Sézary syndrome without erythroderma

Henn, A.; Michel, Laurence; Fite, C.; Deschamps, Lydia; Ortonne, Nicolas; Ingen‐Housz‐Oro, Saskia; Marinho, E.; Beylot‐Barry, M.; Bagot, Martine; Laroche, Liliane; Crickx, B; Maubec, Ève

doi:10.1016/j.jaad.2014.11.015

Cited by 22 publications

(27 citation statements)

References 35 publications

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“…and in all six cases by Foulc et al . In contrast, Henn et al . reported that all six cases of SS without initial erythroderma never developed erythroderma in the disease course.…”

Section: Discussionmentioning

confidence: 81%

“…Based on these facts, it should be kept in mind that SS may initially present as symmetrical erythematous papules. Interestingly, five cases of SS without erythroderma had no apparent cutaneous findings, while skin specimens taken from them revealed dermal perivascular atypical lymphocyte infiltration, consistent with SS . It is unknown why skin lesions in SS without erythroderma varied, while initial skin manifestations might have been associated with the failure of the mechanism developing erythroderma.…”

Section: Discussionmentioning

confidence: 99%

“…There are conflicting reports as for the prognosis of SS without initial erythroderma. Henn et al . demonstrated a better prognosis than classic SS in a 9‐year median follow up, with only one death out of six unrelated to SS.…”

Section: Discussionmentioning

confidence: 99%

“…Similar patients have been reported formerly. [4][5][6][7][8][9][10] However, the clinical presentation of these patients is not fully described.…”

Section: Introductionmentioning

confidence: 99%

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

Kamijo

Miyagaki

Norimatsu

et al. 2018

The Journal of Dermatology

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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.

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“…and in all six cases by Foulc et al . In contrast, Henn et al . reported that all six cases of SS without initial erythroderma never developed erythroderma in the disease course.…”

Section: Discussionmentioning

confidence: 81%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

“…Similar patients have been reported formerly. [4][5][6][7][8][9][10] However, the clinical presentation of these patients is not fully described.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

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

Kamijo

Miyagaki

Norimatsu

et al. 2018

The Journal of Dermatology

View full text Add to dashboard Cite

show abstract

“…Das Sézary‐Syndrom zeigt eine B2‐leukämische Beteiligung mit ≥ 1.000 Zellen/μl und ist definiert als ein klonales Rearrangement des T‐Zell‐Rezeptors auf peripheren Lymphozyten, überwiegend mit einem CD4‐ oder CD3‐Phänotyp und einer CD4/CD8‐Ratio > 10 oder erhöhten CD4‐positiven T‐Zellen mit einem abnormalen Phänotyp einschließlich CD7‐ oder CD26‐Verlust .…”

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