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

Abstract

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.

Aprepitant in Anti-histamine-refractory Chronic Nodular Prurigo: A Multicentre, Randomized, Double-blind, Placebo-controlled, Cross-over, Phase-II trial (APREPRU)

“…Similar results have been published recently for the use of aprepitant in patients with atopic dermatitis in an open randomized controlled trial (RCT) (44) and in patients with Sézary syndrome in an RCT with crossover design (45). In 19 patients with atopic dermatitis, there was no improvement in the extent of the disease, intensity of pruritus or scratching movements found when aprepitant, 80 mg daily for 7 days, was added to topical therapy with a moderately strong steroid and a moisturizer (44).…”

“…In 19 patients with atopic dermatitis, there was no improvement in the extent of the disease, intensity of pruritus or scratching movements found when aprepitant, 80 mg daily for 7 days, was added to topical therapy with a moderately strong steroid and a moisturizer (44). Also, no antipruritic effect was found in 5 patients with Sézary syndrome receiving aprepitant 80 mg daily for 7 days (45).…”

Sézary Syndrome in West Sweden: Exploring Epidemiology, Clinical Features, and Treatment Patterns in a Registry-Based Retrospective Analysis

“…At the time of diagnosis, only 35% of patients showed the classical triad of symptoms, which suggests that these symptoms are not mandatory for a diagnosis. A prior study by Kamijo et al reviewed 37 cases of SS without initial erythroderma, 12 of which exhibited only lymphadenopathy [ 26 ]. Five patients in our study did not initially present with erythroderma.…”

Poor prognostic factors of Sézary syndrome: A retrospective single‐center study from Japan

“…Although one patient did not have erythroderma, he had symmetric ill‐demarcated erythema with itching and met criteria other than erythroderma and we decided to incorporate the patient into this study. Actually, the presence of nonerythrodermic SS has been widely known 14 . The collected clinical findings were age, gender, performance status (PS), and TNMB staging at diagnosis.…”

“…Actually, the presence of nonerythrodermic SS has been widely known. 14 The collected clinical findings were age, gender, performance status (PS), and TNMB staging at diagnosis. The collected hematological findings were serum levels of soluble IL-2 receptor (sIL-2R), LDH, thymus and activation-regulated chemokine (TARC), and immunoglobulin E (IgE), Sézary cell count, Sézary cell ratio among white blood cells, and eosinophil count in peripheral blood.…”