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

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

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

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

Mature T-cell leukemias are a group of neoplasms derived from mature or post-thymic T-cells, and a number of distinctive disease entities have been defined in the World Health Organization (WHO) classification. Here we report a 54-year-old female patient with multi-lobated atypical cells expressing the classic T-cell antigens involving multiple lymph nodes, peripheral blood, and bone marrow. The clinical, laboratory, and pathologic features of her disease did not fit into any of the entities in the WHO Classification. There was no evidence of rapidly rising lymphocyte counts, TCL1 expression, eosinophilia, erythroderma, Sezary cells, autoimmune phenomena, cytotoxic granules, nor evidence of HTLV-1 infection, and thus, T-cell prolymphocytic leukemia, Sezary syndrome, T-cell granular lymphocytic leukemia, and adult T-cell leukemia/lymphoma were all ruled out. This case suggests that further characterization and definition of the "unclassifiable" cases of mature T-cell neoplasm is needed to better understand the group of disorders.
Adult ; Bone Marrow ; Classification ; Eosinophilia ; Female ; Glycogen Storage Disease Type VI ; Human T-lymphotropic virus 1 ; Humans ; Leukemia, Lymphoid ; Leukemia, Prolymphocytic, T-Cell ; Leukemia, T-Cell* ; Lymph Nodes ; Lymphocyte Count ; Middle Aged ; Sezary Syndrome ; T-Lymphocytes* ; World Health Organization

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

No Abstract Available.
Sezary Syndrome*

Sezary syndrome (SS), one of the cutaneous T-cell lymphomas (CTCLs), is an erythrodermic variant of mycosis fungoides (MF). SS is characterized by a pruritic exfoliation or infiltrated erythroderma accompanied by circulating tumor cells with convoluted nuclei in the peripheral blood. The malignant cells in the SS usually have a mature CD4+ T-helper cell phenotype. The reported cases of SS are rare in Korea. We report a case of SS who had intractable erythroderma for 2 years and who subsequently developed generalized lymphadenopathy. The laboratory data revealed a white blood cell count of 61x109/L with 78% of lymphocytes, the majority having a convoluted or cerebriform nucleus. The skin biopsy showed Pautrier's microabscesses with dermal infiltrate of mononuclear cells containing convoluted nuclei. The lymph node biopsy demonstrated paracortical infiltration by convoluted lymphocytes. Consistent with his diagnosis, 92% of peripheral mononuclear cells expressed CD4+.
Biopsy ; Dermatitis, Exfoliative ; Diagnosis ; Korea ; Leukocyte Count ; Lymph Nodes ; Lymphatic Diseases ; Lymphocytes ; Lymphoma, T-Cell, Cutaneous ; Mycosis Fungoides ; Neoplastic Cells, Circulating ; Phenotype ; Sezary Syndrome* ; Skin

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*

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