Clinical Features

Specific skin lesions occur in approximately 8 of patients with B-CLL. The reported incidence of cutaneous lesions ranges from 4 to 45 (3), if one takes account of nonspecific findings including purpura, ecchymoses, and maculopapular eruptions. Specific lesions usually present as red or violaceous macules, papules, or nodules (4-7). Single or multiple lesions may be present. Lesions are typically seen on the face, particularly the ears, but may also frequently be seen on the scalp, trunk, and...

Soft Xray Orthovolt Radiotherapy

X-rays are electromagnetic radiations of high energy. When x-rays are used to treat superficial lesions, the energy spectrum of the x-ray beam is adjusted by selecting an appropriate combination of x-ray tube filtration and voltage, which is 30-100 kV (soft x-rays) in dermatology settings. Treatment of localized lesions and teleroentgen therapy with soft x-rays are two different approaches. The first Nobel Prize in physics was awarded to Hans Conrad Rontgen in Wurzburg, Germany, for his...

Historical Perspectives

The term parapsoriasis is confusing and requires explanation. It encompasses a number of different pathologic states clinically manifested by chronic recalcitrant erythematous scaling lesions. About 100 years ago, Unna et al. (1) described two cases of the so-called parakeratosis variegata. Brocq (2), some years later, saw one of Unna and colleagues' cases, as well as some similar cases, and described it as erythrodermies pityriasiques en plaques desseminees.'' Brocq (1856-1928) in his article...

Classic Paraneoplastic Diseases

The most common paraneoplastic skin disorders comprise acanthosis nigricans (2), acrokeratosis Bazex (3), erythema gyratum repens (4) (Fig. 1), necrolytic migratory erythema (5), acquired hypertrichosis lanuginosa (6), and the Leser-Trelat sign (7). These paraneoplastic dermatoses are mostly associated with solid tumors, in particular those involving visceral organs such as the lung and gastrointestinal system. Only rarely, do these dermatoses indicate the presence of nodal or primary cutaneous...

Extracorporeal Photopheresis ECP

The ECP is based on extracorporeal irradiation of circulating pathogenic T cells (e.g., malignant or autoimmune cells) with UV-A light in the presence of photosensitizing psoralens. It was developed in the late 1970s and early 1980s by Edelson and colleagues at the National Institutes of Health and later Yale University with the primary goal to treat patients with a leukemic form of cutaneous T-cell lymphoma i.e., SS. One rationale for targeting phototherapy to blood cells circulating in SS...

References

Letter HLA and mycosis fungoides. Lancet 1976 1 1179. 2. Shelley WB. Familial mycosis fungoides revisited. Arch Dermatol 1980 116 1177-1178. 3. Cameron OJ. Mycosis fungoides in mother and in daughter. Arch Dermatol Syphilol 1933 27 232-236. 4. Sandbank M, Katzenellenbogen I. Mycosis fungoides of prolonged duration in siblings. Arch Dermatol 1968 98 620-627. 5. Matutes E, Spittle MF, Smith NP, Eady RA, Catovsky D. The first report of familial adult T-cell leukaemia...

Molecular Biology

Monoclonal rearrangement of IgH genes has been demonstrated in the skin lesions 9 . Cutaneous involvement in Waldenstrom macroglobulinemia Clinical features Elderly adults, multiple systemic symptoms including hyperviscosity syndrome Specific cutaneous changes with IgM storage papules and neoplastic cell infiltration Histological features Storage papules deposits of IgM as hyaline amorphous material Small to medium-sized plymphs Immunophenotype CD19 , CD20 , CD5-, CD10-, CD23-, CD79a , CD138 -,...

Lymphomatoid Contact Dermatitis

Contact Dermatitis Lymphomatoid

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...

Histology

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...

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 ....

Other Variants of Mycosis Fungoides

Mycosis Fungoides Face

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...

Roman Specker

Indiae Utrisque Naturali Medica

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....

Immature Hematopoietic Malignancies

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...