new classification is presented. This is based on a combination of clinical, histological and immunophenotypic criteria and it recognizes distinct clinico-pathological entities within this group of diseases.

Mycosis Fungoides is a T-cell lymphoma having a broad clinical spectrum ranging from localized cutaneous to rapidly fatal systemic disease. Early clinical presentation is non specific, delaying correct diagnosis. Compared to clinical, the insurable spectrum is narrow. Staging for skin, lymph node and other organ manifestations is presented. Factors which influence mortality within each stage are elucidated. The survival curves of stages and stage groupings are illustrated and discussed to facilitate risk classification. Cutaneous (T) and lymph node (LN) stages are the most important prognosticators. Substages T1/T2, LN1/LN2 without associated palpable adenopathy, eosinophilia, visceral and blood positive findings are insurable. It would be most appropriate to place them in a tumor class of mild/moderate risk after the initial excessive mortality period ends. Higher T and LN substages, adenopathy and visceral disease have highly adverse mortality. These ultimately reveal a flattening of survival curves at 8-10 years. Although numerous treatment modalities have been used, none appear to consistently prolong life expectancy except in the earliest stage skin disease.

Background: This study was undertaken to compare three classification schemes used to evaluate lymph nodes (LN) obtained from patients with cutaneous T-cell lymphoma (CTCL): a modified Rappaport classification, the National Cancer Institute-Veterans Administration (NCI-VA) classification based on the relative numbers of cerebriform cells in the paracortical areas, and the Dutch classification based on the presence of cerebriform cells with large nuclei in mycosis fungoides (MF) and diffuse infiltration by cerebriform cells in Sézary syndrome., Methods: A study set of 195 LN obtained from patients with CTCL (MF, Sézary syndrome, and nonepidermotropic T-cell lymphomas) and 14 LN from patients with benign dermatoses was reviewed independently by three groups of pathologists familiar with each classification system., Results: Each classification system provided useful prognostic information. However, contrary to prior reports, no significant difference in survival was apparent in patients with uneffaced LN when classified according to the NCI-VA (LN0-2 versus LN3) or Dutch (Gr0-1 versus Gr2) ratings. In addition, all classification systems demonstrated a poor survival time associated with effaced LN. By combining results from the modified Rappaport and Dutch classifications, three prognostic groups could be identified based on cell morphology: a low-grade category with a small cell histologic subtype (median survival time, 40 months); a high-grade immunoblastic subtype (median survival time, 9 months) composed of cells with an oval nucleus containing a large, usually solitary central nucleolus; and an intermediate-grade category composed of all cases without the distinctive small cell and immunoblastic morphologies (median survival time, 26 months)., Conclusions: The authors propose that clearly involved LN in CTCL can be categorized on the basis of cell morphology into prognostic groups analogous to what has been proposed for the Working Formulation for Non-Hodgkin's Lymphomas for Clinical Usage.

Most patients with mycosis fungoides are between 40 and 60 years of age. The disease has three clinical stages: (1) the premycotic, or patch, stage, consisting of macular, scaling, faint pink to red pruritic patches, usually on unexposed surfaces; (2) the mycotic, or plaque, stage, consisting of reddish, purple-brown plaques, often annular in shape and symmetric in distribution, and (3) the tumor stage, consisting of red-brown to violaceous, dome-shaped, firm tumors with a predilection for the face and body folds. The Sézary syndrome is a leukemic variant. Treatment depends on the extent of disease and includes topical or systemic chemotherapy, radiotherapy and psoralen plus long-wave ultraviolet light therapy.

