Leser-Trélat Sign Associated with Sézary Syndrome

A 74-year-old Japanese male had developed generalized erythroderma and rapid growth of multiple verrucous lesions over the entire surface of his face, trunk, and extremities three months before he was seen. Histologically seborrheic keratoses were revealed. Laboratory examinations showed peripheral leukocytosis with atypical lymphocytes and high levels of IgE and IgG. On the basis of these clinical and histopathologic findings, we diagnosed the patient as having Leser-Trélat sign associated with Sézary syndrome. The erythroderma subsided after administration of oral predonisone, and no new formations of seborrheic keratosis were observed. However, because of subsequent aggravation of the generalized erythroderma, we administered chemotherapy. Six months after the initial examination, lung cancer was found, and the patient subsequently died of respiratory and renal failure.     

CD25+ folliculotropic Sézary syndrome with CD30+ large cell transformation

Folliculotropic Sézary syndrome is a rare and unique variant of cutaneous T‐cell lymphoma (CTCL) characterised by both follicular and leukaemic involvement of mycosis fungoides (MF). It is associated with a more aggressive clinical course and fatal outcomes. Large cell transformation (LCT) of mycosis fungoides/Sézary syndrome is also associated with an aggressive disease course and shortened survival, requiring an intensive therapeutic approach. This report describes a case of folliculotropic Sézary syndrome with CD30+ LCT. Most of the larger lymphocytes in the lesions were positive for CD25, the expression of which is associated with advanced CTCL. In addition, we review the literature on this unusual CTCL and provide evidence that this entity represents a distinct clinicopathological entity occasionally associated with extracutaneous involvement and LCT.     

Sézary syndrome without erythroderma: A case report and review of published work

Sézary syndrome (SS) is defined by erythroderma and circulating atypical T cells, with or without lymphadenopathy. Recently, Thompson et al. identified a distinct population of SS patients with an atypical presentation: a high blood tumor burden of Sézary cells fulfilling criteria for SS but without fulfilling the criteria for erythroderma at the diagnosis. Here, we report a case of a 49‐year‐old Japanese man with SS who did not present with erythroderma initially, but exhibited erythematous itchy papules symmetrically located on the legs and arms. We also reviewed reported cases of SS without initial erythroderma. The skin manifestations at diagnosis varied from patches to tumors often seen in mycosis fungoides, and other rarer findings such as excoriation, palmoplantar keratoderma and alopecia. Pruritus was reported in most patients (86%), unlike early mycosis fungoides, and could be the main clue to the diagnosis of SS. Notably, three patients were reported to have presented with papular lesions, similar to our case. Little is known about why skin lesions in SS without erythroderma vary and why these cases did not exhibit erythroderma initially. Attenuated stimulation by colonized Staphylococcus aureus, impairment in recruitment of malignant T cells and suppression of inflammatory response induced by malignant T cells with regulatory phenotype may be associated with skin manifestations. Further studies are necessary to elucidate the etiology of this entity.     

Granulomatous slack skin‐like clinical findings in Sézary syndrome

Granulomatous slack skin (GSS) is a very rare condition that has been described as a variant of mycosis fungoides. It is characterized by the development of bulky and pendulous skin folds in flexural areas that are histologically formed by atypical T lymphocytes, histiocytes and giant cells. We report the case of a 37‐year‐old African‐American female with history of Sézary syndrome (SS) that while on treatment for the disease and in a space of 1 month developed exorbitant slack folds in the axillae and cervical area mimicking GSS. The absence of giant cells and epithelioid granulomas in the biopsy ruled out this diagnosis. We report this peculiar SS presentation that clinically resembles GSS, but with histopathology that does not show the typical features of this condition. We also review the literature in regard to SS, GSS and granulomatous mycosis fungoides (GMF), particularly the existing criteria to differentiate these various entities.     

New aspects of the clinicopathological features and treatment of mycosis fungoides and Sézary syndrome

Mycosis fungoides and Sézary syndrome are T‐helper (Th)2‐skewed cutaneous lymphomas. The clinical course of mycosis fungoides is classically indolent, manifesting as patches, plaques and tumors. Along with their progression, Th2 dominance tends to be accelerated. In this review, we discuss the epidemiology, clinicopathogenetic features and therapeutic approaches in mycosis fungoides and Sézary syndrome.     

Mycosis fungoides and Sézary syndrome: Australian clinical practice statement

Primary cutaneous lymphomas represent a heterogeneous group of T‐ and B‐cell lymphomas with distinct clinical presentations, histopathologic features, treatment approaches and outcomes. The cutaneous T‐cell lymphomas, which include mycosis fungoides and Sézary syndrome, account for the majority of the cutaneous lymphomas. This Clinical Practice Statement is reflective of the current clinical practice in Australia. An expanded form of the Clinical Practice Statement (and updates), along with helpful patient resources and access to support groups, can be found at the following ( http://www.australasianlymphomaalliance.org.au ).     

