References Yzx
1. Alibert J. Tableau du pian fongoide. Description des maladies de la peau, observ es a l'Hopital Saint-Louis et exposition des meilleurs methodes suivies pour le traitment. Paris, Barrois L'Aine amp Fils, 1806. 2. Vidal E, Brocq L. Etude sur le mycosis fungoide. La France Med 1885 2 946-1019. 3. Letterer E. Aleukamische Retikulose. Frankfurt Z Path 1924 30 377-394. 4. Burg G, Braun-Falco O. Cutaneous lymphomas, pseudolymphomas, and related disorders. Berlin Springer Verlag, 1983. 5....
Cbcl And Bcell Pseudolymphoma
Lymphoid cells of B-cell lineage express CD20 in most cases. CD19, CD22, and CD79a are also useful to verify the B-cell type. Follicular structures can be easily demonstrated by the presence of CD21-positive follicular dendritic cells FDC forming networks engulfing germinal center cells such as centroblasts and centro-cytes. CD138 antigen is useful for identification of plasma cell differentiation. Immu-nophenotypic features of mature B-cell neoplasms are summarized in Table 4. Often, a...
Classification of Nodal Lymphomas in Historical Perspective
Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts, U.S.A. Considerable progress has been made in the classification of nodal lymphomas. The earliest classification schemes were based strictly on morphology. The Jackson and Parker classification included lymphosarcoma, reticulum cell sarcoma, and giant follicle lymphoma Brill-Symmer disease corresponding to small lymphocytic lymphoma, large cell lymphoma, and follicular lymphoma, respectively. Other rare types...
References Ibb
1. Kipps T. Chronic lymphocytic leukemia. Curr Opin Hematol 2000 7 223-234. 2. Wierda WG, Kipps T. Chronic lymphocytic leukemia. Curr Opin Hematol 1999 6 253-261. 3. Ratnam KV, Khor CJL, Su WPD. Leukemia cutis. Dermatol Clin 1994 12 419-431. 4. Su WPD, Buechner SA, Li CY. Clinicopathologic correlations in leukemia cutis. J Am Acad Dermatol 1984 11 121-128. 5. Su WPD. Clinical, histopathologic, and immunohistochemical correlations in leukemia cutis. Semin Dermatol 1994 13 223-230. 6. Buechner...
Clinical Features Peb
Sezary syndrome is an uncommon CTCL which accounts for less than 5 of CTCL 4,5 . Sezary syndrome is characterized by distinct clinical features with erythroderma accompanied by intense and generalized pruritus, diffuse alopecia, palmoplantar hyperkeratosis and onychodystrophy as well as lymphadenopathy Figs. 1-3 . In the peripheral blood, lymphoid cells with hyperconvoluted nuclei so-called Sezary or Lutzner cells of varying size 8 to gt 12 micrometer in diameter and number are found 6 ....
Lymphomatoid Contact Dermatitis
Lymphomatoid contact dermatitis has been reported as a reaction to various allergens including nickel 6,7 , cobalt naphthenate 8 , isopropyl-diphenylenediamine 9 , and others. Genotyping has shown clonal rearrangement in some cases. Such cases may be closely related to clonal dermatitis some of which develop into overt CTCL 10 . Histologically, epidermotropism of lymphocytes and accumulations of CDla-positive Langerhans cells may be found Figs. 4 and 5 . Evolution into cutaneous T-cell lymphoma...
Actinic Reticuloid
Ive et al. in 1969 13 first described actinic reticuloid AR as a chronic dermatosis associated with severe photosensitivity and bearing a clinical and histological resemblance to malignant lymphoma, especially to Sezary syndrome. Nowadays, AR Figure 5 Accumulations of Langerhans cells expressing CD1a. Figure 5 Accumulations of Langerhans cells expressing CD1a. Figure 6 Eczema-like red and scaly infiltration of the sun-exposed skin. belongs to the group of so-called chronic actinic dermatitis....
Pseudolymphomatous Infiltrates in the Context of Other Skin Disorders
Department of Dermatology, University Hospital, Zurich, Switzerland There are reactive lymphocytic infiltrates that can be referred to as pseudolympho-matous reactions in an even broader sense. These infiltrates have been observed in infestations such as scabies, viral infections e.g., molluscum contagiosum , after use of Hirudo medicinalis Fig. 1 , or in neoplasms, especially those of epithelial origin, which can be accompanied by a significant lymphocytic infiltrate, sometimes with follicular...
