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March 2005

Dyscrasias With “Undetermined Significance”

Arch Dermatol. 2005;141(3):382-384. doi:10.1001/archderm.141.3.382

Sézary and Bouvrain,1 at the Séance du 13 Février 1938 de la Société Française de Dermatologie et de Syphilographie, described a 58-year-old woman with “erythrodermie avec présence de cellules monstrueuses dans le derme et le sang circulant.” The patient also had pruritus, edema, lymphadenopathy, and hair loss.

Gniadecki and Lukowsky describe a group of patients with chronic recalcitrant erythroderma accompanied by a monoclonal expansion of CD4+72629+ T-lymphocytes and designate them as having “erythroderma with monoclonal T-cell dyscrasia of undetermined significance (MTUS-E).”2 These patients fulfill the criteria of the pre-Sezary syndrome, described by Winkelmann et al.3 Clinically, all patients had chronic recalcitrant erythroderma, but none developed any hematological malignancy during their lifetime or fulfilled the criteria of cutaneous lymphoma or Sezary syndrome.

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