Increased interleukin‐36γ expression in skin and sera of patients with atopic dermatitis and mycosis fungoides/Sézary syndrome

Interleukin (IL)‐36γ is expressed by keratinocytes and functions as a key initiator of inflammation in the skin. IL‐36γ expression is enhanced by tumor necrosis factor‐α and IL‐17A, having a strong association with psoriasis. In this study, we examined the role of IL‐36γ in atopic dermatitis (AD) and mycosis fungoides (MF)/Sézary syndrome (SS). Serum levels of IL‐36γ in AD patients and MF/SS patients were elevated compared with those of healthy controls. Importantly, serum IL‐36γ levels in AD patients positively correlated with Eczema Area and Severity Index and those of MF/SS patients positively correlated with serum soluble IL‐2 receptor levels. IL‐36γ mRNA levels in AD skin and MF/SS skin were significantly higher than those of normal skin. IL‐36γ mRNA levels in MF/SS skin positively correlated with IL‐17A mRNA levels. Immunohistochemical staining revealed that IL‐36γ was highly expressed in keratinocytes in lesional skin of AD and MF/SS. Taken together, our study demonstrated that IL‐36γ expression was increased in sera and skin of patients with AD and MF/SS as was reported in psoriatic patients.     

Combination therapy with extracorporeal photopheresis,interferon‐α, PUVA and topical corticosteroids in the management of Sézary syndrome

Background: Extracorporeal photopheresis (ECP) is recommended for the treatment of Sézary syndrome (SS), the leukemic variant of cutaneous T‐cell lymphoma (CTCL). Several combination therapies are used to increase response rates to ECP. Patients and Methods: We report our experience with the combination therapy of ECP, interferon‐α, PUVA and topical corticosteroids in SS. Results: The treatment outcome in 12 SS patients was retrospectively analyzed and showed an overall response rate to this combination treatment of 42 % with 4/12 patients achieving a partial remission and 1/12 patients a stable disease. The median overall survival time was 42 months. We investigated several clinical and laboratory parameters as an indicator for a response to treatment in our patient cohort. A combined analysis of the erythroderma assessment scale, WBC, LDH, CD4/CD8 ratio and the number of Sézary cells revealed that a reduction of several parameters significantly correlated with response to treatment. The parameters which correlated best with response were number of Sézary cells, CD4/CD8 ratio and WBC. Conclusions: The investigated combination therapy was effective and well‐tolerated in a subgroup of SS patients but needs to be evaluated in a larger patient population.     

Lymphomatoid contact dermatitis *

Five elderly patients with recurrent and progressive erythematous patchy dermatitis had temporary flares of generalized erythema and associated positive patch tests. The histopathologic findings were of a distinct pattern of lymphocytic dermal reticulosis and chronic epithelial response. We believe this represents a potential pre-Sézary state that can he recognized by the course and the histologic pattern.     

A continuous T-cell line from a patient with Sézary syndrome

Summary A continuous cell line, Se-Ax, from a patient with Sézary syndrome has been established. The Se-Ax cell line is IL-2 dependent, requires human serum for permanent growth, and has the following phenotype: CD1-, CD2+, CD3+, CD4-, CD5-, CD8-, CD20-, CD25+; it expresses the T9, T10, and HLA-DR antigens. This cell line reveals multiple chromosome aberrations as seen in the most abundant abnormal clone in peripheral blood. Therefore, it is not unlikely that it derives from tumor cells. A putative cytotoxic cell line derived from the same patient has only weak killer-cell activity against the autologous permanent cell line: this CD8+ cytotoxic cell line has a 14q+ chromosomal marker. The fact that the patient demonstrated no natural killer-cell or activated killer-cell activity against the Se-Ax cell line may in part explain the successful establishment of the continuous cell line from bulk culture.     

Sézary syndrome with early immunoblastic transformation

Summary Two patients with clinical manifestations of Sézary syndrome are reported. In both cases from an early stage of the disease in addition to characteristic Sézary cells large numbers of immunoblasts were present in skin lesions and peripheral lymph nodes and in one case also in the blood. Their relationship to the characteristic Sézary cells was shown by morphological, cytochemical and immunological methods. The infiltrates in the skin were epidermotropic in one case and nonepidermotropic in the other. Lymph node structure was effaced by diffuse infiltration of abnormal lymphoid cells. These were found to proliferate in the skin as well as in lymph nodes. Cytogenetical studies of blood lymphocytes indicated an abnormal hypodiploid clone in both cases. Immunologically the tumour cells had properties of peripheral T-lymphocytes but whereas all abnormal cells exhibited inducer/helper cell characteristics in one case, only a minority of the lymphocytes revealed these characteristics in the other case. In this case the tumour cell population changed into a more pleomorphic type. The classification of the cases is discused.