Histology Cyo
A superficial perivascular infiltrate of small lymphoid cells is the most prevalent finding in skin biopsies of SS patients. Larger studies of SS biopsies demonstrated that a subepidermal perivascular or band-like infiltrate composed of predominantly small lymphocytes with or without nuclear atypia is the most common finding in SS, Figure 3 Palmar hyperkeratosis and erythema. Figure 3 Palmar hyperkeratosis and erythema. Figure 4 Sparse lymphocytic infiltrate in the upper dermis with few...
Definition Eis
Premalignant form of parapsoriasis with a tendency to evolve into mycosis fungoides or other CTCLs in up to 50 of the cases. Synonyms Prereticulotic poikiloderma, parapsoriasis en grandes plaques poikilodermiques poikiloderma vasculare atrophicans parapsoriasis lichenoides parakeratosis variegata. Figure 1 Large plaque parapsoriasis large erythematous and scaling plaques with slight poikiloderma on the buttocks. Figure 1 Large plaque parapsoriasis large erythematous and scaling plaques with...
Leg Type Clinical Features
Cutaneous DLBCL comprise about 1-3 of all cutaneous lymphomas and approximately 5-10 of cutaneous B-cell lymphomas. People over the age of 70 years are most commonly affected. There are contradictory reports on gender preponderance 3,4 . In the vast majority of patients, the tumor s are located on the lower legs Fig. 1 . They present with solitary or multiple red to violaceous dense nodules of Figure 2 Dense lymphocytic infiltrate in the upper and mid dermis with a more perivascular pattern in...
Clonal Tcell Disorders
Werner Kempf, Dmitry V. Kazakov, and G nter Burg Department of Dermatology, University Hospital, Zurich, Switzerland The T-cell receptor TCR is the antigen-specific receptor for T cells. During its differentiation, each T cell undergoes rearrangement of its TCR genes. This constitutes a unique signature or fingerprint for each T cell and all its clonal progeny 1 . In the past, detection of clonal rearrangement of TCR genes by Southern blot or PCR was widely used for differentiation between a...
Clonal Dermatitis
Clonality studies employing PCR techniques led to the identification of cases with chronic, nonspecific dermatitis harboring T-cell clones. These cases have been referred to as clonal dermatitis 1 . Some of these patients progressed to overt CTCL. About 25 of clonal dermatitis cases progress to overt CTCL within five years 1 . These observations indicate that at least some cases of clonal dermatitis represent CTCL precursor lesions. Clonal dermatitis would thus be one of the earliest...
References Onh
1. Ploysangam T, Breneman DL, Mutasim DF. Cutaneous pseudolymphomas. J Am Acad Dermatol 1998 38 877-895. 2. Kaposi M. Idiopathisches multiples Pigmentsarkom der Haut. Arch Dermatol Syphilol 1872 4 265-273. 3. Baefverstedt B. Ueber Lymphadenosis benigna cutis. Eine klinische pathologischanatomische Studie. Acta Derm Venereol Suppl XI Stockh 1944 24 1-102. 4. Spiegler E. Uber die sogenannte Sarkomatosis cutis. Arch Dermatol Syphilol 1894 27. 5. Fendt H. Beitrage zur Kenntnis der sogenannten...
Cd
Clinically, disorders belonging to this category show a chronic long-standing course, no spontaneous regression in most cases, and no extracutaneous spread with involvement of visceral organs. In some cases, clonality of the infiltrate can be demonstrated. However, in most cases, the neoplastic cell clone never overcomes host control mechanisms and cannot expand and therefore never converts into clinically aggressive and spreading lymphoma. This category includes, for example large plaque...
Marshall E Kadin
Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts, U.S.A. The lymphoid system is comprised of B and T lymphocytes and dendritic antigen presenting cells. These cells have their origin in the bone marrow and undergo morphologic and functional maturation in peripheral lymphoid organs, including the thymus, lymph nodes, and spleen. Here circulating lymphocytes come into contact with antigen presenting cells which prime them for functional activity. B lymphocytes...
Lichen Planus And Psoriasis
Clonal T-cell populations have been found in inflammatory skin diseases such as lichen planus and psoriasis. Preferential usage of V beta 3 and or V beta 13.1 genes by the lesional CD8 T cells has been found in psoriatic lesions 5 . Various clones of T cells have been isolated from mucosal lichen planus 6 . Preferential use of V alpha 2 and V beta 3 of TCR was found on lymphocytes in mucosal lichen planus, whereas T-cells in Candida-induced lesions did not show a restricted TCR pattern 7 ....
