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Sezary Syndrome*

Humans ; Lymphoma, T-Cell, Cutaneous ; diagnosis ; Male ; Middle Aged ; Sezary Syndrome ; diagnosis

Patients with idiopathic erythroderma have often been regarded to have a pre-Sezary syndrome because some of them have developed a cutaneous T-cell lymphoma during follow-up. Sezary syndrome is a form of leukemia-lymphoma characterized clinically by erythroderma, pruritus, adenopathy, and circulating atypical cells with cerebriform nuclei. We describe a case of Sezary syndrome in a 40-year-old man, who suffered from idiopathic erythroderma for 3 years. We suggest that close and long-term follow-up should be performed on patients with idiopathic erythroderma.
Adult ; Dermatitis, Exfoliative ; Follow-Up Studies ; Humans ; Lymphoma, T-Cell, Cutaneous ; Pruritus ; Sezary Syndrome*

Aminopyridines ; Benzamides ; Humans ; Programmed Cell Death 1 Receptor ; Sezary Syndrome/drug therapy* ; Skin Neoplasms/drug therapy*

Sezary syndrome is a variant of cutaneous T-cell lymphoma (CTCL) featuring erythroderma, pruritus, adenopathy and circulating atypical T-lymphocytes. Recent studies suggest that patients with prolonged idiopathic erythroderma, possibility of Sezary syndrome should be considered because in a certain proportion of these patients, they may develop CTCL. We report a case of a 35-year-old man with a prolonged erythroderma for three years. On physical examination, multiple swellings of lymph nodes were noted. Presence of Sezary cells in the peripheral blood, skin and lymph nodes supported the diagnosis of Sezary syndrome.
Adult ; Dermatitis, Exfoliative ; Diagnosis ; Humans ; Lymph Nodes ; Lymphoma, T-Cell, Cutaneous ; Physical Examination ; Pruritus ; Sezary Syndrome* ; Skin ; T-Lymphocytes

Sezary syndrome in case of the aggressive systemic lymphoma. This disease is 2.2 times more common in man than woman and the etiology remains unknown. It is difficult to diagnose accurately from severe inflammation in early stage although it can be diagnosed through the clinical findings and histopathological findings. We experienced a 64-year-old male patient who assumed mycosis fungoides and had an eczema like mass in scalp of skin. In this case, we diagnosed this case as mycosis fungoides through observation of the atypical lymphocytes infiltration in the dermis and microabscess in the basal layer of the skin histopathologically.]]>
Dermis ; Eczema ; Female ; Humans ; Inflammation ; Lymphatic Diseases ; Lymphocytes ; Lymphoma ; Male ; Middle Aged ; Mycosis Fungoides ; Scalp ; Sezary Syndrome ; Skin ; T-Lymphocytes

Cutaneous T-cell lymphomas other than mycosis fungoides and Sezary syndrome are heterogeneous; they deseve further scientific attention about their natural history and effective therapy. Pleomorphic T-cell lymphoma is a recently defined lymphoma type that can occur in the skin. We report the case of a man in whom such a tumor manifested itself with multiple subcutaneous lesions. The skin biopsy specimen showed diffuse dermal infiltration of atypical lymphocytes with highly pleomorphic nuclei. Testing for the antibody against HTLV-1 was negative and immunohistochemical staiiiing was compatible with pleomorphic T-cell lymphoma.
Biopsy ; Human T-lymphotropic virus 1 ; Lymphocytes ; Lymphoma ; Lymphoma, T-Cell* ; Lymphoma, T-Cell, Cutaneous ; Mycosis Fungoides ; Natural History ; Sezary Syndrome ; Skin ; T-Lymphocytes*