47 patients suffering from mycosis fungoides and 13 with Sézary's syndrome were retrospectively studied with regard to prognosis in relation to the histologic substrate of cutaneous lesions. On account of the wide spectrum of T-cell cytomorphology as well as the pattern and composition of the infiltrate, we distinguished 4 histopathological types: small cell lymphoid type (45%), mixed cell polymorphic type (40%), large cell type (immunoblastic and pleomorphic, 10%), and medium-sized cell type (5%). Statistical analysis revealed survival rates of 10 years in 68.3% for the small cell lymphoid type and in 30.3% for the mixed cell polymorphic type. All patients showing the large cell type died within 6 years after diagnosis; those bearing the medium-sized cell type died within 2 years after diagnosis. The forms of mycosis fungoides showing the last two types of infiltrate may be classified as lymphomas of extreme malignancy. The different histological features are not only significant with regard to the diagnosis but also permit prognostic estimation before systemic signs have become evident.

The authors present the historical evolution of the concepts of non-Hodgkin malignant lymphomas which lead to the new functional classifications of Lukes and of Kiel. These classifications are based on the discoveries of modern immunology and are relying on the marking techniques for the identification of the T, B and U lymphocytes. Arguments are presented why these modern nomenclatures should progressively replace the Rappaport classification which preceded the major developments of modern immunology. The Kiel nomenclature is briefly described.

The authors subdivide the primary non-Hodgkin cutaneous malignant lymphomas into "proper" and "non-proper" types. "Proper" lymphomas are those which have in the skin their proper site of localization, and include mycosis fungoides, Pagetoid reticulosis, Baccaredda-Sézary syndrome and possibly lymphomatoid papulosis. They are T-cell lymphomas arising in the papillary dermis, characterized by epidermotropism, having a specific clinical feature in that they are unlikely to be simulated by other cutaneous malignant lymphomas. "Non-proper" lymphomas are those which do not usually arise in the skin, but in various other organs. They are B-, T- or null-cell lymphomas, arising in the middle dermis, infrequently epidermotropic, having a papular-nodular-tumoural clinical feature, which are indistinguishable clinically from other neoplastic types such as plasmacytoma and Hodgkin's disease. The three classifications of non-Hodgkin lymphomas most followed are not directly applicable to cutaneous lymphomas because some of the former are not primarily sited in the skin, and because a follicular morphology is infrequently seen in the latter. Whereas the first classification reported for cutaneous lymphomas utilized malignancy as a criterion, the present classification here proposed utilizes the propriety of the site of localization as the criterion for subdivision into "proper" and "non-proper" types.

For the purpose of describing a new case of the Woringer-Kolopp disease, we are up-dating the problem and its classification and contributing clinical, histopathological, and ultrastructural facts which show that this is a lymphoma of the T cells and therefore should be classified along with the mycosis fungoides and the Sézary syndrome. The identification test of the lymphocytes and the ultrastructural study bring to light new facts of this disease.

It was the purpose of our studies to achieve reevaluation of the histo- and cytomorphology of cutaneous lymphomas by means of additional enzymecyto-chemical and funtional tests. Skin biopsy specimens from 99 patients with cutaneous lymphomas and pseudolymphomas were stained for HE, Giemsa, PAS, Gormori, several hydrolytic enzymes and peroxidase. In cell suspensions extracted from skin lesions B and T cell differentiation was performed using surface markers. In addition tissue cells were tested for PHA response in suspensions and for intracytoplasmatic Ig on smears. Based on these studies cutaneous lymphomas were filed, within the "Kiel" classification of low grade and high grade malignant lymphomas according to their histological, enzymecytochemical and immunological features. It got evident that B cell and T cell lymphomas of low grade malignancy in the skin both present distinct histological patterns, indicating areas B and T cell microenvironmental specificity.

Woringer-Kolopp disease is characterized by the presence of a monomorphic, tumorous infiltrate in the epidermis. A light- and electron microscopic study, and cytophotometry, of one case were performed and the results are discussed. No explanation has been found for the epidermotropic character of this condition, although an intraepidermal origin of the tumor infiltrate seems improbable. The cytomorphologic characteristics of the tumor cells shows a striking analogy to the atypical cells in the Sezary syndrome and mycosis fungoides. These findings support the hypothesis that Woringer-Kolopp disease represents a clinical variant of mycosis fungoides and the Sezary syndrome.