Diffuse Growth Pattern
There is diffuse infiltration of the whole dermis with or without epidermotropism. Both the band-like feature of T-cell pattern and the nodular feature of B-cell pattern are lacking, even though the macroscopic correlates are usually large nodules or tumors. The cells are usually medium to large size. This pattern is typically seen in highly proliferative large B- or T-cell lymphomas or in non-lymphoid proliferations such as histiocytic tumors Fig. 4 . Figure 6 Interstitial indian file-like...
Classification of Cutaneous Lymphomas in Historical Perspective
Christian A. Sander and Michael J. Flaig Department of Dermatology, LMU, Munich, Germany Department of Dermatology, University Hospital, Zurich, Switzerland Going back in history, classification of cutaneous lymphomas began with the introduction of mycosis fungoides by Alibert in the last century 1 and was extended by the description of the d'emblee form by Vidal and Brocq in 1885 2 . In 1924, Letterer 3 introduced the term malignant reticulosis, which defined a proliferation of either benign...
References Roc
1. Waldenstrom J. Incipient myelomatosis or essential hyperglobulinemia with fibrinogen-openia new syndrome Acta Med Scand 1944 117 216-247. 2. Dimopoulos MA, Alexanian R. Waldenstrom's macroglobulinemia. Blood 1994 83 14521459. 3. Herrinton LJ, Weiss NS. Incidence of Waldenstrom's macroglobulinemia. Blood 1993 82 3148-3150. 4. Owen RG, Parapia LA, Richards SJ. Should Waldenstrom's macroglobulinaemia be considered part of the spectrum of marginal zone lymphoma Blood 1999 94 suppl 1 85 Abstract...
Other Variants of Mycosis Fungoides
Dmitry V. Kazakov, Werner Kempf, and G nter Burg Department of Dermatology, University Hospital, Zurich, Switzerland Apart from the classical form of mycosis fungoides MF , there are several variants of this disease with unusual or atypical clinical and or histopathological features Table 1 . Follicular MF has been given many names including folliculotropic MF, pilo-tropic MF, folliculocentric MF, and MF-associated follicular mucinosis 1-6 . Some authors believe that the latter should be...
Cutaneous Inflammatory Pseudotumor
Dmitry V. Kazakov, G nter Burg, and Werner Kempf Department of Dermatology, University Hospital, Zurich, Switzerland Inflammatory pseudotumor IPT plasma cell granuloma, inflammatory myofibroblasts pseudotumor refers to a spectrum of idiopathic benign conditions with unknown etiology that can develop in various organs and deep tissues, particularly in the lung. Whatever the anatomic location, the lesions share common histological features, namely good circumscription, proliferation of...
Differential Diagnosis Gxa
The most relevant differential diagnoses are primary cutaneous follicle center lymphoma FCL , immunocytoma and cutaneous B-cell pseudolymphoma B- Primary cutaneous marginal zone B-cell lymphoma Clinical features Adults aged over 40 years Red to violaceous infiltrated plaques or nodules with an erythematous periphery on trunk and arms Histological features Nodular or diffuse infiltrate of small- to medium-sized lymphocytes with pale cytoplasm Immunophenotype CD19 , CD20 , CD22 , CD79a , CD5-,...
Cutaneous Bcell Pseudolymphoma
G nter Burg, Werner Kempf, and Dmitry V. Kazakov Department of Dermatology, University Hospital, Zurich, Switzerland The prototype of B-cell pseudolymphoma B-PSL is Borrelia-associated lymphadenosis benigna cutis LABC . Other B-PSL includes tattoo-induced lymphocytoma cutis and postzoster scar lymphocytoma cutis. In some cases, the cause cannot be identified and are thus referred as idiopathic lymphocytoma cutis. There is a considerable overlap between T- and B-PSL as in persistent nodular...
References Rqs
1. Perniciaro C, Zalla MJ, White JJ, Menke DM. Subcutaneous T-cell lymphoma. Report of two additional cases and further observations. Arch Dermatol 1993 129 1171-1176. 2. Mehregan, Su WP, Kurtin PJ. Subcutaneous T-cell lymphoma a clinical, histopathologic, and immunohistochemical study of six cases. J Cutan Pathol 1994 21 110-117. 3. Salhany KE, Macon WR, Choi JK, Elenitsas R, Lessin SR, Felgar RE, Wilson DM, Przyblski GK, Lister J, Wasik MA, Swerdlow SH. Subcutaneous panniculitis-like T-cell...
Definition Whw
Most cases within this group cytomorphologically represent large-cell pleomorphic lymphomas with large pleomorphic cells which by definition do not express CD30 1,2 . It belongs to the group of noncytotoxic peripheral T-cell lymphomas. Recent studies show that many cases included previously in this group in fact, belong to extranodal T NK-cell lymphoma, blastic NK-cell lymphoma synonym agranular CD4 CD56 hematodermic neoplasm , and other distinct entities 3 . CD30- PLTCL per se is a rare...