BACKGROUND: Actinic reticuloid is a severe persistent photodermatitis that usually affects older men. It is characterized by extreme photosensitivity to a broad spectrum of ultraviolet radiation. Actinic reticuloid has many of the clinical and histological features of mycosis fungoides and Sezary syndrome. No Korean studies are available regarding a histopathological and immunohistochemical comparison of actinic reticuloid and mycosis fungoides. OBJECTIVE: This study was designed to evaluate the histopathological characteristics and conduct a immunophenotypic analysis to distinguish actinic reticuloid from mycosis fungoides. METHODS: We reviewed the histomorphological findings of 10 patients diagnosed with actinic reticuloid and those of 15 patients diagnosed with mycosis fungoides from 1996 to 2012 at our clinic. In addition, an immunophenotypic analysis was performed on the skin to assess the proportions of CD4+ and CD8+ T cells in all patients. RESULTS: We found a broad overlap in non-specific inflammatory histological features and more prominent histological features between the two diseases. Irregular acanthosis, vertically-streaked collagen in the papillary dermis, thickened and increased blood vessels, and an eosinophil and plasma cell infiltrate were significantly higher in patients with actinic reticuloid than in those with mycosis fungoides. Stuffed lymphocytes in dermal papillae and a psoriasiform lichenoid pattern were observed significantly more often in patients with mycosis fungoides. Solar elastosis (80%) was seen only in the actinic reticuloid cases. CD8-positive T cells were predominant in the epidermis in eight of 10 patients with actinic reticuloid. In contrast, CD4-positive T cells in the epidermis and dermis predominated in the majority of patients with mycosis fungoides (73.3%) compared with CD8-positive T cells. CONCLUSION: Histopathological findings and immunophenotypic analyses may be an important adjunct to distinguish actinic reticuloid from mycosis fungoides, but a correlation with clinicopathological findings, phototest, patch test, and photopatchtest is necessary to reliably distinguish actinic reticuloid from mycosis fungoides. Further prospective studies should be conducted on a larger number of cases regarding the differences between actinic reticuloid and mycosis fungoides.
Actins ; Blood Vessels ; Collagen ; Dermis ; Eosinophils ; Epidermis ; Humans ; Lymphocytes ; Male ; Mycosis Fungoides ; Patch Tests ; Photosensitivity Disorders ; Plasma Cells ; Sezary Syndrome ; Skin ; T-Lymphocytes

BACKGROUND: Peripheral T-cell lymphomas(PTCL) frequently involve the skin and may occur as distinct clinicopathologic entities. However, clinicopathologic features of PTCLs involving the skin other than mycosis fungoides and Sezary syndrome have been poorly understood. OBJECT: This study aimed to characterize the nature of PTCL involving the skin in the Korean population. METHODS: Twenty-three cases of PTCL which involved the skin were collected during period from July, 1991 to June, 1995 In addition to reviewing the clinical records and routine histologic slides of t.hese cases, the immunohistochemical studies with several antibodies and in situhybridization for Epstein-Barr virus(EBV) early RNAs were performed on selected cases. RESULTS: We recognized five subtypes; mycosis fungoides, 5 cases, angiocentric T-cell lympho-ma, 9 cases, diffuse large cell lymphoma, 4 cases(Ki-1 positive, 3 cases, Ki-1 negative, 1 case), subcutaneous T-cell lymphoma, 1 case, and unspecified PTCL, 4 cases. Mycosis fungoides tends to involve papillary dermis with epidermotropism, where as angiocentric T-cell lymphoma and unspecified PTCI. predominantly affact adnexae, venules, and subcutis. Among 9 cases of angiocentric T-cell lymphoma, EBV RNAs were detected in 5 cases. In the prognostic point of view, mycosis fungoides and Ki- 1 positive large cell lymphoma were notably favorable and angiocentric T-cell lymphoma, especially EBV related, was guarded. CONCLUSION: A variety of the distinct entities of PTCL which frequently involve the skin were identified in the Korean population. In addition to the clinicopathologic information, immunophenotyping and EBV detection are thought to be crucial in diagnosing and predicting the behavior of T-cell neoplasms.
Antibodies ; Dermis ; Herpesvirus 4, Human ; Immunophenotyping ; Lymphoma* ; Lymphoma, Large B-Cell, Diffuse ; Lymphoma, T-Cell ; Lymphoma, T-Cell, Peripheral ; Mycosis Fungoides ; RNA ; Sezary Syndrome ; Skin* ; T-Lymphocytes ; Venules

Sezary syndrome is a variant of cutaneous T-cell lymphoma and likely represents the leukemic phase of mycosis fungoides. Sezary syndrome is characterized by generalized erythroderma, pruritus, generalized lymphadenopathy, and circulating atypical cells with cerebriform nuclei. Histopathological features are variable, as this is a later stage in the development of mycosis fungoides. Atypical lymphocytes in the dermis and Pautrier's microabscesses may be present on a histopathological examination but up to one-third of cases may have non-specific findings. Immunological findings supporting a diagnosis of Sezary syndrome include a predominance of CD4+ lymphocytes in both skin biopsy specimens and peripheral blood. In our case, a 48-year-old male was referred for evaluation of generalized hyperpigmentation with exfoliation on his entire body. We suspected that the clinical features represented Sezary syndrome. We obtained a lymph node biopsy, immunological cell typing, and a peripheral blood smear and diagnosed Sezary syndrome.
Biopsy ; Dermatitis, Exfoliative ; Dermis ; Humans ; Hyperpigmentation ; Lymph Nodes ; Lymphatic Diseases ; Lymphocytes ; Lymphoma, T-Cell, Cutaneous ; Male ; Middle Aged ; Mycosis Fungoides ; Pruritus ; Sezary Syndrome ; Skin