Marshall E Kadin 1
Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts, U.S.A. Lymph nodes have a well-defined architecture including sinuses, cortical, paracor-tical, and medullary regions Fig. 1 . Sinuses are potential spaces lined by sinus histiocytes which are professional macrophages Fig. 2 . Cortical areas of the lymph node contain lymphoid follicles which bring developing B lymphocytes into contact with antigen expressing follicular dendritic cells Fig. 3 . Na ve B cells are...
References Gnr
1. Jorg B, Kerl H, Thiers BH, Brocker EB, Burg G. Therapeutic approaches in cutaneous lymphoma. Dermatol Clin 1994 12 433-441. 2. Duvic M. Current treatment of cutaneous T-cell lymphoma. Dermatol Online J 2001 7 3. 3. Tuyp E, Burgoyne A, Aitchison T, MacKie R. A case-control study of possible causative factors in mycosis fungoides. Arch Dermatol 1987 123 196-200. Figure 1 Stepwise approach of diagnosis in cutaneous lymphomas. 4. Whittemore AS, Holly EA, Lee IM, Abel EA, Adams RM, Nickoloff BJ,...
Tumor Burden Index
An alternative approach for the assessment of prognosis is to determine the tumor mass using the TBI. The calculation of the TBI is based on the rough clinical estimation of tumor mass obtained by multiplying the percentage of body surface involved by patches, plaques, and tumors by weighted factors. The procedure for calculating the TBI is given in Table 2. In a study based on the material collected by the EORTC between 1975 and 1995, whole body mapping of the skin lesions was performed in 116...
Leukemic Infiltrates Of The Skin
Histologic diagnosis of leukemic infiltrates may be very difficult when based on routine histologic examination and requires IHC phenotyping of tumor cells. Chloroacetate esterase, lysozyme, MPO, CD13, CD33, CD15, CD117, and CD16 are very useful markers, since leukemic infiltrates of monocytic or myelomonocytic origin are variably positive, whereas lymphoid cells are consistently negative for all these markers. The MPO activity in myeloblasts is granular and usually concentrated in the Golgi...
Roman Specker
Rehetobelstrasse, St. Gallen, Switzerland The first detailed medical descriptions of skin diseases can be found in the medical literature at the end of the 18th century, when scientists started to use the concept of the skin as an organ. Before that time, it made no sense to describe details of skin changes as we do today, since they were thought to be only signs of an uneven distribution of internal body fluids. These disturbances could much better be diagnosed by examination of urine....
Contributors
Stanislaw Buechner Department of Dermatology, University of Basel, Basel, Switzerland G nter Burg Department of Dermatology, University Hospital, Z rich, Switzerland Antonio Cozzio Department of Dermatology, University Hospital, Z rich, Switzerland Udo Doebbeling Department of Dermatology, University Hospital, Z rich, Switzerland Reinhard Dummer Department of Dermatology, University Hospital, Z rich, Switzerland Michael J. Flaig Department of Dermatology, LMU, Munich, Germany Michael Geiges...
Immature Hematopoietic Malignancies
37. Elastic NK-Cell Werner Kempf, Reinhard Dummer, Dmitry V. Kazakov, and G nter Burg Clinical Molecular Clinical Course and 38. Precursor Lymphoblastic 38.1. Precursor T-Lymphoblastic Werner Kempf, Dmitry V. Kazakov, and G nter Burg Clinical Molecular Clinical Course and Differential 38.2. Precursor B-Lymphoblastic Werner Kempf, Dmitry V. Kazakov, and G nter Burg Clinical Molecular Clinical Course and Differential 39. Cutaneous Manifestations of Hodgkin Marshall E. Kadin, Werner Kempf, and...
Cutaneous Bcell Lymphomas
31. Primary Cutaneous Marginal Zone B-cell Werner Kempf, Dmitry V. Kazakov, and G nter Burg Clinical Molecular Clinical Course and Clinical Molecular Clinical Course and Differential 31.2. Primary Cutaneous Plasmacytoma PCP 243 Dmitry V. Kazakov, G nter Burg, and Werner Kempf Clinical Molecular Clinical Course and Differential 31.3. Cutaneous Follicular Lymphoid Hyperplasia with Monotypic Plasma Cells 249 Dmitry V. Kazakov, G nter Burg, and Werner Kempf Clinical Molecular Clinical Course and...